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acrac_3185039_9

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Most commonly, a widened mediastinum is appreciated in patients with pathology extending to the proximal to mid thoracic aorta, and a posteroanterior (PA) projection is found to be significantly more accurate than an anteroposterior (AP) projection [53]. Other studies have found that a chest radiograph is not sensitive (64%) or specific (87%) for thoracic aortic disease [54,55]. Given the relatively low sensitivity and specificity, chest radiography should not substitute for cross-sectional imaging. Furthermore, the role of chest radiography in the follow-up of known thoracoabdominal disease is limited because radiographs would be unlikely to appreciate subtle changes in aortic size. Radiography Chest, Abdomen, and Pelvis There is no relevant literature for chest, abdomen, and pelvis radiographs for the follow-up of thoracoabdominal aortic dissection or aneurysm. Chest radiographs demonstrate abnormalities in a large percentage of patients with acute thoracoabdominal pathology. Most commonly, a widened mediastinum is appreciated in patients with pathology extending to the proximal to mid thoracic aorta, and a PA projection is found to be significantly more accurate than an AP projection [53]. Other studies have found that a chest radiograph is not sensitive (64%) or specific (87%) for thoracic aortic disease [54,55]. Given the relatively low sensitivity and specificity, chest radiography should not substitute for cross-sectional imaging. Furthermore, the role of chest radiography in the follow-up of known thoracoabdominal disease is limited because radiographs would be unlikely to appreciate subtle changes in aortic size. US Duplex Doppler Aorta Abdomen Duplex ultrasound (US) of the abdominal aorta is an option for evaluation of the thoracoabdominal aorta, although the ability to evaluate the aorta above the diaphragm may be markedly limited by acoustic windows.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Most commonly, a widened mediastinum is appreciated in patients with pathology extending to the proximal to mid thoracic aorta, and a posteroanterior (PA) projection is found to be significantly more accurate than an anteroposterior (AP) projection [53]. Other studies have found that a chest radiograph is not sensitive (64%) or specific (87%) for thoracic aortic disease [54,55]. Given the relatively low sensitivity and specificity, chest radiography should not substitute for cross-sectional imaging. Furthermore, the role of chest radiography in the follow-up of known thoracoabdominal disease is limited because radiographs would be unlikely to appreciate subtle changes in aortic size. Radiography Chest, Abdomen, and Pelvis There is no relevant literature for chest, abdomen, and pelvis radiographs for the follow-up of thoracoabdominal aortic dissection or aneurysm. Chest radiographs demonstrate abnormalities in a large percentage of patients with acute thoracoabdominal pathology. Most commonly, a widened mediastinum is appreciated in patients with pathology extending to the proximal to mid thoracic aorta, and a PA projection is found to be significantly more accurate than an AP projection [53]. Other studies have found that a chest radiograph is not sensitive (64%) or specific (87%) for thoracic aortic disease [54,55]. Given the relatively low sensitivity and specificity, chest radiography should not substitute for cross-sectional imaging. Furthermore, the role of chest radiography in the follow-up of known thoracoabdominal disease is limited because radiographs would be unlikely to appreciate subtle changes in aortic size. US Duplex Doppler Aorta Abdomen Duplex ultrasound (US) of the abdominal aorta is an option for evaluation of the thoracoabdominal aorta, although the ability to evaluate the aorta above the diaphragm may be markedly limited by acoustic windows.

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acrac_3185039_10

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Prior studies comparing US, CT, and MRI of the abdominal aorta found that US is a reliable method to diagnose and follow AAAs [56]. In the evaluation of thoracoabdominal aortic dissection, US can also be used to evaluate blood flow in the true and false lumens and to directly and dynamically monitor the motion of dissection flaps [43]. As such, abdomen US can be performed serially to evaluate for aortic size changes or dissection hemodynamic changes. However, a limited ability to evaluate the thoracic aorta due to difficult acoustic windows could result in poor image quality or the inability to view changes to the aorta. US Echocardiography Transthoracic Resting Transthoracic echocardiogram (TTE) allows visualization of the heart and portions of the thoracic aorta. TTE can be used to evaluate the heart for complications such as pericardial effusion in patients with new symptoms [57]. However, portions of the proximal descending thoracic aorta may be poorly visualized with TTE because of patient acoustic windows and habitus, creating the possibility of false-negative examinations in patients with new symptoms [58]. These characteristics have resulted in a low sensitivity of 31% to 55% for the diagnosis of acute type B aortic dissection on TTE [33]. TTE is also limited by the ability to visualize the abdominal aorta to determine the extent of thoracoabdominal aortic pathology. Variant 2: Planning for endovascular or open repair of thoracoabdominal aorta aneurysm or dissection. Aortography Chest Abdomen Pelvis There is no relevant literature regarding aortography for the planning of thoracoabdominal aortic endovascular or open repair. Aortogram with DSA for evaluation of the thoracic and abdominal aorta has a sensitivity of up to 90% and a specificity of 95% for acute aortic syndrome [27]. The role of aortography before endovascular or open repair is limited to noninvasive modalities such as CTA and MRA.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Prior studies comparing US, CT, and MRI of the abdominal aorta found that US is a reliable method to diagnose and follow AAAs [56]. In the evaluation of thoracoabdominal aortic dissection, US can also be used to evaluate blood flow in the true and false lumens and to directly and dynamically monitor the motion of dissection flaps [43]. As such, abdomen US can be performed serially to evaluate for aortic size changes or dissection hemodynamic changes. However, a limited ability to evaluate the thoracic aorta due to difficult acoustic windows could result in poor image quality or the inability to view changes to the aorta. US Echocardiography Transthoracic Resting Transthoracic echocardiogram (TTE) allows visualization of the heart and portions of the thoracic aorta. TTE can be used to evaluate the heart for complications such as pericardial effusion in patients with new symptoms [57]. However, portions of the proximal descending thoracic aorta may be poorly visualized with TTE because of patient acoustic windows and habitus, creating the possibility of false-negative examinations in patients with new symptoms [58]. These characteristics have resulted in a low sensitivity of 31% to 55% for the diagnosis of acute type B aortic dissection on TTE [33]. TTE is also limited by the ability to visualize the abdominal aorta to determine the extent of thoracoabdominal aortic pathology. Variant 2: Planning for endovascular or open repair of thoracoabdominal aorta aneurysm or dissection. Aortography Chest Abdomen Pelvis There is no relevant literature regarding aortography for the planning of thoracoabdominal aortic endovascular or open repair. Aortogram with DSA for evaluation of the thoracic and abdominal aorta has a sensitivity of up to 90% and a specificity of 95% for acute aortic syndrome [27]. The role of aortography before endovascular or open repair is limited to noninvasive modalities such as CTA and MRA.

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acrac_3185039_11

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Furthermore, the projectional nature of catheter angiograms limits its ability to evaluate the 3-D configuration of vessels, increasing the risk of error if procedures are planned based on angiogram [59]. Thoracoabdominal Aortic Aneurysm or Dissection CT Chest, Abdomen, and Pelvis With IV Contrast There is no relevant literature for venous phase CT chest, abdomen, and pelvis for the planning of thoracoabdominal aortic endovascular or open repair. Most preprocedural CT imaging for thoracoabdominal pathology uses an arterially timed contrast bolus in the form of a CTA (discussed below). Although typically not performed for the purpose of procedural planning, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes in thoracoabdominal dissection or aneurysm. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest, Abdomen, and Pelvis Without and With IV Contrast There is no relevant literature for venous phase CT chest, abdomen, and pelvis with noncontrast phase for the planning of thoracoabdominal aortic endovascular or open repair. Most preprocedural CT imaging for thoracoabdominal pathology uses an arterially timed contrast bolus in the form of a CTA (discussed below). Although typically not performed for the purpose of procedural planning, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes in thoracoabdominal dissection or aneurysm. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Furthermore, the projectional nature of catheter angiograms limits its ability to evaluate the 3-D configuration of vessels, increasing the risk of error if procedures are planned based on angiogram [59]. Thoracoabdominal Aortic Aneurysm or Dissection CT Chest, Abdomen, and Pelvis With IV Contrast There is no relevant literature for venous phase CT chest, abdomen, and pelvis for the planning of thoracoabdominal aortic endovascular or open repair. Most preprocedural CT imaging for thoracoabdominal pathology uses an arterially timed contrast bolus in the form of a CTA (discussed below). Although typically not performed for the purpose of procedural planning, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes in thoracoabdominal dissection or aneurysm. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest, Abdomen, and Pelvis Without and With IV Contrast There is no relevant literature for venous phase CT chest, abdomen, and pelvis with noncontrast phase for the planning of thoracoabdominal aortic endovascular or open repair. Most preprocedural CT imaging for thoracoabdominal pathology uses an arterially timed contrast bolus in the form of a CTA (discussed below). Although typically not performed for the purpose of procedural planning, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes in thoracoabdominal dissection or aneurysm. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology.

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acrac_3185039_12

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

CT Chest, Abdomen, and Pelvis Without IV Contrast There is no relevant literature for CT chest, abdomen, and pelvis without IV contrast for the planning of thoracoabdominal aortic endovascular or open repair. CT without IV contrast would likely be able to assess aortic size and for nonvascular findings, but utility for preprocedure planning would be markedly limited in the absence of IV contrast. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest and Abdomen With IV Contrast There is no relevant literature for venous phase CT chest and abdomen for the planning of thoracoabdominal aortic endovascular or open repair. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis from the field of view carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest and Abdomen Without and With IV Contrast There is no relevant literature for venous phase CT chest and abdomen with noncontrast phase for the planning of thoracoabdominal aortic endovascular or open repair. Most preprocedural CT imaging for thoracoabdominal pathology uses an arterially timed contrast bolus in the form of a CTA (discussed below).

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. CT Chest, Abdomen, and Pelvis Without IV Contrast There is no relevant literature for CT chest, abdomen, and pelvis without IV contrast for the planning of thoracoabdominal aortic endovascular or open repair. CT without IV contrast would likely be able to assess aortic size and for nonvascular findings, but utility for preprocedure planning would be markedly limited in the absence of IV contrast. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest and Abdomen With IV Contrast There is no relevant literature for venous phase CT chest and abdomen for the planning of thoracoabdominal aortic endovascular or open repair. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis from the field of view carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest and Abdomen Without and With IV Contrast There is no relevant literature for venous phase CT chest and abdomen with noncontrast phase for the planning of thoracoabdominal aortic endovascular or open repair. Most preprocedural CT imaging for thoracoabdominal pathology uses an arterially timed contrast bolus in the form of a CTA (discussed below).

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acrac_3185039_13

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Although typically not performed for the purpose of procedural planning, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes in thoracoabdominal dissection or aneurysm. However, exclusion of the pelvis from the field of view carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest and Abdomen Without IV Contrast There is no relevant literature CT chest and abdomen without contrast for the planning of thoracoabdominal aortic endovascular or open repair. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis from the field of view carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. Thoracoabdominal Aortic Aneurysm or Dissection CTA Chest, Abdomen, and Pelvis With IV Contrast The high spatial resolution and profound, homogenous enhancement of the aorta and branch vessels in CTA allows for excellent preoperative assessment.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Although typically not performed for the purpose of procedural planning, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes in thoracoabdominal dissection or aneurysm. However, exclusion of the pelvis from the field of view carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. CT Chest and Abdomen Without IV Contrast There is no relevant literature CT chest and abdomen without contrast for the planning of thoracoabdominal aortic endovascular or open repair. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis from the field of view carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients but may be needed for preprocedure sizing and evaluation of pathology. Thoracoabdominal Aortic Aneurysm or Dissection CTA Chest, Abdomen, and Pelvis With IV Contrast The high spatial resolution and profound, homogenous enhancement of the aorta and branch vessels in CTA allows for excellent preoperative assessment.

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acrac_3185039_14

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Prior studies dating back to the 2000s have shown CTA to be a valuable tool to evaluate anatomic suitability for endovascular repair of the thoracic or abdominal aorta [31,60-63]. With anatomic coverage from the aortic root to the superficial femoral arteries, CTA can rapidly evaluate the extent of thoracoabdominal aneurysm or dissection as well as provide information valuable for preoperative planning such as aortic tortuosity, branch vessel location and patency, and suitability of femoral access vessels for endovascular repair. CTA can also readily evaluate for complications related to aortic pathology that could affect surgical or procedural plan including aortic rupture, dissection extension, or malperfusion syndrome. Although TAAA is traditionally treated with open repair, hybrid and endovascular repair of TAAA or dissections have increasingly been used over the past decade [14,64]. Techniques including snorkels/periscopes to allow perfusion of aortic branches, fenestrated EVAR, and branched EVAR have been used for thoracoabdominal aneurysms, and fenestration, branch vessel stenting, and true lumen stenting have progressively been used for thoracoabdominal dissection [9,14,64,65]. Many of these are complex procedures, and precise measurements of the aortic aneurysm or dissection before the procedure facilitates planning and ensures appropriate device availability at the time of procedure [9,65]. The thin slices, high spatial resolution, and isotropic data acquired with CTA permits advanced reconstruction techniques such as centerline measurements and double orthogonal measurements, permitting precise assessment of the aortic and branch vessel anatomy and detailed procedure planning [60- 62,66,67]. Inclusion of the pelvis in the study also aids in procedure planning by allowing evaluation of the iliofemoral access vessels.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Prior studies dating back to the 2000s have shown CTA to be a valuable tool to evaluate anatomic suitability for endovascular repair of the thoracic or abdominal aorta [31,60-63]. With anatomic coverage from the aortic root to the superficial femoral arteries, CTA can rapidly evaluate the extent of thoracoabdominal aneurysm or dissection as well as provide information valuable for preoperative planning such as aortic tortuosity, branch vessel location and patency, and suitability of femoral access vessels for endovascular repair. CTA can also readily evaluate for complications related to aortic pathology that could affect surgical or procedural plan including aortic rupture, dissection extension, or malperfusion syndrome. Although TAAA is traditionally treated with open repair, hybrid and endovascular repair of TAAA or dissections have increasingly been used over the past decade [14,64]. Techniques including snorkels/periscopes to allow perfusion of aortic branches, fenestrated EVAR, and branched EVAR have been used for thoracoabdominal aneurysms, and fenestration, branch vessel stenting, and true lumen stenting have progressively been used for thoracoabdominal dissection [9,14,64,65]. Many of these are complex procedures, and precise measurements of the aortic aneurysm or dissection before the procedure facilitates planning and ensures appropriate device availability at the time of procedure [9,65]. The thin slices, high spatial resolution, and isotropic data acquired with CTA permits advanced reconstruction techniques such as centerline measurements and double orthogonal measurements, permitting precise assessment of the aortic and branch vessel anatomy and detailed procedure planning [60- 62,66,67]. Inclusion of the pelvis in the study also aids in procedure planning by allowing evaluation of the iliofemoral access vessels.

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acrac_3185039_15

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Femoral access is typically preferred for endovascular repair, although access vessels must typically be suitable in size, tortuosity, and calcification of the iliac vessels to permit device delivery to the aorta. In patients with inappropriate iliofemoral vessels, groin cutdown with conduit, direct aortoiliac access, or brachial access can be used. Before open or endovascular repair of a thoracoabdominal aneurysm or dissection, some authors have advocated for imaging for identification of the artery of Adamkiewicz, because preoperative identification could potentially reduce the risk of spinal cord ischemia [68,69]. Given the variability of the origin of this artery, preoperative identification of the origin of this vessel allows for operative planning that minimizes the risk of damage and can reduce surgical times [69]. Multiple studies evaluating the ability of CT and MRI to identify the artery of Adamkiewicz show that the artery can be identified and traced in >75% of patients, with some studies finding >90% identification [68,70-72]. CTA Chest and Abdomen With IV Contrast Compared with CTA of the chest, abdomen, and pelvis, exclusion of the pelvis carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. MRA Chest, Abdomen, and Pelvis Without and With IV Contrast Although the spatial resolution of MRA without and with IV contrast is less than the spatial resolution of CTA with IV contrast, MRA provides good evaluation of the aorta and branch vessels to allow for procedural or operative planning [38,62,67].

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Femoral access is typically preferred for endovascular repair, although access vessels must typically be suitable in size, tortuosity, and calcification of the iliac vessels to permit device delivery to the aorta. In patients with inappropriate iliofemoral vessels, groin cutdown with conduit, direct aortoiliac access, or brachial access can be used. Before open or endovascular repair of a thoracoabdominal aneurysm or dissection, some authors have advocated for imaging for identification of the artery of Adamkiewicz, because preoperative identification could potentially reduce the risk of spinal cord ischemia [68,69]. Given the variability of the origin of this artery, preoperative identification of the origin of this vessel allows for operative planning that minimizes the risk of damage and can reduce surgical times [69]. Multiple studies evaluating the ability of CT and MRI to identify the artery of Adamkiewicz show that the artery can be identified and traced in >75% of patients, with some studies finding >90% identification [68,70-72]. CTA Chest and Abdomen With IV Contrast Compared with CTA of the chest, abdomen, and pelvis, exclusion of the pelvis carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. MRA Chest, Abdomen, and Pelvis Without and With IV Contrast Although the spatial resolution of MRA without and with IV contrast is less than the spatial resolution of CTA with IV contrast, MRA provides good evaluation of the aorta and branch vessels to allow for procedural or operative planning [38,62,67].

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acrac_3185039_16

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

A 2011 study compared image quality, vessel measurements, and proposed endograft selection among MRA and CTA in 30 patients scheduled for EVAR [67]. This analysis found small differences in measured aortic diameter at multiple locations, all <1 mm between MRA and CTA [67]. Ultimately, this study concluded that the image quality for both CTA and MRA was, in general, adequate and that differences were not clinically relevant because all 30 patients had the same endograft components selected based on measurements [67]. Although the study was focused on AAA, the result is likely applicable to measurements of thoracoabdominal aneurysm or dissection, with the caveat that the complexity of endovascular thoracoabdominal repair may place a higher value on precise measurements. MRA is thus able to evaluate the extent of thoracoabdominal aneurysm or dissection as well as to provide information valuable for preoperative planning such as aortic tortuosity, branch vessel location and patency, and suitability of femoral access vessels for endovascular repair. MRA can also evaluate for complications related to aortic pathology that could affect surgical or procedural plan including aortic rupture, dissection extension, or malperfusion syndrome. Thoracoabdominal Aortic Aneurysm or Dissection Although TAAA is traditionally treated with open repair, hybrid and endovascular repair of TAAA or dissections have increasingly been used over the past decade [14,64]. Techniques including snorkels/periscopes to allow perfusion of aortic branches, fenestrated EVAR, and branched EVAR have been used for thoracoabdominal aneurysms, and fenestration, branch vessel stenting, and true lumen stenting have progressively been used for thoracoabdominal dissection [9,14,64,65].

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. A 2011 study compared image quality, vessel measurements, and proposed endograft selection among MRA and CTA in 30 patients scheduled for EVAR [67]. This analysis found small differences in measured aortic diameter at multiple locations, all <1 mm between MRA and CTA [67]. Ultimately, this study concluded that the image quality for both CTA and MRA was, in general, adequate and that differences were not clinically relevant because all 30 patients had the same endograft components selected based on measurements [67]. Although the study was focused on AAA, the result is likely applicable to measurements of thoracoabdominal aneurysm or dissection, with the caveat that the complexity of endovascular thoracoabdominal repair may place a higher value on precise measurements. MRA is thus able to evaluate the extent of thoracoabdominal aneurysm or dissection as well as to provide information valuable for preoperative planning such as aortic tortuosity, branch vessel location and patency, and suitability of femoral access vessels for endovascular repair. MRA can also evaluate for complications related to aortic pathology that could affect surgical or procedural plan including aortic rupture, dissection extension, or malperfusion syndrome. Thoracoabdominal Aortic Aneurysm or Dissection Although TAAA is traditionally treated with open repair, hybrid and endovascular repair of TAAA or dissections have increasingly been used over the past decade [14,64]. Techniques including snorkels/periscopes to allow perfusion of aortic branches, fenestrated EVAR, and branched EVAR have been used for thoracoabdominal aneurysms, and fenestration, branch vessel stenting, and true lumen stenting have progressively been used for thoracoabdominal dissection [9,14,64,65].

3185039

acrac_3185039_17

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Many of these are complex procedures, and precise measurements of the aortic aneurysm or dissection before procedure facilitates planning and ensures appropriate device availability at the time of procedure [9,65] Inclusion of the pelvis in the study also aids in procedure planning by allowing evaluation of the iliofemoral access vessels. Femoral access is typically preferred for endovascular repair, although access vessels must typically be suitable in size, tortuosity, and calcification of the iliac vessels to permit device delivery to the aorta. In patients with inappropriate iliofemoral vessels, groin cutdown with conduit, direct aortoiliac access, or brachial access can be used. Before open or endovascular repair of a thoracoabdominal aneurysm or dissection, some authors have advocated for imaging for identification of the artery of Adamkiewicz because preoperative identification could potentially reduce the risk of spinal cord ischemia [68]. Given the variability of the origin of this artery, preoperative identification of the origin of this vessel allows for operative planning that minimizes risk of damage. Multiple studies evaluating the ability of CT and MRI to identify the artery of Adamkiewicz show that the artery can be identified and traced in >75% of patients, with identification rates typically higher in MRA compared with CTA [68,70-73]. MRA Chest, Abdomen, and Pelvis Without IV Contrast A 2012 study by Shaida et al [63] compared aortic measurements in 20 patients undergoing both CTA and noncontrast MRI before EVAR. The study measured vessel diameter at multiple points as well as several vessel lengths and found small discrepancies between MRI and CTA, typically <1 mm for diameters or 5 mm for lengths [63]. The authors concluded such measurements were unlikely to alter planning of the repair but favored CTA in most patients [63].

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Many of these are complex procedures, and precise measurements of the aortic aneurysm or dissection before procedure facilitates planning and ensures appropriate device availability at the time of procedure [9,65] Inclusion of the pelvis in the study also aids in procedure planning by allowing evaluation of the iliofemoral access vessels. Femoral access is typically preferred for endovascular repair, although access vessels must typically be suitable in size, tortuosity, and calcification of the iliac vessels to permit device delivery to the aorta. In patients with inappropriate iliofemoral vessels, groin cutdown with conduit, direct aortoiliac access, or brachial access can be used. Before open or endovascular repair of a thoracoabdominal aneurysm or dissection, some authors have advocated for imaging for identification of the artery of Adamkiewicz because preoperative identification could potentially reduce the risk of spinal cord ischemia [68]. Given the variability of the origin of this artery, preoperative identification of the origin of this vessel allows for operative planning that minimizes risk of damage. Multiple studies evaluating the ability of CT and MRI to identify the artery of Adamkiewicz show that the artery can be identified and traced in >75% of patients, with identification rates typically higher in MRA compared with CTA [68,70-73]. MRA Chest, Abdomen, and Pelvis Without IV Contrast A 2012 study by Shaida et al [63] compared aortic measurements in 20 patients undergoing both CTA and noncontrast MRI before EVAR. The study measured vessel diameter at multiple points as well as several vessel lengths and found small discrepancies between MRI and CTA, typically <1 mm for diameters or 5 mm for lengths [63]. The authors concluded such measurements were unlikely to alter planning of the repair but favored CTA in most patients [63].

3185039

acrac_3185039_18

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Although this study focused on AAA, the result is likely applicable to measurements of thoracoabdominal aneurysm or dissection, with the caveat that the complexity of endovascular thoracoabdominal repair may place a higher value on precise measurements. Similar conclusions were reached by a 2013 study comparing pre-EVAR MRI without IV contrast and CTA [61,74]. MRA Chest and Abdomen Without and With IV Contrast Compared with MRA of the chest, abdomen, and pelvis, exclusion of the pelvis carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. MRA Chest and Abdomen Without IV Contrast Compared with MRA of the chest, abdomen, and pelvis, exclusion of the pelvis carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. Radiography Chest There is no relevant literature regarding the use of radiography for planning thoracoabdominal aortic endovascular or open repair. Radiography Chest Abdomen Pelvis There is no relevant literature regarding the use of radiography for planning thoracoabdominal aortic endovascular or open repair.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Although this study focused on AAA, the result is likely applicable to measurements of thoracoabdominal aneurysm or dissection, with the caveat that the complexity of endovascular thoracoabdominal repair may place a higher value on precise measurements. Similar conclusions were reached by a 2013 study comparing pre-EVAR MRI without IV contrast and CTA [61,74]. MRA Chest and Abdomen Without and With IV Contrast Compared with MRA of the chest, abdomen, and pelvis, exclusion of the pelvis carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. MRA Chest and Abdomen Without IV Contrast Compared with MRA of the chest, abdomen, and pelvis, exclusion of the pelvis carries the drawback of precluding evaluation of the iliofemoral access vessels if endovascular repair is considered. Furthermore, if the thoracoabdominal dissection or aneurysm extends into the pelvis, a lack of pelvis evaluation could result in incomplete evaluation of the aortic pathology. In select cases with recent imaging of the pelvis or in cases of planned open repair without extension into the pelvic vasculature, further imaging of the pelvis vasculature may not be needed. Radiography Chest There is no relevant literature regarding the use of radiography for planning thoracoabdominal aortic endovascular or open repair. Radiography Chest Abdomen Pelvis There is no relevant literature regarding the use of radiography for planning thoracoabdominal aortic endovascular or open repair.

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acrac_3185039_19

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

US Duplex Doppler Aorta Abdomen There is no relevant literature regarding the use of duplex US for planning thoracoabdominal aortic endovascular or open repair. Although US can be used to evaluate the abdominal aorta for dissection or aneurysm, limitations to Thoracoabdominal Aortic Aneurysm or Dissection the evaluation of the spatial relationship of the aorta to branch vessels and the inability to visualize portions of the aorta limit its utility as a sole modality for procedural planning. US Echocardiography Transthoracic Resting There is no relevant literature regarding the use of TTE for planning thoracoabdominal aortic endovascular or open repair. Although TTE can evaluate for cardiac complications such as aortic valve regurgitation [33] and evaluate portions of the thoracic aorta, limitations to the evaluation of the spatial relationship of the aorta to branch vessels, the inability to visualize portions of the aorta, and a dependence on operator and patient characteristics limit its utility as a sole modality for procedural planning. Variant 3: Follow-up after endovascular repair of thoracoabdominal aortic aneurysm or dissection. Aortography Chest Abdomen Pelvis Aortogram with DSA for evaluation of the thoracic and abdominal aorta has a sensitivity of up to 90% and a specificity of 95% for acute aortic syndrome [27]. The role of aortography for routine follow-up after endovascular repair is limited in favor of noninvasive modalities such as CTA and MRA, and CTA has been found to have a higher sensitivity than angiogram [75,76]. In cases of increasing aneurysm sac size likely to require treatment of endoleak, aortogram could be considered because it would allow rapid transition from diagnosis to treatment. Additional benefits of angiogram include the ability to evaluate directionality of endoleaks, which can be difficult on CTA [76].

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. US Duplex Doppler Aorta Abdomen There is no relevant literature regarding the use of duplex US for planning thoracoabdominal aortic endovascular or open repair. Although US can be used to evaluate the abdominal aorta for dissection or aneurysm, limitations to Thoracoabdominal Aortic Aneurysm or Dissection the evaluation of the spatial relationship of the aorta to branch vessels and the inability to visualize portions of the aorta limit its utility as a sole modality for procedural planning. US Echocardiography Transthoracic Resting There is no relevant literature regarding the use of TTE for planning thoracoabdominal aortic endovascular or open repair. Although TTE can evaluate for cardiac complications such as aortic valve regurgitation [33] and evaluate portions of the thoracic aorta, limitations to the evaluation of the spatial relationship of the aorta to branch vessels, the inability to visualize portions of the aorta, and a dependence on operator and patient characteristics limit its utility as a sole modality for procedural planning. Variant 3: Follow-up after endovascular repair of thoracoabdominal aortic aneurysm or dissection. Aortography Chest Abdomen Pelvis Aortogram with DSA for evaluation of the thoracic and abdominal aorta has a sensitivity of up to 90% and a specificity of 95% for acute aortic syndrome [27]. The role of aortography for routine follow-up after endovascular repair is limited in favor of noninvasive modalities such as CTA and MRA, and CTA has been found to have a higher sensitivity than angiogram [75,76]. In cases of increasing aneurysm sac size likely to require treatment of endoleak, aortogram could be considered because it would allow rapid transition from diagnosis to treatment. Additional benefits of angiogram include the ability to evaluate directionality of endoleaks, which can be difficult on CTA [76].

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

CT Chest, Abdomen, and Pelvis With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis for the follow-up of endovascular repair of thoracoabdominal aneurysm or dissection because most follow-up imaging uses an arterially timed contrast bolus in the form of a CTA (discussed below). However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology, although with a reduced sensitivity to delineating endoleak and subtle changes to aorta and branch artery diameter. Although typically not performed for the purpose of follow-up of thoracoabdominal aortic pathology, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes after endovascular repair including endoprosthesis migration or aortic rupture. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without and With IV Contrast There is no relevant literature for venous and unenhanced CT chest, abdomen, and pelvis without an arterial phase for follow-up after thoracoabdominal endovascular repair. However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology, although with a reduced sensitivity to delineating endoleak and subtle changes to aorta and branch artery diameter. Although typically not performed for the purpose of follow-up of thoracoabdominal aortic pathology, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes after endovascular repair including endoprosthesis migration or aortic rupture. The addition of a noncontrast phase would be expected to aid in identifying endoleak and distinguishing endoleak from other sources of sac/false lumen opacification, although a lack of an arterial phase limits sensitivity.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. CT Chest, Abdomen, and Pelvis With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis for the follow-up of endovascular repair of thoracoabdominal aneurysm or dissection because most follow-up imaging uses an arterially timed contrast bolus in the form of a CTA (discussed below). However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology, although with a reduced sensitivity to delineating endoleak and subtle changes to aorta and branch artery diameter. Although typically not performed for the purpose of follow-up of thoracoabdominal aortic pathology, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes after endovascular repair including endoprosthesis migration or aortic rupture. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without and With IV Contrast There is no relevant literature for venous and unenhanced CT chest, abdomen, and pelvis without an arterial phase for follow-up after thoracoabdominal endovascular repair. However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology, although with a reduced sensitivity to delineating endoleak and subtle changes to aorta and branch artery diameter. Although typically not performed for the purpose of follow-up of thoracoabdominal aortic pathology, contrast-enhanced CT performed to evaluate for extravascular pathology can often assess for acute changes after endovascular repair including endoprosthesis migration or aortic rupture. The addition of a noncontrast phase would be expected to aid in identifying endoleak and distinguishing endoleak from other sources of sac/false lumen opacification, although a lack of an arterial phase limits sensitivity.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without IV Contrast Typical CT follow-up after endovascular thoracoabdominal aortic repair incorporates arterial and delayed venous phases to evaluate for endoleak [77]. However, unenhanced CT can be used to evaluate aortic caliber to detect changes to a thoracoabdominal aneurysm or aneurysmal degeneration of a thoracoabdominal dissection. Drawbacks of unenhanced imaging include an inability to identify endoleak, evaluate branch vessel patency, or evaluate for false lumen thrombosis in aortic dissection. Nonetheless, some authors have advocated for routine use of noncontrast-enhanced CT over contrast-enhanced CT in patients after EVAR with stable aneurysm sac size, with contrast-enhanced CTA used for evaluation if aortic sac size changes [32,76]. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen With IV Contrast There is no relevant literature regarding venous phase CT chest and abdomen for the follow-up of endovascular repair of thoracoabdominal aneurysm or dissection. Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new Thoracoabdominal Aortic Aneurysm or Dissection pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without and With IV Contrast There is no relevant literature for venous and unenhanced CT without an arterial phase for follow-up after thoracoabdominal endovascular repair.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without IV Contrast Typical CT follow-up after endovascular thoracoabdominal aortic repair incorporates arterial and delayed venous phases to evaluate for endoleak [77]. However, unenhanced CT can be used to evaluate aortic caliber to detect changes to a thoracoabdominal aneurysm or aneurysmal degeneration of a thoracoabdominal dissection. Drawbacks of unenhanced imaging include an inability to identify endoleak, evaluate branch vessel patency, or evaluate for false lumen thrombosis in aortic dissection. Nonetheless, some authors have advocated for routine use of noncontrast-enhanced CT over contrast-enhanced CT in patients after EVAR with stable aneurysm sac size, with contrast-enhanced CTA used for evaluation if aortic sac size changes [32,76]. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen With IV Contrast There is no relevant literature regarding venous phase CT chest and abdomen for the follow-up of endovascular repair of thoracoabdominal aneurysm or dissection. Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new Thoracoabdominal Aortic Aneurysm or Dissection pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without and With IV Contrast There is no relevant literature for venous and unenhanced CT without an arterial phase for follow-up after thoracoabdominal endovascular repair.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without IV Contrast Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CTA Chest, Abdomen, and Pelvis With IV Contrast CTA is ideal for evaluation after endovascular repair because of its sensitivity in detecting endoleaks, ability to detect changes in aortic diameter, evaluation of false lumen thrombosis, endograft infection, and assessment of branch vessel/stent patency [1,2,77-79]. Because most endovascular repairs of TAAA or dissection are complex, the need for routine surveillance after treatment is crucial, and often, lifelong follow-up is recommended. A 2016 study of 354 patients undergoing endovascular repair for thoracoabdominal aortic pathology underscores the need for close follow-up, because 36% of patients required further intervention within 36 months, most commonly for endoleak [14]. Typically, follow-up imaging is obtained at 1, 3, and 6 months after intervention to evaluate for endoleak, increase in aortic diameter, incomplete false lumen thrombosis (in dissection), and other procedure complications, and prior studies have shown poor compliance with follow-up and increased risk of aortic rupture in these patients with limited imaging follow-up [17,80]. After 6 months, individualized imaging follow-up may be planned based on personal risk factors, with imaging typically at 6- to 12-month intervals.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without IV Contrast Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CTA Chest, Abdomen, and Pelvis With IV Contrast CTA is ideal for evaluation after endovascular repair because of its sensitivity in detecting endoleaks, ability to detect changes in aortic diameter, evaluation of false lumen thrombosis, endograft infection, and assessment of branch vessel/stent patency [1,2,77-79]. Because most endovascular repairs of TAAA or dissection are complex, the need for routine surveillance after treatment is crucial, and often, lifelong follow-up is recommended. A 2016 study of 354 patients undergoing endovascular repair for thoracoabdominal aortic pathology underscores the need for close follow-up, because 36% of patients required further intervention within 36 months, most commonly for endoleak [14]. Typically, follow-up imaging is obtained at 1, 3, and 6 months after intervention to evaluate for endoleak, increase in aortic diameter, incomplete false lumen thrombosis (in dissection), and other procedure complications, and prior studies have shown poor compliance with follow-up and increased risk of aortic rupture in these patients with limited imaging follow-up [17,80]. After 6 months, individualized imaging follow-up may be planned based on personal risk factors, with imaging typically at 6- to 12-month intervals.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Although direct evidence comparing CTA to other modalities in endoleak identification after endovascular thoracoabdominal aneurysm repair is lacking, multiple studies and meta-analyses have compared CTA with MRI and US for endoleak identification after EVAR for AAA [78,79,82,83]. A meta-analysis by Guo et al [82] in 2016 evaluated 3,853 patients after EVAR with paired scans of different modalities (CTA, MRA, US) within a 1-month period. In 2,346 paired CTA and duplex US scans, CTA identified 214 additional endoleaks not seen on duplex US (including 26 type I or type III endoleaks), whereas duplex US identified 77 additional endoleaks not seen on CTA (no type I or III endoleaks) [82]. In 1,694 paired CTA and MRA scans, CTA identified 2 additional endoleaks, whereas MRA identified 42 additional endoleaks [82]. A subsequent meta-analysis in 2018 by Zaiem et al [84] also noted that MRI identified more endoleaks than CTA. These findings suggest higher sensitivity of MRI to detect endoleaks, although the authors caution that the increased endoleaks identified could represent false-positive findings or endoleaks that are not clinically important [84]. CTA Chest and Abdomen With IV Contrast Compared with CTA of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature that could require additional intervention. MRA Chest, Abdomen, and Pelvis Without and With IV Contrast The spatial resolution of MRA is less than that of CTA, but the ability to detect branch vessel complications, endoleaks, stent graft complication, or infection has established MRA as an effective modality for imaging after endovascular repair, particularly for nitinol stent grafts, which have reduced susceptibility artifact.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Although direct evidence comparing CTA to other modalities in endoleak identification after endovascular thoracoabdominal aneurysm repair is lacking, multiple studies and meta-analyses have compared CTA with MRI and US for endoleak identification after EVAR for AAA [78,79,82,83]. A meta-analysis by Guo et al [82] in 2016 evaluated 3,853 patients after EVAR with paired scans of different modalities (CTA, MRA, US) within a 1-month period. In 2,346 paired CTA and duplex US scans, CTA identified 214 additional endoleaks not seen on duplex US (including 26 type I or type III endoleaks), whereas duplex US identified 77 additional endoleaks not seen on CTA (no type I or III endoleaks) [82]. In 1,694 paired CTA and MRA scans, CTA identified 2 additional endoleaks, whereas MRA identified 42 additional endoleaks [82]. A subsequent meta-analysis in 2018 by Zaiem et al [84] also noted that MRI identified more endoleaks than CTA. These findings suggest higher sensitivity of MRI to detect endoleaks, although the authors caution that the increased endoleaks identified could represent false-positive findings or endoleaks that are not clinically important [84]. CTA Chest and Abdomen With IV Contrast Compared with CTA of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature that could require additional intervention. MRA Chest, Abdomen, and Pelvis Without and With IV Contrast The spatial resolution of MRA is less than that of CTA, but the ability to detect branch vessel complications, endoleaks, stent graft complication, or infection has established MRA as an effective modality for imaging after endovascular repair, particularly for nitinol stent grafts, which have reduced susceptibility artifact.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Because most thoracoabdominal aortic repairs are complex, the need for routine surveillance after treatment is crucial, and often, lifelong follow-up is recommended. A 2016 study of 354 patients undergoing endovascular repair for Thoracoabdominal Aortic Aneurysm or Dissection thoracoabdominal aortic pathology underscores the need for close follow-up, because 36% of patients required further intervention within 36 months, most commonly for endoleak [14]. Typically, follow-up imaging is obtained at 1, 3, and 6 months after intervention to evaluate for endoleak, increase in aortic diameter, incomplete false lumen thrombosis (in dissection), and other procedure complications, and prior studies have shown poor compliance with follow-up with increased risk of aortic rupture in patients with poor follow-up [17,80]. After 6 months, individualized imaging follow-up may be planned based on personal risk factors, with imaging typically at 6 to 12 month intervals. Early studies evaluating the ability of MRA to evaluate for endoleak or other complication after EVAR established MRI as a suitable alternative to CTA [78,85,86]. Direct evidence comparing MRA with IV contrast to other modalities in endoleak identification after endovascular thoracoabdominal aneurysm repair is lacking, but multiple additional studies and meta-analyses have compared MRA with CTA in identifying endoleaks after EVAR [78,79,82,83,85,86]. A meta-analysis by Guo et al [82] in 2016 evaluated 3,853 patients after EVAR with paired scans of different modalities (CTA, MRA, US) obtained within a 1-month period. In 1,694 paired CTA and MRA scans, CTA identified 2 additional endoleaks, whereas MRA identified 42 additional endoleaks [82]. A subsequent meta-analysis in 2018 by Zaiem et al also noted that MRI identified more endoleaks than CTA [84].

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Because most thoracoabdominal aortic repairs are complex, the need for routine surveillance after treatment is crucial, and often, lifelong follow-up is recommended. A 2016 study of 354 patients undergoing endovascular repair for Thoracoabdominal Aortic Aneurysm or Dissection thoracoabdominal aortic pathology underscores the need for close follow-up, because 36% of patients required further intervention within 36 months, most commonly for endoleak [14]. Typically, follow-up imaging is obtained at 1, 3, and 6 months after intervention to evaluate for endoleak, increase in aortic diameter, incomplete false lumen thrombosis (in dissection), and other procedure complications, and prior studies have shown poor compliance with follow-up with increased risk of aortic rupture in patients with poor follow-up [17,80]. After 6 months, individualized imaging follow-up may be planned based on personal risk factors, with imaging typically at 6 to 12 month intervals. Early studies evaluating the ability of MRA to evaluate for endoleak or other complication after EVAR established MRI as a suitable alternative to CTA [78,85,86]. Direct evidence comparing MRA with IV contrast to other modalities in endoleak identification after endovascular thoracoabdominal aneurysm repair is lacking, but multiple additional studies and meta-analyses have compared MRA with CTA in identifying endoleaks after EVAR [78,79,82,83,85,86]. A meta-analysis by Guo et al [82] in 2016 evaluated 3,853 patients after EVAR with paired scans of different modalities (CTA, MRA, US) obtained within a 1-month period. In 1,694 paired CTA and MRA scans, CTA identified 2 additional endoleaks, whereas MRA identified 42 additional endoleaks [82]. A subsequent meta-analysis in 2018 by Zaiem et al also noted that MRI identified more endoleaks than CTA [84].

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

These findings suggest a higher sensitivity of MRI in identification of endoleak compared with CTA, although the authors caution that the increased endoleaks identified could represent false-positive findings or endoleaks that are not clinically important [84]. MRA with IV contrast also allows superior evaluation of flow dynamics compared with CTA. Time resolved and 4-D flow MRA can be used to improve detection and classification of endoleaks [76,87,88]. Use of multiple phases of contrast can be used in 4-D flow MRA to ascertain not only the presence of endoleak but also the endoleak type [88]. MRA with IV contrast has also shown value in predicting the persistence of type II endoleak. A retrospective review of MRAs with type II endoleak performed by Katahashi et al determined that the use of flow quantification could be used to accurately predict persistence or resolution of type II endoleak after EVAR, although this algorithm has not been applied prospectively [20]. MRA Chest, Abdomen, and Pelvis Without IV Contrast Literature supporting the use of MRA without IV contrast for evaluation after endovascular thoracoabdominal aortic repair is limited compared with evidence of MRA with IV contrast. A 2019 study evaluating 8 patients used a noncontrast MRI protocol to assess for endoleak after EVAR [89]. This study compared noncontrast MRA with contrast-enhanced CTA and angiogram and found the MRAs had comparable ability to detect endoleaks and assess aneurysm size [89]. The applicability of these findings to imaging post-thoracoabdominal endovascular intervention is unknown, although applicability may be incomplete because of multiple stents within the aorta and branch vessels. Additionally, the ability of this sequence to obtain widespread use is unclear. As such, noncontrast MRA would generally be expected to have a lower sensitivity for endoleak, false lumen thrombosis, or branch vessel patency, although evidence is lacking.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. These findings suggest a higher sensitivity of MRI in identification of endoleak compared with CTA, although the authors caution that the increased endoleaks identified could represent false-positive findings or endoleaks that are not clinically important [84]. MRA with IV contrast also allows superior evaluation of flow dynamics compared with CTA. Time resolved and 4-D flow MRA can be used to improve detection and classification of endoleaks [76,87,88]. Use of multiple phases of contrast can be used in 4-D flow MRA to ascertain not only the presence of endoleak but also the endoleak type [88]. MRA with IV contrast has also shown value in predicting the persistence of type II endoleak. A retrospective review of MRAs with type II endoleak performed by Katahashi et al determined that the use of flow quantification could be used to accurately predict persistence or resolution of type II endoleak after EVAR, although this algorithm has not been applied prospectively [20]. MRA Chest, Abdomen, and Pelvis Without IV Contrast Literature supporting the use of MRA without IV contrast for evaluation after endovascular thoracoabdominal aortic repair is limited compared with evidence of MRA with IV contrast. A 2019 study evaluating 8 patients used a noncontrast MRI protocol to assess for endoleak after EVAR [89]. This study compared noncontrast MRA with contrast-enhanced CTA and angiogram and found the MRAs had comparable ability to detect endoleaks and assess aneurysm size [89]. The applicability of these findings to imaging post-thoracoabdominal endovascular intervention is unknown, although applicability may be incomplete because of multiple stents within the aorta and branch vessels. Additionally, the ability of this sequence to obtain widespread use is unclear. As such, noncontrast MRA would generally be expected to have a lower sensitivity for endoleak, false lumen thrombosis, or branch vessel patency, although evidence is lacking.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

MRA Chest and Abdomen Without and With IV Contrast Compared with MRA of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis carries the advantage of reduced imaging time although may fail to detect changing or new pathology in the pelvic vasculature that could require further intervention. MRA Chest and Abdomen Without IV Contrast Compared with MRA of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis carries the advantage of reduced imaging time although may fail to detect changing or new pathology in the pelvic vasculature that could require further intervention. Radiography Chest Postintervention radiography can be used to monitor endograft position and integrity, with location relative to bony landmarks used to infer possible stent migration [76]. Additionally, prior studies have reported the value of radiography to evaluate for endograft fracture and kinking [76,90]. However, the low incidence of stent fracture and the inability of radiography to evaluate for increasing aneurysm sac size, branch occlusion, and many other complications limits value in routine use. Additionally, a radiograph of the chest only is likely to include only a portion of the repaired aorta within the field of view. Thoracoabdominal Aortic Aneurysm or Dissection Radiography Chest Abdomen Pelvis Postintervention radiography can be used to monitor endograft position and integrity, with location relative to bony landmarks used to infer possible stent migration [76]. Additionally, prior studies have reported the value of radiography to evaluate for endograft fracture and kinking [76,90]. However, the low incidence of stent fracture and the inability of radiography to evaluate for increasing aneurysm sac size, branch occlusion, and many other complications limits value in routine use.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. MRA Chest and Abdomen Without and With IV Contrast Compared with MRA of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis carries the advantage of reduced imaging time although may fail to detect changing or new pathology in the pelvic vasculature that could require further intervention. MRA Chest and Abdomen Without IV Contrast Compared with MRA of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis carries the advantage of reduced imaging time although may fail to detect changing or new pathology in the pelvic vasculature that could require further intervention. Radiography Chest Postintervention radiography can be used to monitor endograft position and integrity, with location relative to bony landmarks used to infer possible stent migration [76]. Additionally, prior studies have reported the value of radiography to evaluate for endograft fracture and kinking [76,90]. However, the low incidence of stent fracture and the inability of radiography to evaluate for increasing aneurysm sac size, branch occlusion, and many other complications limits value in routine use. Additionally, a radiograph of the chest only is likely to include only a portion of the repaired aorta within the field of view. Thoracoabdominal Aortic Aneurysm or Dissection Radiography Chest Abdomen Pelvis Postintervention radiography can be used to monitor endograft position and integrity, with location relative to bony landmarks used to infer possible stent migration [76]. Additionally, prior studies have reported the value of radiography to evaluate for endograft fracture and kinking [76,90]. However, the low incidence of stent fracture and the inability of radiography to evaluate for increasing aneurysm sac size, branch occlusion, and many other complications limits value in routine use.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

US Duplex Doppler Aorta Abdomen Duplex US of the abdominal aorta carries the advantages of being a readily obtainable bedside examination for evaluation of the distal thoracic and abdominal aorta. After EVAR, duplex US can be used to evaluate aortic diameter and presence of endoleak, with a good correlation of aortic size with CTA in most patients [76,91]. US Echocardiography Transthoracic Resting TTE allows visualization of the heart and portions of the thoracic aorta. However, portions of the proximal descending thoracic aorta may be poorly visualized with TTE, and examination may be limited by poor acoustic windows, creating the possibility of false-negative examinations in following patients after endovascular thoracoabdominal aortic repair [58]. These characteristics have resulted in a low sensitivity of 31% to 55% for diagnosis of acute type B aortic dissection on TTE [33]. TTE is also limited by the ability to visualize the abdominal aorta to determine the extent of thoracoabdominal aortic pathology. Variant 4: Follow-up after open repair of thoracoabdominal aortic aneurysm or dissection. Aortography Chest Abdomen Pelvis There is no relevant literature regarding the use of aortography in the evaluation of follow-up of open repair of TAAA or dissection. Aortogram with DSA for evaluation of the thoracic and abdominal aorta has a sensitivity of up to 90% and a specificity of 95% for acute aortic syndrome [27]. Although it has the advantage of allowing immediate intervention if an abnormality is identified, aortography is an invasive procedure that is now typically performed for treatment after diagnosis of a new or worsening aortic pathology. CT Chest, Abdomen, and Pelvis With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis for the follow-up of open repair of TAAA or dissection because most follow-up imaging uses an arterially timed contrast bolus in the form of a CTA (discussed below).

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. US Duplex Doppler Aorta Abdomen Duplex US of the abdominal aorta carries the advantages of being a readily obtainable bedside examination for evaluation of the distal thoracic and abdominal aorta. After EVAR, duplex US can be used to evaluate aortic diameter and presence of endoleak, with a good correlation of aortic size with CTA in most patients [76,91]. US Echocardiography Transthoracic Resting TTE allows visualization of the heart and portions of the thoracic aorta. However, portions of the proximal descending thoracic aorta may be poorly visualized with TTE, and examination may be limited by poor acoustic windows, creating the possibility of false-negative examinations in following patients after endovascular thoracoabdominal aortic repair [58]. These characteristics have resulted in a low sensitivity of 31% to 55% for diagnosis of acute type B aortic dissection on TTE [33]. TTE is also limited by the ability to visualize the abdominal aorta to determine the extent of thoracoabdominal aortic pathology. Variant 4: Follow-up after open repair of thoracoabdominal aortic aneurysm or dissection. Aortography Chest Abdomen Pelvis There is no relevant literature regarding the use of aortography in the evaluation of follow-up of open repair of TAAA or dissection. Aortogram with DSA for evaluation of the thoracic and abdominal aorta has a sensitivity of up to 90% and a specificity of 95% for acute aortic syndrome [27]. Although it has the advantage of allowing immediate intervention if an abnormality is identified, aortography is an invasive procedure that is now typically performed for treatment after diagnosis of a new or worsening aortic pathology. CT Chest, Abdomen, and Pelvis With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis for the follow-up of open repair of TAAA or dissection because most follow-up imaging uses an arterially timed contrast bolus in the form of a CTA (discussed below).

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology and in identifying postsurgical complications. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without and With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis with noncontrast phase for the follow-up of open repair of TAAA or dissection because most follow-up imaging uses an arterially timed contrast bolus in the form of a CTA (discussed below). However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology and in identifying postsurgical complications. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without IV Contrast There is no relevant literature for CT chest, abdomen, and pelvis without IV contrast in the evaluation of follow-up of open repair of TAAA or dissection. Although most follow-up after open thoracoabdominal aortic repair is performed with IV contrast [29-31], select groups of patients may be monitored with noncontrast CT to evaluate measurements of aortic size and for surrounding changes that could suggest inflammation or infection. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. Thoracoabdominal Aortic Aneurysm or Dissection CT Chest and Abdomen With IV Contrast There is no relevant literature regarding venous phase CT chest and abdomen for the follow-up of open repair of TAAA or dissection. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis may fail to detect changing or new pathology in the pelvic vasculature.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology and in identifying postsurgical complications. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without and With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis with noncontrast phase for the follow-up of open repair of TAAA or dissection because most follow-up imaging uses an arterially timed contrast bolus in the form of a CTA (discussed below). However, contrast-enhanced CT can provide similar information as CTA in evaluating the size and extent of aortic pathology and in identifying postsurgical complications. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest, Abdomen, and Pelvis Without IV Contrast There is no relevant literature for CT chest, abdomen, and pelvis without IV contrast in the evaluation of follow-up of open repair of TAAA or dissection. Although most follow-up after open thoracoabdominal aortic repair is performed with IV contrast [29-31], select groups of patients may be monitored with noncontrast CT to evaluate measurements of aortic size and for surrounding changes that could suggest inflammation or infection. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. Thoracoabdominal Aortic Aneurysm or Dissection CT Chest and Abdomen With IV Contrast There is no relevant literature regarding venous phase CT chest and abdomen for the follow-up of open repair of TAAA or dissection. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis may fail to detect changing or new pathology in the pelvic vasculature.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without and With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis with noncontrast phase for the follow-up of open repair of TAAA or dissection. Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without IV Contrast There is no relevant literature for CT chest and abdomen without IV contrast in the evaluation of follow-up of open repair of TAAA or dissection. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CTA Chest, Abdomen, and Pelvis With IV Contrast CTA of the chest, abdomen, and pelvis with IV contrast uses fast scan times to allow for rapid evaluation of the aorta. Acquisition of thin axial slices with subsequent reconstruction along with extensive and homogenous luminal opacification allows for precise and reproducible measurements of the aorta, which are valuable for monitoring aortic growth and interval changes [33,34]. In addition to monitoring aortic size and postsurgical complications such as anastomotic pseudoaneurysm, CTA can evaluate for endograft complications, kinking or occlusion/stenosis of branches of the graft, infection, or complications including thoracoabdominal aneurysm rupture or dissection extension [31,35]. Evidence comparing CTA with MRA for follow-up after surgical thoracoabdominal aortic repair is limited.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without and With IV Contrast There is no relevant literature regarding venous phase CT chest, abdomen, and pelvis with noncontrast phase for the follow-up of open repair of TAAA or dissection. Compared with CT of the chest, abdomen, and pelvis, evaluation of the chest and abdomen only without imaging of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CT Chest and Abdomen Without IV Contrast There is no relevant literature for CT chest and abdomen without IV contrast in the evaluation of follow-up of open repair of TAAA or dissection. Compared with CT of the chest, abdomen, and pelvis, exclusion of the pelvis may fail to detect changing or new pathology in the pelvic vasculature. If venous phase or unenhanced CT has already been performed, additional imaging with CTA may not be required in some patients. CTA Chest, Abdomen, and Pelvis With IV Contrast CTA of the chest, abdomen, and pelvis with IV contrast uses fast scan times to allow for rapid evaluation of the aorta. Acquisition of thin axial slices with subsequent reconstruction along with extensive and homogenous luminal opacification allows for precise and reproducible measurements of the aorta, which are valuable for monitoring aortic growth and interval changes [33,34]. In addition to monitoring aortic size and postsurgical complications such as anastomotic pseudoaneurysm, CTA can evaluate for endograft complications, kinking or occlusion/stenosis of branches of the graft, infection, or complications including thoracoabdominal aneurysm rupture or dissection extension [31,35]. Evidence comparing CTA with MRA for follow-up after surgical thoracoabdominal aortic repair is limited.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

A 2015 review of imaging of the thoracic aorta concluded that contrast-enhanced CT was the optimal modality evaluation of the aorta after surgical repair, although MRI is also comparable and with image resolution comparable to CTA [33]. Compared with CTA of the chest and abdomen, imaging of the pelvis carries the benefit of evaluation of the iliofemoral vessels to assess for new or worsening pathology of the pelvic vessels. CTA Chest and Abdomen With IV Contrast Compared with CTA of the chest, abdomen, and pelvis, CTA of the chest and abdomen may fail to identify new or worsening pelvic pathology. MRA Chest, Abdomen, and Pelvis Without and With IV Contrast MRA of the chest, abdomen, and pelvis without and with IV contrast can be used as an alternative to CTA for follow-up after open thoracoabdominal aortic repair. Spatial resolution of MRA is not as high as CTA, although some authors have found MRI to be an alternative option for imaging in younger patients [33]. In addition to monitoring aortic size and postsurgical complication such as anastomotic pseudoaneurysm, CTA can evaluate for graft complications, kinking or occlusion/stenosis of branches of the graft, infection, or complications including thoracoabdominal aneurysm rupture or dissection extension. MRA can also assess flow dynamics including wall stress and turbulent flow patterns, which may be valuable in certain patients [23,24]. Compared with MRA of the chest and abdomen, imaging of the pelvis carries the benefit of evaluation of the iliofemoral vessels to assess for new or worsening pathology of the pelvic vessels. The drawback of inclusion of the pelvis is increased acquisition time. MRA Chest, Abdomen, and Pelvis Without IV Contrast Evidence supporting use of MRA without IV contrast for evaluation after open thoracoabdominal aortic repair is limited compared with evidence of MRA with IV contrast.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. A 2015 review of imaging of the thoracic aorta concluded that contrast-enhanced CT was the optimal modality evaluation of the aorta after surgical repair, although MRI is also comparable and with image resolution comparable to CTA [33]. Compared with CTA of the chest and abdomen, imaging of the pelvis carries the benefit of evaluation of the iliofemoral vessels to assess for new or worsening pathology of the pelvic vessels. CTA Chest and Abdomen With IV Contrast Compared with CTA of the chest, abdomen, and pelvis, CTA of the chest and abdomen may fail to identify new or worsening pelvic pathology. MRA Chest, Abdomen, and Pelvis Without and With IV Contrast MRA of the chest, abdomen, and pelvis without and with IV contrast can be used as an alternative to CTA for follow-up after open thoracoabdominal aortic repair. Spatial resolution of MRA is not as high as CTA, although some authors have found MRI to be an alternative option for imaging in younger patients [33]. In addition to monitoring aortic size and postsurgical complication such as anastomotic pseudoaneurysm, CTA can evaluate for graft complications, kinking or occlusion/stenosis of branches of the graft, infection, or complications including thoracoabdominal aneurysm rupture or dissection extension. MRA can also assess flow dynamics including wall stress and turbulent flow patterns, which may be valuable in certain patients [23,24]. Compared with MRA of the chest and abdomen, imaging of the pelvis carries the benefit of evaluation of the iliofemoral vessels to assess for new or worsening pathology of the pelvic vessels. The drawback of inclusion of the pelvis is increased acquisition time. MRA Chest, Abdomen, and Pelvis Without IV Contrast Evidence supporting use of MRA without IV contrast for evaluation after open thoracoabdominal aortic repair is limited compared with evidence of MRA with IV contrast.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

Similar to CTA, MRA of the thoracoabdominal aorta allows for precise and reproducible assessment of aortic sac size in aneurysm or extent of dissection in thoracoabdominal aortic dissection [33,37-39]. Multiple unenhanced Thoracoabdominal Aortic Aneurysm or Dissection MRA techniques such as time-of-flight, phase-contrast imaging, and SSFP have been developed that allow for evaluation of aortic dissection and aneurysm, including after open surgical repair [44,45]. Even without IV contrast, MRA can be used to precisely evaluate the thoracoabdominal aorta [37,39]. A 2017 observational study comparing AAA measurements in contrast-enhanced CTA and noncontrast-enhanced MRA showed strong agreement, with intraclass coefficient >0.99 and interobserver reproducibility >0.99 for both CTA and MRA [45]. Although some studies have shown more accurate measurements with contrast-enhanced MRA compared with noncontrast-enhanced MRA, other studies have shown equal ability to detect aortic pathology and to measure aortic size [44,50,51]. A 2014 study comparing thoracic aortic measurements and pathology in 76 patients undergoing both contrast- and noncontrast-enhanced MRA showed high agreement between study types, with low intra- and interobserver dependency (intraclass correlation coefficient 0.99) [51]. A similar 2017 study comparing thoracic aorta measurements/findings on contrast- and noncontrast-enhanced MRA performed on a group of 50 patients favored noncontrast-enhanced MRA over contrast-enhanced MRA as the technique of choice because of superior image quality and better vessel sharpness in the ascending aorta [44]. Although these studies do not focus on postsurgical patients, the findings are likely applicable to this patient population.

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. Similar to CTA, MRA of the thoracoabdominal aorta allows for precise and reproducible assessment of aortic sac size in aneurysm or extent of dissection in thoracoabdominal aortic dissection [33,37-39]. Multiple unenhanced Thoracoabdominal Aortic Aneurysm or Dissection MRA techniques such as time-of-flight, phase-contrast imaging, and SSFP have been developed that allow for evaluation of aortic dissection and aneurysm, including after open surgical repair [44,45]. Even without IV contrast, MRA can be used to precisely evaluate the thoracoabdominal aorta [37,39]. A 2017 observational study comparing AAA measurements in contrast-enhanced CTA and noncontrast-enhanced MRA showed strong agreement, with intraclass coefficient >0.99 and interobserver reproducibility >0.99 for both CTA and MRA [45]. Although some studies have shown more accurate measurements with contrast-enhanced MRA compared with noncontrast-enhanced MRA, other studies have shown equal ability to detect aortic pathology and to measure aortic size [44,50,51]. A 2014 study comparing thoracic aortic measurements and pathology in 76 patients undergoing both contrast- and noncontrast-enhanced MRA showed high agreement between study types, with low intra- and interobserver dependency (intraclass correlation coefficient 0.99) [51]. A similar 2017 study comparing thoracic aorta measurements/findings on contrast- and noncontrast-enhanced MRA performed on a group of 50 patients favored noncontrast-enhanced MRA over contrast-enhanced MRA as the technique of choice because of superior image quality and better vessel sharpness in the ascending aorta [44]. Although these studies do not focus on postsurgical patients, the findings are likely applicable to this patient population.

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Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up

MRA Chest and Abdomen Without and With IV Contrast Compared with MRA of the chest, abdomen, and pelvis, imaging of the chest and abdomen reduces acquisition time, although is unable to assess for new or worsening pathology within the pelvis. MRA Chest and Abdomen Without IV Contrast Compared with MRA of the chest, abdomen, and pelvis, imaging of the chest and abdomen reduces acquisition time, although is unable to assess for new or worsening pathology within the pelvis. Radiography Chest There is no relevant literature for radiography in the evaluation of follow-up of open repair of TAAA or dissection. After surgical repair of the thoracoabdominal aorta, radiography is unlikely to carry a sufficient sensitivity or specificity to be used routinely. Radiography Chest Abdomen Pelvis There is no relevant literature for radiography in the evaluation of follow-up of open repair of TAAA or dissection. After surgical repair of the thoracoabdominal aorta, radiography is unlikely to carry a sufficient sensitivity or specificity to be used routinely. US Duplex Doppler Aorta Abdomen Duplex US of the abdominal aorta is an option for evaluation of the thoracoabdominal aorta, although the ability to evaluate the aorta above the diaphragm may be markedly limited by acoustic windows. Prior studies comparing US, CT, and MRI of the abdominal aorta found that US is a reliable method to diagnose and follow AAAs [56]. In the evaluation of thoracoabdominal aortic dissection, US can also be used to evaluate blood flow in the true and false lumens and to directly and dynamically monitor the motion of dissection flaps [43]. As such, abdomen US can be performed serially to evaluate for aortic size changes or dissection hemodynamic changes. However, a limited ability to evaluate the thoracic aorta with dependence on patient acoustic windows could result in poor image quality or the inability to view changes to the aorta. Thoracoabdominal Aortic Aneurysm or Dissection

Thoracoabdominal Aneurysm or Dissection Treatment Planning and Follow Up. MRA Chest and Abdomen Without and With IV Contrast Compared with MRA of the chest, abdomen, and pelvis, imaging of the chest and abdomen reduces acquisition time, although is unable to assess for new or worsening pathology within the pelvis. MRA Chest and Abdomen Without IV Contrast Compared with MRA of the chest, abdomen, and pelvis, imaging of the chest and abdomen reduces acquisition time, although is unable to assess for new or worsening pathology within the pelvis. Radiography Chest There is no relevant literature for radiography in the evaluation of follow-up of open repair of TAAA or dissection. After surgical repair of the thoracoabdominal aorta, radiography is unlikely to carry a sufficient sensitivity or specificity to be used routinely. Radiography Chest Abdomen Pelvis There is no relevant literature for radiography in the evaluation of follow-up of open repair of TAAA or dissection. After surgical repair of the thoracoabdominal aorta, radiography is unlikely to carry a sufficient sensitivity or specificity to be used routinely. US Duplex Doppler Aorta Abdomen Duplex US of the abdominal aorta is an option for evaluation of the thoracoabdominal aorta, although the ability to evaluate the aorta above the diaphragm may be markedly limited by acoustic windows. Prior studies comparing US, CT, and MRI of the abdominal aorta found that US is a reliable method to diagnose and follow AAAs [56]. In the evaluation of thoracoabdominal aortic dissection, US can also be used to evaluate blood flow in the true and false lumens and to directly and dynamically monitor the motion of dissection flaps [43]. As such, abdomen US can be performed serially to evaluate for aortic size changes or dissection hemodynamic changes. However, a limited ability to evaluate the thoracic aorta with dependence on patient acoustic windows could result in poor image quality or the inability to view changes to the aorta. Thoracoabdominal Aortic Aneurysm or Dissection

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acrac_3148734_0

Suspected Spine Infection PCAs

Introduction/Background Spine infection is a disease that occurs when either microorganisms or viruses invade and involve one or more structures within or surrounding the spine [1-7]. Although uncommon, the incidence of spine infection appears to be increasing because of a combination of predisposing factors, such as an increasing number of susceptible hosts, an increase in the number of interventional and surgical spine procedures, and an increase in diagnostic testing [5,8- 11]. Potential host factors include preexisting extraspinal infection (endocarditis, HIV, pulmonary infection), intravenous (IV) drug use, diabetes mellitus, hepatic or renal failure, rheumatologic disease, or immunosuppression [12,13]. Spine infection presents a diagnostic and management challenge [14]. Diagnostic delay is not uncommon because of an often indolent clinical presentation with nonspecific presenting signs and symptoms such as back pain, fever, and, less commonly, neurologic compromise [3,8,10]. The location of the spine infection is also important because it may influence the clinical presentation and the subsequent imaging evaluation. One or more spine structures and/or compartments may be infected, and this also influences the imaging findings [1,14]. Spine infection is often extradural, initially invading the vertebral endplate in adults via a hematogenous route and centering about the vertebral endplate (osteomyelitis) and intervertebral disc (discitis). Spine infection may also arise initially within a facet joint. Spine infection occurs less frequently in children and initially affects the intervertebral disc [15]. Epidural and paraspinal soft tissue involvement are not uncommon [16,17]. Other important clinical manifestations of spine infection with imaging and management implications include epidural (abscess), subarachnoid (meningitis) space involvement, or spinal cord involvement (myelitis) [18,19].

Suspected Spine Infection PCAs. Introduction/Background Spine infection is a disease that occurs when either microorganisms or viruses invade and involve one or more structures within or surrounding the spine [1-7]. Although uncommon, the incidence of spine infection appears to be increasing because of a combination of predisposing factors, such as an increasing number of susceptible hosts, an increase in the number of interventional and surgical spine procedures, and an increase in diagnostic testing [5,8- 11]. Potential host factors include preexisting extraspinal infection (endocarditis, HIV, pulmonary infection), intravenous (IV) drug use, diabetes mellitus, hepatic or renal failure, rheumatologic disease, or immunosuppression [12,13]. Spine infection presents a diagnostic and management challenge [14]. Diagnostic delay is not uncommon because of an often indolent clinical presentation with nonspecific presenting signs and symptoms such as back pain, fever, and, less commonly, neurologic compromise [3,8,10]. The location of the spine infection is also important because it may influence the clinical presentation and the subsequent imaging evaluation. One or more spine structures and/or compartments may be infected, and this also influences the imaging findings [1,14]. Spine infection is often extradural, initially invading the vertebral endplate in adults via a hematogenous route and centering about the vertebral endplate (osteomyelitis) and intervertebral disc (discitis). Spine infection may also arise initially within a facet joint. Spine infection occurs less frequently in children and initially affects the intervertebral disc [15]. Epidural and paraspinal soft tissue involvement are not uncommon [16,17]. Other important clinical manifestations of spine infection with imaging and management implications include epidural (abscess), subarachnoid (meningitis) space involvement, or spinal cord involvement (myelitis) [18,19].

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Suspected Spine Infection PCAs

Multilevel or multifocal spine infection may be observed in specific patient groups, such as IV drug users, postoperative spine patients, or in geographic regions with endemic infections such as tuberculosis, coccidioidomycosis, or neurocysticercosis [5,20-22]. The type of infection, whether pyogenic, granulomatous, parasitic, or viral, will likewise influence the clinical and imaging presentation [2,4,5,7,23-26]. Imaging is important for suggesting the diagnosis of spine infection, guiding percutaneous spine biopsy procedures, defining the full extent of infection for the purposes of determining medical and/or surgical management, and for possible clinical follow-up [27-31]. Diagnostic imaging can be used to assess suspected spinal cord compression as well as to evaluate for potential spinal instability, either of which, if present will influence surgical intervention [8,32]. Several diagnostic imaging examinations have been previously utilized in the evaluation and management of spine infection and include radiography, CT, nuclear scintigraphy with various radionuclides, and MRI [20,31,33-37]. Recently, PET/CT has seen increasing application in the evaluation of suspected spine infection, particularly in the postoperative spine [38-45]. PET/MRI is undergoing preliminary investigation for possible use in spine infection [46]. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization. Reprint requests to: [email protected] Suspected Spine Infection Although imaging studies have a role in the diagnostic evaluation of suspected spine infection, a high index of clinical suspicion for an infectious etiology is required in order to initiate the clinical workup [47].

Suspected Spine Infection PCAs. Multilevel or multifocal spine infection may be observed in specific patient groups, such as IV drug users, postoperative spine patients, or in geographic regions with endemic infections such as tuberculosis, coccidioidomycosis, or neurocysticercosis [5,20-22]. The type of infection, whether pyogenic, granulomatous, parasitic, or viral, will likewise influence the clinical and imaging presentation [2,4,5,7,23-26]. Imaging is important for suggesting the diagnosis of spine infection, guiding percutaneous spine biopsy procedures, defining the full extent of infection for the purposes of determining medical and/or surgical management, and for possible clinical follow-up [27-31]. Diagnostic imaging can be used to assess suspected spinal cord compression as well as to evaluate for potential spinal instability, either of which, if present will influence surgical intervention [8,32]. Several diagnostic imaging examinations have been previously utilized in the evaluation and management of spine infection and include radiography, CT, nuclear scintigraphy with various radionuclides, and MRI [20,31,33-37]. Recently, PET/CT has seen increasing application in the evaluation of suspected spine infection, particularly in the postoperative spine [38-45]. PET/MRI is undergoing preliminary investigation for possible use in spine infection [46]. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization. Reprint requests to: [email protected] Suspected Spine Infection Although imaging studies have a role in the diagnostic evaluation of suspected spine infection, a high index of clinical suspicion for an infectious etiology is required in order to initiate the clinical workup [47].

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Suspected Spine Infection PCAs

Important laboratory parameters that may be assessed include serum erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cell (WBC) count with differential, and blood cultures [37,48-50]. Other testing such as for brucella or mycobacterium may be helpful if the patient is from the appropriate endemic area [2,4,5,7]. Because the imaging appearance of spine infection may overlap with other noninfectious pathologic entities, such as degenerative disc disease, inflammation, trauma, or neoplasm, spine biopsy with microbiologic and histopathologic analysis of the infected tissue is often required for diagnostic confirmation [35,51-58]. Special Imaging Considerations Although the clinical presentation and physical examination can help localize the level of suspected spine infection, in specific clinical situations it may be beneficial to image the entire spine [21,59]. This may be influenced by clinical presentation and patient factors such as a history of IV drug use, specific pathogens such as tuberculosis, or initial imaging findings that demonstrate multilevel spine involvement [21,59-61]. Like other spine infections, spinal epidural abscess has increased in incidence and is now seen in 2.5 to 3/10,000 patients [3,60]. Epidural abscess is often associated with diagnostic delay that can potentially lead to significant neurologic morbidity and mortality. Patients with preexisting risk factors, such as having a potential source for infection (preexisting infection, IV drug use, recent spine procedure) or a reason for being immunosuppressed (diabetes, steroid use) and patients with an elevated ESR, may be at increased risk for epidural abscess [18,21,22,62].

Suspected Spine Infection PCAs. Important laboratory parameters that may be assessed include serum erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cell (WBC) count with differential, and blood cultures [37,48-50]. Other testing such as for brucella or mycobacterium may be helpful if the patient is from the appropriate endemic area [2,4,5,7]. Because the imaging appearance of spine infection may overlap with other noninfectious pathologic entities, such as degenerative disc disease, inflammation, trauma, or neoplasm, spine biopsy with microbiologic and histopathologic analysis of the infected tissue is often required for diagnostic confirmation [35,51-58]. Special Imaging Considerations Although the clinical presentation and physical examination can help localize the level of suspected spine infection, in specific clinical situations it may be beneficial to image the entire spine [21,59]. This may be influenced by clinical presentation and patient factors such as a history of IV drug use, specific pathogens such as tuberculosis, or initial imaging findings that demonstrate multilevel spine involvement [21,59-61]. Like other spine infections, spinal epidural abscess has increased in incidence and is now seen in 2.5 to 3/10,000 patients [3,60]. Epidural abscess is often associated with diagnostic delay that can potentially lead to significant neurologic morbidity and mortality. Patients with preexisting risk factors, such as having a potential source for infection (preexisting infection, IV drug use, recent spine procedure) or a reason for being immunosuppressed (diabetes, steroid use) and patients with an elevated ESR, may be at increased risk for epidural abscess [18,21,22,62].

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Suspected Spine Infection PCAs

OR Discussion of Procedures by Variant Variant 1: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with new or worsening back or neck pain, with or without fever, who may have one or more of the following red flags (diabetes mellitus, IV drug use, cancer, HIV, or dialysis) or abnormal lab values. Initial imaging. The annual incidence of spine infection ranges from 4 to 24 per million per year [10]. In the presence of red flag conditions (diabetes mellitus, IV drug use, cancer, HIV, or dialysis) or abnormal lab values, imaging may be indicated if there is a clinical suspicion for spine infection in a patient with neck or back pain with or without fever. Clinically, it may be difficult to differentiate spine infection from other causes of neck or back pain such as degenerative disease, trauma, inflammatory spondyloarthropathy, or neoplastic involvement of the spine [3,35,55]. As any one of these clinical entities has the potential to mimic the imaging appearance of spine infection, it is important to use the combination of clinical presentation, laboratory values such as an elevated ESR and CRP and imaging findings in order to consider the diagnosis of spine infection [8]. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. CT Spine Area of Interest As a result of its excellent delineation of osseous detail and greater sensitivity than radiography, CT can be used in the evaluation of spine infection [3]. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection.

Suspected Spine Infection PCAs. OR Discussion of Procedures by Variant Variant 1: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with new or worsening back or neck pain, with or without fever, who may have one or more of the following red flags (diabetes mellitus, IV drug use, cancer, HIV, or dialysis) or abnormal lab values. Initial imaging. The annual incidence of spine infection ranges from 4 to 24 per million per year [10]. In the presence of red flag conditions (diabetes mellitus, IV drug use, cancer, HIV, or dialysis) or abnormal lab values, imaging may be indicated if there is a clinical suspicion for spine infection in a patient with neck or back pain with or without fever. Clinically, it may be difficult to differentiate spine infection from other causes of neck or back pain such as degenerative disease, trauma, inflammatory spondyloarthropathy, or neoplastic involvement of the spine [3,35,55]. As any one of these clinical entities has the potential to mimic the imaging appearance of spine infection, it is important to use the combination of clinical presentation, laboratory values such as an elevated ESR and CRP and imaging findings in order to consider the diagnosis of spine infection [8]. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. CT Spine Area of Interest As a result of its excellent delineation of osseous detail and greater sensitivity than radiography, CT can be used in the evaluation of spine infection [3]. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection.

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acrac_3148734_4

Suspected Spine Infection PCAs

In those cases in which a contrast- enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively [33]. CT has low sensitivity (6%) for the identification of epidural abscess Suspected Spine Infection [33]. CT is often utilized to evaluate suspected spine infection when MRI is equivocal [3]. It is also of value in presurgical planning for patients with suspected infection-related spine instability, cord compression, as well as follow-up evaluation of the instrumented spine [30]. CT is often used to facilitate percutaneous image-guided spine biopsy [52,57]. Radiography Spine Area of Interest Radiography can be used as part of the initial evaluation in patients with suspected spine infection. Radiographs may not show any abnormalities during the early course of spine infection [3]. Imaging findings such as disc space narrowing, vertebral endplate erosion, and gross paraspinal soft tissue changes that can be seen on radiography lag behind the clinical course of spine infection by at least 2 to 8 weeks [3,10,31]. Nevertheless, the possible presence of one or more of these findings may increase the clinical suspicion for infection and may help guide subsequent imaging management. Radiographs, however, provide an overall view of the status and alignment of the vertebral column and can be used to assess for spinal instability [8]. 3-Phase Bone Scan Complete Spine A 3-phase bone scan with Tc-99m-methylene diphosphonate (MDP) has variable moderate-to-high sensitivity (81.4%) and low specificity (40.7%) for spine infection [31]. The advantages of skeletal scintigraphy include that it can be performed and completed in 1 day. Because of the imaging time required for a 3-phase bone scan, it tends to be utilized in select situations [3].

Suspected Spine Infection PCAs. In those cases in which a contrast- enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively [33]. CT has low sensitivity (6%) for the identification of epidural abscess Suspected Spine Infection [33]. CT is often utilized to evaluate suspected spine infection when MRI is equivocal [3]. It is also of value in presurgical planning for patients with suspected infection-related spine instability, cord compression, as well as follow-up evaluation of the instrumented spine [30]. CT is often used to facilitate percutaneous image-guided spine biopsy [52,57]. Radiography Spine Area of Interest Radiography can be used as part of the initial evaluation in patients with suspected spine infection. Radiographs may not show any abnormalities during the early course of spine infection [3]. Imaging findings such as disc space narrowing, vertebral endplate erosion, and gross paraspinal soft tissue changes that can be seen on radiography lag behind the clinical course of spine infection by at least 2 to 8 weeks [3,10,31]. Nevertheless, the possible presence of one or more of these findings may increase the clinical suspicion for infection and may help guide subsequent imaging management. Radiographs, however, provide an overall view of the status and alignment of the vertebral column and can be used to assess for spinal instability [8]. 3-Phase Bone Scan Complete Spine A 3-phase bone scan with Tc-99m-methylene diphosphonate (MDP) has variable moderate-to-high sensitivity (81.4%) and low specificity (40.7%) for spine infection [31]. The advantages of skeletal scintigraphy include that it can be performed and completed in 1 day. Because of the imaging time required for a 3-phase bone scan, it tends to be utilized in select situations [3].

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acrac_3148734_5

Suspected Spine Infection PCAs

Gallium Scan Whole Body Ga-67 scintigraphy combined with single-photon emission computed tomography (SPECT) can be used to evaluate suspected spine infection. Ga-67 is less sensitive (73%) but more specific (61%) than skeletal scintigraphy [31]. The disadvantages of the gallium examination include a requirement for delayed images (24 to 72 hours). [31]. A Tc-99m-MDP study can be combined with Ga-67-citrate in order to improve the overall specificity of the examination (81%) while maintaining a sensitivity of 78% [31,36]. This combined examination may be utilized in select clinical situations such as when multifocal infection is suspected [3]. WBC Scan and Sulfur Colloid Scan Complete Spine A labeled leukocyte and sulfur colloid study is limited in the evaluation of spine infection as areas of infection often demonstrate decreased or absent radionuclide uptake [31]. Suspected Spine Infection Variant 2: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with recent intervention (such as surgery with or without hardware, pain injection, or stimulator implantation). Initial imaging. The mean incidence of postoperative instrumented spine infection is approximately 2% to 3% [40]. The diagnosis of postintervention spine infection is a clinical challenge given an overlap of clinical symptoms such as neck or back pain between postoperative and spine infection patients. The identification of abnormal laboratory parameters, such as leukocytosis or elevated ESR or CRP, may increase the clinical suspicion for spine infection in the postintervention patient [8,9]. The timing of the imaging examination with respect to when the spine intervention is performed is particularly important, because expected findings such as alteration of soft tissue and osseous structures, edema, and small paraspinal fluid collections such as seromas may represent the normal sequelae of an intervention shortly (a few days to weeks) after the procedure [28,29,48,69].

Suspected Spine Infection PCAs. Gallium Scan Whole Body Ga-67 scintigraphy combined with single-photon emission computed tomography (SPECT) can be used to evaluate suspected spine infection. Ga-67 is less sensitive (73%) but more specific (61%) than skeletal scintigraphy [31]. The disadvantages of the gallium examination include a requirement for delayed images (24 to 72 hours). [31]. A Tc-99m-MDP study can be combined with Ga-67-citrate in order to improve the overall specificity of the examination (81%) while maintaining a sensitivity of 78% [31,36]. This combined examination may be utilized in select clinical situations such as when multifocal infection is suspected [3]. WBC Scan and Sulfur Colloid Scan Complete Spine A labeled leukocyte and sulfur colloid study is limited in the evaluation of spine infection as areas of infection often demonstrate decreased or absent radionuclide uptake [31]. Suspected Spine Infection Variant 2: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with recent intervention (such as surgery with or without hardware, pain injection, or stimulator implantation). Initial imaging. The mean incidence of postoperative instrumented spine infection is approximately 2% to 3% [40]. The diagnosis of postintervention spine infection is a clinical challenge given an overlap of clinical symptoms such as neck or back pain between postoperative and spine infection patients. The identification of abnormal laboratory parameters, such as leukocytosis or elevated ESR or CRP, may increase the clinical suspicion for spine infection in the postintervention patient [8,9]. The timing of the imaging examination with respect to when the spine intervention is performed is particularly important, because expected findings such as alteration of soft tissue and osseous structures, edema, and small paraspinal fluid collections such as seromas may represent the normal sequelae of an intervention shortly (a few days to weeks) after the procedure [28,29,48,69].

3148734

acrac_3148734_6

Suspected Spine Infection PCAs

The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. CT Spine Area of Interest CT may be used to assess the spine for suspected infection, particularly following any surgical or interventional procedure, without or with spinal implants [3,30]. When initially considering the diagnosis of spine infection, it is important to use a combination of clinical presentation in the context of these red flags, abnormal laboratory values such as elevated ESR and CRP or leukocytosis, and abnormal imaging findings [8]. In the postoperative spine, CT may show implant loosening or malpositioning as well as malalignment and imaging findings that may be caused by infection. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection. In those cases in which a contrast-enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively, but CT has low sensitivity (6%) for the identification of epidural abscess [33]. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast has a sensitivity of 96% and a specificity of 94% for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI without and with IV contrast is often utilized for the evaluation of patients who have undergone recent spine interventions and have suspected spine infection [3,7,14,16,24,37,51]. Artifact reduction techniques are often required in patients who have spinal instrumentation. MRI also provides optimal depiction of

Suspected Spine Infection PCAs. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. CT Spine Area of Interest CT may be used to assess the spine for suspected infection, particularly following any surgical or interventional procedure, without or with spinal implants [3,30]. When initially considering the diagnosis of spine infection, it is important to use a combination of clinical presentation in the context of these red flags, abnormal laboratory values such as elevated ESR and CRP or leukocytosis, and abnormal imaging findings [8]. In the postoperative spine, CT may show implant loosening or malpositioning as well as malalignment and imaging findings that may be caused by infection. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection. In those cases in which a contrast-enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively, but CT has low sensitivity (6%) for the identification of epidural abscess [33]. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast has a sensitivity of 96% and a specificity of 94% for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI without and with IV contrast is often utilized for the evaluation of patients who have undergone recent spine interventions and have suspected spine infection [3,7,14,16,24,37,51]. Artifact reduction techniques are often required in patients who have spinal instrumentation. MRI also provides optimal depiction of

3148734

acrac_3148734_7

Suspected Spine Infection PCAs

Suspected Spine Infection Radiography Spine Area of Interest Radiographs are insensitive during the early course of spine infection [3]. In the subacute or chronic phase of infection, radiographs can be helpful in the follow-up evaluation of the posttreatment spine because serial radiographic studies may show new abnormalities such as implant loosening or alteration in spinal alignment that might be caused by infection [10]. 3-Phase Bone Scan A 3-phase bone scan with Tc-99m-MDP has variable moderate-to-high sensitivity (81.4%) and low specificity (40.7%) for spine infection [31]. Gallium Scan Whole Body Ga-67 scintigraphy combined with SPECT can be used to evaluate suspected spine infection in patients who have undergone recent spine interventions. Ga-67 is less sensitive (73%) but more specific (61%) than skeletal scintigraphy [31]. A dual Ga-67 and Tc-99m-MDP study can increase the overall specificity of the examination to 81% with a sensitivity of 73% [3,10,31,36]. This combined study can be used to assess the postoperative or postprocedure spine in cases of suspected spine infection when MRI imaging findings are equivocal [3,10]. WBC Scan and Sulfur Colloid Scan Complete Spine A labeled leukocyte and sulfur colloid study is limited in the evaluation of spine infection because areas of infection often demonstrate decreased or absent radionuclide uptake [31]. FDG-PET/CT Whole Body FDG-PET with CT has seen increasing application for the assessment of suspected spine infection in select cases as a complementary examination [10,31]. Increased FDG uptake is seen at sites of infection with an elevated SUVmax value. FDG-PET/CT can be used in the evaluation of the postsurgical or postprocedure spine for suspected infection when the MRI examination is inconclusive. Initial studies with FDG-PET/CT have shown the utility of this study in the initial evaluation of potentially infected spinal implants in selected patients [40].

Suspected Spine Infection PCAs. Suspected Spine Infection Radiography Spine Area of Interest Radiographs are insensitive during the early course of spine infection [3]. In the subacute or chronic phase of infection, radiographs can be helpful in the follow-up evaluation of the posttreatment spine because serial radiographic studies may show new abnormalities such as implant loosening or alteration in spinal alignment that might be caused by infection [10]. 3-Phase Bone Scan A 3-phase bone scan with Tc-99m-MDP has variable moderate-to-high sensitivity (81.4%) and low specificity (40.7%) for spine infection [31]. Gallium Scan Whole Body Ga-67 scintigraphy combined with SPECT can be used to evaluate suspected spine infection in patients who have undergone recent spine interventions. Ga-67 is less sensitive (73%) but more specific (61%) than skeletal scintigraphy [31]. A dual Ga-67 and Tc-99m-MDP study can increase the overall specificity of the examination to 81% with a sensitivity of 73% [3,10,31,36]. This combined study can be used to assess the postoperative or postprocedure spine in cases of suspected spine infection when MRI imaging findings are equivocal [3,10]. WBC Scan and Sulfur Colloid Scan Complete Spine A labeled leukocyte and sulfur colloid study is limited in the evaluation of spine infection because areas of infection often demonstrate decreased or absent radionuclide uptake [31]. FDG-PET/CT Whole Body FDG-PET with CT has seen increasing application for the assessment of suspected spine infection in select cases as a complementary examination [10,31]. Increased FDG uptake is seen at sites of infection with an elevated SUVmax value. FDG-PET/CT can be used in the evaluation of the postsurgical or postprocedure spine for suspected infection when the MRI examination is inconclusive. Initial studies with FDG-PET/CT have shown the utility of this study in the initial evaluation of potentially infected spinal implants in selected patients [40].

3148734

acrac_3148734_8

Suspected Spine Infection PCAs

Variant 3: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with new neurologic deficit or cauda equina syndrome. Initial imaging. The presence of a new neurologic deficit or cauda equina syndrome may be due to spinal cord or cauda equina compromise by either epidural abscess, displaced infected vertebral and/or disc material, or infection-mediated spinal malalignment or instability. The incidence of epidural abscess is 2.5 to 3 per 10,000 hospital admissions [14,60]. Although neurologic deficits are seen in 10% to 15% of cases of spine infection, these clinical situations require immediate imaging attention because the imaging evaluation helps to determine the location and extent of the spinal canal compromise [3,60]. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. Suspected Spine Infection CT Spine Area of Interest Noncontrast and contrast-enhanced CT have an overall low sensitivity (6%) for the identification of epidural abscess [33]. Gross spinal cord compression with compromise of the spinal canal (>50% canal narrowing) may be seen in more advanced cases of spine infection [71]. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection. In those cases in which a contrast-enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively [33]. CT with multiplanar reformations is often used in surgical planning and follow-up [8].

Suspected Spine Infection PCAs. Variant 3: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with new neurologic deficit or cauda equina syndrome. Initial imaging. The presence of a new neurologic deficit or cauda equina syndrome may be due to spinal cord or cauda equina compromise by either epidural abscess, displaced infected vertebral and/or disc material, or infection-mediated spinal malalignment or instability. The incidence of epidural abscess is 2.5 to 3 per 10,000 hospital admissions [14,60]. Although neurologic deficits are seen in 10% to 15% of cases of spine infection, these clinical situations require immediate imaging attention because the imaging evaluation helps to determine the location and extent of the spinal canal compromise [3,60]. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. Suspected Spine Infection CT Spine Area of Interest Noncontrast and contrast-enhanced CT have an overall low sensitivity (6%) for the identification of epidural abscess [33]. Gross spinal cord compression with compromise of the spinal canal (>50% canal narrowing) may be seen in more advanced cases of spine infection [71]. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection. In those cases in which a contrast-enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively [33]. CT with multiplanar reformations is often used in surgical planning and follow-up [8].

3148734

acrac_3148734_9

Suspected Spine Infection PCAs

MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast has a sensitivity of 96% and a specificity of 94% for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI also provides optimal depiction of the intraspinal contents including the epidural space and the spinal cord [6,17,18]. The use of MRI without and with IV contrast on an emergent or urgent basis, in patients with preexisting risk factors for possible spine infection and with an elevated ESR, may facilitate a more prompt diagnosis of spinal canal compromise by epidural abscess or other infected displaced structures. Epidural abscess is a feared complication of spine infection that may result in spinal cord and or cauda equina compression. The use of IV contrast helps to identify these abnormal epidural fluid collections, define their size and extent, and determine the presence of spinal cord and/or cauda equina compression [3]. MRI when performed without IV contrast may have utility, because it can show findings that are suggestive of possible spine infection, including marrow or paraspinal muscle edema, abnormal fluid collections, areas of abnormal signal, abnormality within the intervertebral disc, and adjacent vertebral endplates and gross structural abnormalities of the involved spine segment(s) [3,10,14,16,17,30,63-66,69]. MRI performed with IV contrast only is not considered to be useful because the precontrast MRI study is required for comparison in order to confirm areas of suspected abnormality within the spine segment(s) of interest. The presence and extent or the absence of contrast enhancement are important imaging features in suspected spinal infection and are best evaluated by comparing the pre- and postcontrast MRI examinations.

Suspected Spine Infection PCAs. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast has a sensitivity of 96% and a specificity of 94% for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI also provides optimal depiction of the intraspinal contents including the epidural space and the spinal cord [6,17,18]. The use of MRI without and with IV contrast on an emergent or urgent basis, in patients with preexisting risk factors for possible spine infection and with an elevated ESR, may facilitate a more prompt diagnosis of spinal canal compromise by epidural abscess or other infected displaced structures. Epidural abscess is a feared complication of spine infection that may result in spinal cord and or cauda equina compression. The use of IV contrast helps to identify these abnormal epidural fluid collections, define their size and extent, and determine the presence of spinal cord and/or cauda equina compression [3]. MRI when performed without IV contrast may have utility, because it can show findings that are suggestive of possible spine infection, including marrow or paraspinal muscle edema, abnormal fluid collections, areas of abnormal signal, abnormality within the intervertebral disc, and adjacent vertebral endplates and gross structural abnormalities of the involved spine segment(s) [3,10,14,16,17,30,63-66,69]. MRI performed with IV contrast only is not considered to be useful because the precontrast MRI study is required for comparison in order to confirm areas of suspected abnormality within the spine segment(s) of interest. The presence and extent or the absence of contrast enhancement are important imaging features in suspected spinal infection and are best evaluated by comparing the pre- and postcontrast MRI examinations.

3148734

acrac_3148734_10

Suspected Spine Infection PCAs

Radiography Spine Area of Interest Radiography is insensitive to the evaluation of the epidural space and to possible spinal cord compression and is therefore not useful as the initial imaging examination in patients presenting with neurologic compromise. As a complementary imaging study, radiography may help guide the imaging evaluation in those cases in which frank disc and vertebral body involvement by an infectious process is evident. Radiography can serve as a complementary test in order to assist with surgical management in those patients who may require surgical decompression and stabilization of the affected spinal segment [72]. 3-Phase Bone Scan There is no relevant literature regarding the use of bone scans in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. WBC Scan and Sulfur Colloid Scan Complete Spine There is no relevant literature regarding the use of WBC scans in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. Gallium Scan Whole Body There is no relevant literature regarding the use of gallium scans in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. FDG-PET/CT Whole Body There is no relevant literature regarding the use of FDG-PET/CT in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. Variant 4: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with decubitus ulcer or wound overlying spine. Initial imaging. Decubitus ulcers are often encountered at the level of the sacrum in chronically bedridden patients but may also be seen at other pressure sites along the back in immobile patients. When there is a clinical concern for possible spine infection extending from a decubitus ulcer or wound due to surgery or other causes [14], imaging may be necessary

Suspected Spine Infection PCAs. Radiography Spine Area of Interest Radiography is insensitive to the evaluation of the epidural space and to possible spinal cord compression and is therefore not useful as the initial imaging examination in patients presenting with neurologic compromise. As a complementary imaging study, radiography may help guide the imaging evaluation in those cases in which frank disc and vertebral body involvement by an infectious process is evident. Radiography can serve as a complementary test in order to assist with surgical management in those patients who may require surgical decompression and stabilization of the affected spinal segment [72]. 3-Phase Bone Scan There is no relevant literature regarding the use of bone scans in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. WBC Scan and Sulfur Colloid Scan Complete Spine There is no relevant literature regarding the use of WBC scans in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. Gallium Scan Whole Body There is no relevant literature regarding the use of gallium scans in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. FDG-PET/CT Whole Body There is no relevant literature regarding the use of FDG-PET/CT in the initial imaging evaluation of a suspected spinal infection with a new neurologic deficit or cauda equina syndrome. Variant 4: Suspected spine infection (such as epidural abscess or discitis osteomyelitis), with decubitus ulcer or wound overlying spine. Initial imaging. Decubitus ulcers are often encountered at the level of the sacrum in chronically bedridden patients but may also be seen at other pressure sites along the back in immobile patients. When there is a clinical concern for possible spine infection extending from a decubitus ulcer or wound due to surgery or other causes [14], imaging may be necessary

3148734

acrac_3148734_11

Suspected Spine Infection PCAs

Suspected Spine Infection for further evaluation of the involved spinal segment. Imaging can be utilized to distinguish between superficial infection or cellulitis and deeper infections including osteomyelitis and paraspinal or epidural abscess formation [31,48]. The body regions covered in this clinical scenario are the cervical, thoracic, lumbar spine, and sacrum. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. CT Spine Area of Interest As a result of its excellent delineation of osseous detail and greater sensitivity than radiography, CT can be used in the evaluation of suspected osteomyelitis as a complication from a decubitus ulcer or wound overlying the spine [3]. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection. In those cases in which a contrast-enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively [33]. CT may be used to assess the spine for suspected infection following any surgical or interventional procedure [3,30]. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast can be utilized for the evaluation of patients with suspected spine infection at the site of a decubitus ulcer or wound [3,7,14,16,24,37,51]. The sensitivity and specificity of MRI for spine infection is 96% and 94%, respectively [14].

Suspected Spine Infection PCAs. Suspected Spine Infection for further evaluation of the involved spinal segment. Imaging can be utilized to distinguish between superficial infection or cellulitis and deeper infections including osteomyelitis and paraspinal or epidural abscess formation [31,48]. The body regions covered in this clinical scenario are the cervical, thoracic, lumbar spine, and sacrum. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information. CT Spine Area of Interest As a result of its excellent delineation of osseous detail and greater sensitivity than radiography, CT can be used in the evaluation of suspected osteomyelitis as a complication from a decubitus ulcer or wound overlying the spine [3]. The addition of IV contrast increases the conspicuity of paraspinal soft tissue abnormalities, such as inflammation or abscess that may be caused by infection. In those cases in which a contrast-enhanced CT is to be performed, it is not necessary or useful to perform a noncontrast-enhanced CT first, because this latter examination does not add more diagnostic information. The sensitivity and specificity of CT for spine infection is 79% and 100%, respectively [33]. CT may be used to assess the spine for suspected infection following any surgical or interventional procedure [3,30]. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast can be utilized for the evaluation of patients with suspected spine infection at the site of a decubitus ulcer or wound [3,7,14,16,24,37,51]. The sensitivity and specificity of MRI for spine infection is 96% and 94%, respectively [14].

3148734

acrac_3148734_12

Suspected Spine Infection PCAs

The use of IV contrast not only increases lesion conspicuity, characterized by foci of abnormal soft tissue enhancement and peripherally enhancing fluid collections within and/or surrounding the affected spinal segment, but also helps to define the extent of the infectious process [3]. MRI is also used to help distinguish expected postoperative changes at the surgical skin site from infection and contrast-enhanced MRI can be used to assess postoperative fluid collections for suspected infection [28,29,69,70]. MRI when performed without IV contrast may have utility, because it can show findings that are suggestive of possible spine infection, including marrow or paraspinal muscle edema, abnormal fluid collections, areas of abnormal signal, abnormality within the intervertebral disc, and adjacent vertebral endplates and gross structural abnormalities of the involved spine segment(s) [3,10,14,16,17,30,63-66,69]. MRI performed with IV contrast only is not considered to be useful because the precontrast MRI study is required for comparison in order to confirm areas of suspected abnormality within the spine segment(s) of interest. The presence and extent or the absence of contrast enhancement are important imaging features in suspected spinal infection and are best evaluated by comparing the pre- and postcontrast MRI examinations. Radiography Spine Area of Interest Radiography provides a quick survey of the soft tissues and underlying osseous structures at the site of suspected spine infection when either a decubitus ulcer or wound is present [3]. Radiography can be used to tailor a subsequent cross-sectional imaging examination especially in patients with prior spine surgery or interventions [8]. 3-Phase Bone Scan A 3-phase bone scan with Tc-99m-MDP has variable moderate-to-high sensitivity (81.4%) and low specificity (40.7%) for suspected spine infection with decubitus ulcer or wound overlying the spine [31].

Suspected Spine Infection PCAs. The use of IV contrast not only increases lesion conspicuity, characterized by foci of abnormal soft tissue enhancement and peripherally enhancing fluid collections within and/or surrounding the affected spinal segment, but also helps to define the extent of the infectious process [3]. MRI is also used to help distinguish expected postoperative changes at the surgical skin site from infection and contrast-enhanced MRI can be used to assess postoperative fluid collections for suspected infection [28,29,69,70]. MRI when performed without IV contrast may have utility, because it can show findings that are suggestive of possible spine infection, including marrow or paraspinal muscle edema, abnormal fluid collections, areas of abnormal signal, abnormality within the intervertebral disc, and adjacent vertebral endplates and gross structural abnormalities of the involved spine segment(s) [3,10,14,16,17,30,63-66,69]. MRI performed with IV contrast only is not considered to be useful because the precontrast MRI study is required for comparison in order to confirm areas of suspected abnormality within the spine segment(s) of interest. The presence and extent or the absence of contrast enhancement are important imaging features in suspected spinal infection and are best evaluated by comparing the pre- and postcontrast MRI examinations. Radiography Spine Area of Interest Radiography provides a quick survey of the soft tissues and underlying osseous structures at the site of suspected spine infection when either a decubitus ulcer or wound is present [3]. Radiography can be used to tailor a subsequent cross-sectional imaging examination especially in patients with prior spine surgery or interventions [8]. 3-Phase Bone Scan A 3-phase bone scan with Tc-99m-MDP has variable moderate-to-high sensitivity (81.4%) and low specificity (40.7%) for suspected spine infection with decubitus ulcer or wound overlying the spine [31].

3148734

acrac_3148734_13

Suspected Spine Infection PCAs

Gallium Scan Whole Body Ga-67 scintigraphy combined with SPECT can be used to evaluate suspected infection involving a decubitus ulcer or wound overlying the spine. Ga-67 is less sensitive (73%) but more specific (61%) than skeletal scintigraphy [31]. The disadvantages of the gallium examination include a requirement for delayed images (24 to 72 hours) [31]. A dual Ga-67 and Tc-99m-MDP examination has a similar sensitivity (73%) and an increased specificity (81%) [31]. This combined examination may be utilized in select clinical situations such as when spine infection is suspected adjacent to a decubitus ulcer or wound [31]. WBC Scan and Sulfur Colloid Scan Complete Spine A labeled leukocyte and sulfur colloid study is limited in the evaluation of spine infection because areas of infection often demonstrate decreased or absent radionuclide uptake [31]. Suspected Spine Infection FDG-PET/CT Whole Body FDG-PET with CT has seen increasing application for the assessment of suspected spine infection in select cases as a complementary examination [10,31]. Increased FDG uptake is seen at sites of infection with an elevated SUVmax value. FDG-PET/CT can be used in the evaluation of the postsurgical spine for suspected infection of the skin wound when MRI is inconclusive. Variant 5: Suspected spine infection (such as epidural abscess or discitis osteomyelitis). Abnormal radiographs or CT findings. Next imaging study. When an imaging study such as a radiograph or CT of the spine raises a concern for possible spine infection, additional imaging may be required. Because there are other pathologic entities, such as degenerative, traumatic, or inflammatory spondyloarthropathy, which can simulate spine infection on the initial radiographs or CT images, additional imaging is used in conjunction with the clinical evaluation in order to make the appropriate diagnosis [55]. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine.

Suspected Spine Infection PCAs. Gallium Scan Whole Body Ga-67 scintigraphy combined with SPECT can be used to evaluate suspected infection involving a decubitus ulcer or wound overlying the spine. Ga-67 is less sensitive (73%) but more specific (61%) than skeletal scintigraphy [31]. The disadvantages of the gallium examination include a requirement for delayed images (24 to 72 hours) [31]. A dual Ga-67 and Tc-99m-MDP examination has a similar sensitivity (73%) and an increased specificity (81%) [31]. This combined examination may be utilized in select clinical situations such as when spine infection is suspected adjacent to a decubitus ulcer or wound [31]. WBC Scan and Sulfur Colloid Scan Complete Spine A labeled leukocyte and sulfur colloid study is limited in the evaluation of spine infection because areas of infection often demonstrate decreased or absent radionuclide uptake [31]. Suspected Spine Infection FDG-PET/CT Whole Body FDG-PET with CT has seen increasing application for the assessment of suspected spine infection in select cases as a complementary examination [10,31]. Increased FDG uptake is seen at sites of infection with an elevated SUVmax value. FDG-PET/CT can be used in the evaluation of the postsurgical spine for suspected infection of the skin wound when MRI is inconclusive. Variant 5: Suspected spine infection (such as epidural abscess or discitis osteomyelitis). Abnormal radiographs or CT findings. Next imaging study. When an imaging study such as a radiograph or CT of the spine raises a concern for possible spine infection, additional imaging may be required. Because there are other pathologic entities, such as degenerative, traumatic, or inflammatory spondyloarthropathy, which can simulate spine infection on the initial radiographs or CT images, additional imaging is used in conjunction with the clinical evaluation in order to make the appropriate diagnosis [55]. The body regions covered in this clinical scenario are the cervical, thoracic, and lumbar spine.

3148734

acrac_3148734_14

Suspected Spine Infection PCAs

These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information, including prior imaging. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast is often utilized for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI without and with IV contrast has a sensitivity of 96% and a specificity of 94% for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI also provides optimal depiction of the intraspinal contents, including the epidural space and the spinal cord [6,17,18]. The use of IV contrast increases lesion conspicuity, characterized by foci of abnormal soft tissue enhancement and peripherally enhancing fluid collections within and/or surrounding the affected spinal segment, and also helps to define the extent of the infectious process [3]. MRI can be performed as the next imaging study when the initial radiographs and/or CT examination show abnormal findings that may be indicative of spine infection. MRI when performed without IV contrast may have utility, because it can show findings that are suggestive of possible spine infection, including marrow or paraspinal muscle edema, abnormal fluid collections, areas of abnormal signal, abnormality within the intervertebral disc, and adjacent vertebral endplates and gross structural abnormalities of the involved spine segment(s) [3,10,14,16,17,30,63-66,69]. MRI performed with IV contrast only is not considered to be useful because the precontrast MRI study is required for comparison in order to confirm areas of suspected abnormality within the spine segment(s) of interest. The presence and extent or the absence of contrast enhancement are important imaging features in suspected spinal infection and are best evaluated by comparing the pre- and postcontrast MRI examinations.

Suspected Spine Infection PCAs. These body regions might be evaluated separately or in combination as guided by physical examination findings, patient history, and other available information, including prior imaging. MRI Spine Area of Interest Because of its excellent tissue characterization and anatomic delineation, MRI without and with IV contrast is often utilized for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI without and with IV contrast has a sensitivity of 96% and a specificity of 94% for the evaluation of patients with suspected spine infection [3,7,14,16,24,37,51]. MRI also provides optimal depiction of the intraspinal contents, including the epidural space and the spinal cord [6,17,18]. The use of IV contrast increases lesion conspicuity, characterized by foci of abnormal soft tissue enhancement and peripherally enhancing fluid collections within and/or surrounding the affected spinal segment, and also helps to define the extent of the infectious process [3]. MRI can be performed as the next imaging study when the initial radiographs and/or CT examination show abnormal findings that may be indicative of spine infection. MRI when performed without IV contrast may have utility, because it can show findings that are suggestive of possible spine infection, including marrow or paraspinal muscle edema, abnormal fluid collections, areas of abnormal signal, abnormality within the intervertebral disc, and adjacent vertebral endplates and gross structural abnormalities of the involved spine segment(s) [3,10,14,16,17,30,63-66,69]. MRI performed with IV contrast only is not considered to be useful because the precontrast MRI study is required for comparison in order to confirm areas of suspected abnormality within the spine segment(s) of interest. The presence and extent or the absence of contrast enhancement are important imaging features in suspected spinal infection and are best evaluated by comparing the pre- and postcontrast MRI examinations.

3148734

acrac_3102393_0

Clinically Suspected Vascular Malformation of the Extremities

Introduction/Background Vascular anomalies encompass a broad range of pathologies histologically composed of vascular type cells. These lesions are most commonly classified by the International Society for the Study of Vascular Anomalies according to their underlying histology as either vascular malformations or vascular tumors. Vascular malformations represent focal structural abnormalities of the vascular tree, typically related to developmental errors during vasculogenesis [1], whereas, vascular tumors are caused by neoplastic cellular proliferation of the endothelium [1]. The extremities are the most common site of these vascular lesions outside of the head and neck [1,2]. Vascular malformations more commonly represent isolated spontaneous lesions yet can be part of one of several syndromes such as Parkes Weber syndrome [1,3]. These typically grow commensurate with patient age, often in conjunction with hormonal changes, such as puberty and pregnancy [3-6]. Therefore, vascular malformations that are present at birth may not present clinically until adolescence or adulthood. These lesions can be broadly divided into high- and low-flow lesions. High-flow malformations include arteriovenous malformations and arteriovenous fistulas, both of which demonstrate arterial flow and arteriovenous shunting. The former tend to be congenital lesions, and the latter are typically acquired as sequela of prior trauma or surgery. High-flow lesions comprise approximately 10% of peripheral vascular malformations and may present with pain, skin discoloration, warmth, or mass with palpable thrill or bruit [6]. Compression neuropathy, soft-tissue ulceration, bleeding, arterial steal phenomenon, and high-output cardiac failure may be seen in extreme cases [4,7]. Low-flow lesions include capillary, venous, and lymphatic malformations and are overall more common than high-flow lesions.

Clinically Suspected Vascular Malformation of the Extremities. Introduction/Background Vascular anomalies encompass a broad range of pathologies histologically composed of vascular type cells. These lesions are most commonly classified by the International Society for the Study of Vascular Anomalies according to their underlying histology as either vascular malformations or vascular tumors. Vascular malformations represent focal structural abnormalities of the vascular tree, typically related to developmental errors during vasculogenesis [1], whereas, vascular tumors are caused by neoplastic cellular proliferation of the endothelium [1]. The extremities are the most common site of these vascular lesions outside of the head and neck [1,2]. Vascular malformations more commonly represent isolated spontaneous lesions yet can be part of one of several syndromes such as Parkes Weber syndrome [1,3]. These typically grow commensurate with patient age, often in conjunction with hormonal changes, such as puberty and pregnancy [3-6]. Therefore, vascular malformations that are present at birth may not present clinically until adolescence or adulthood. These lesions can be broadly divided into high- and low-flow lesions. High-flow malformations include arteriovenous malformations and arteriovenous fistulas, both of which demonstrate arterial flow and arteriovenous shunting. The former tend to be congenital lesions, and the latter are typically acquired as sequela of prior trauma or surgery. High-flow lesions comprise approximately 10% of peripheral vascular malformations and may present with pain, skin discoloration, warmth, or mass with palpable thrill or bruit [6]. Compression neuropathy, soft-tissue ulceration, bleeding, arterial steal phenomenon, and high-output cardiac failure may be seen in extreme cases [4,7]. Low-flow lesions include capillary, venous, and lymphatic malformations and are overall more common than high-flow lesions.

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Of these, capillary malformations are the most common but rarely require imaging for diagnosis because of their characteristic cutaneous manifestations [3,4]. Venous and lymphatic malformations have a reported prevalence of 1% in the general population with 40% involving the extremities [6]. Although symptomatology is variable, these lesions may present with focal or more generalized extremity pain, swelling, or compressible mass with or without associated skin discoloration. Involvement of the deep tissues, including bone, is not uncommon, and physical examination often underestimates their full extent [2,5]. Vascular tumors are subclassified based on propensity for aggressive and malignant behavior; however, the majority are benign [3]. Infantile hemangiomas are among the most common type of vascular neoplasm. These benign lesions present in infancy or early childhood and demonstrate rapid proliferative growth followed by eventual involution, most often not requiring treatment [7]. Less commonly, vascular tumors such as the intramuscular hemangioma, can present in adulthood with swelling, pain, or mass. The diagnosis of a vascular malformation or neoplasm is frequently made clinically when classic signs and symptoms are present. Imaging is used for confirmation, particularly if the clinical presentation is atypical or vague [3,8] and is generally required for characterization of these lesions. Imaging also plays a critical role in treatment planning, which is often required because of growth, limb deformity, and decreased function, as well as pain. Lesion characteristics such as subtype (high flow versus low flow), depth, and invasion of adjacent structures, as well as inflow and outflow vessels, help in optimal treatment selection [9]. Treatment approach spans the spectrum of conservative to aggressive options, which include compression dressings, sclerotherapy,

Clinically Suspected Vascular Malformation of the Extremities. Of these, capillary malformations are the most common but rarely require imaging for diagnosis because of their characteristic cutaneous manifestations [3,4]. Venous and lymphatic malformations have a reported prevalence of 1% in the general population with 40% involving the extremities [6]. Although symptomatology is variable, these lesions may present with focal or more generalized extremity pain, swelling, or compressible mass with or without associated skin discoloration. Involvement of the deep tissues, including bone, is not uncommon, and physical examination often underestimates their full extent [2,5]. Vascular tumors are subclassified based on propensity for aggressive and malignant behavior; however, the majority are benign [3]. Infantile hemangiomas are among the most common type of vascular neoplasm. These benign lesions present in infancy or early childhood and demonstrate rapid proliferative growth followed by eventual involution, most often not requiring treatment [7]. Less commonly, vascular tumors such as the intramuscular hemangioma, can present in adulthood with swelling, pain, or mass. The diagnosis of a vascular malformation or neoplasm is frequently made clinically when classic signs and symptoms are present. Imaging is used for confirmation, particularly if the clinical presentation is atypical or vague [3,8] and is generally required for characterization of these lesions. Imaging also plays a critical role in treatment planning, which is often required because of growth, limb deformity, and decreased function, as well as pain. Lesion characteristics such as subtype (high flow versus low flow), depth, and invasion of adjacent structures, as well as inflow and outflow vessels, help in optimal treatment selection [9]. Treatment approach spans the spectrum of conservative to aggressive options, which include compression dressings, sclerotherapy,

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Clinically Suspected Vascular Malformation of the Extremities

The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through society representation on expert panels. Participation by representatives from collaborating societies on the expert panel does not necessarily imply individual or society endorsement of the final document. Reprint requests to: [email protected] Vascular Malformation of the Extremities transarterial or transvenous embolization, and surgical resection. Sclerotherapy is often used for low-flow lesions, whereas high-flow lesions are most effectively treated with embolization [8,10]. All elements are essential: 1) timing, 2) reconstructions/reformats, and 3) 3-D renderings. Standard CTs with contrast also include timing issues and recons/reformats. Only in CTA, however, is 3-D rendering a required element. This corresponds to the definitions that the CMS has applied to the Current Procedural Terminology codes. Discussion of Procedures by Variant Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging. The body regions covered in this clinical scenario are shoulder, humerus, elbow, forearm, wrist, hand, hip, femur, knee, tibia/fibula, ankle, and foot. Radiography Extremity Radiographs are often used as the initial imaging modality in the workup of a patient presenting with nonspecific extremity complaints and may be useful for the exclusion of more common causes of extremity pain and deformity. However, radiographs are of limited utility for the specific purpose of vascular malformation imaging. In the setting of a vascular malformation, radiographs may be normal or show a soft-tissue mass [5,12].

Clinically Suspected Vascular Malformation of the Extremities. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through society representation on expert panels. Participation by representatives from collaborating societies on the expert panel does not necessarily imply individual or society endorsement of the final document. Reprint requests to: [email protected] Vascular Malformation of the Extremities transarterial or transvenous embolization, and surgical resection. Sclerotherapy is often used for low-flow lesions, whereas high-flow lesions are most effectively treated with embolization [8,10]. All elements are essential: 1) timing, 2) reconstructions/reformats, and 3) 3-D renderings. Standard CTs with contrast also include timing issues and recons/reformats. Only in CTA, however, is 3-D rendering a required element. This corresponds to the definitions that the CMS has applied to the Current Procedural Terminology codes. Discussion of Procedures by Variant Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging. The body regions covered in this clinical scenario are shoulder, humerus, elbow, forearm, wrist, hand, hip, femur, knee, tibia/fibula, ankle, and foot. Radiography Extremity Radiographs are often used as the initial imaging modality in the workup of a patient presenting with nonspecific extremity complaints and may be useful for the exclusion of more common causes of extremity pain and deformity. However, radiographs are of limited utility for the specific purpose of vascular malformation imaging. In the setting of a vascular malformation, radiographs may be normal or show a soft-tissue mass [5,12].

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Clinically Suspected Vascular Malformation of the Extremities

Phleboliths may also be seen and provide a clue to the diagnosis of venous malformations and hemangiomas, which are reported to contain phleboliths in 20% to 67% of cases [3,12]. Lesions located adjacent to bone may be associated with bone changes, including periosteal reaction, remodeling, or signs of destruction, such as cortical scalloping and lucencies. Although such findings may be visible radiographically, they are not specific for the diagnosis of vascular malformations [6,12]. US Duplex Doppler Extremity Ultrasound (US) with duplex Doppler imaging can be useful for the initial assessment of suspected vascular malformation, particularly if a focal mass or other targetable superficial symptomatology is present. US with Doppler can differentiate high-flow from low-flow malformations, and often provides a specific diagnosis in cases where characteristic vascular malformation features are present [3,5,13,14]. US may also be diagnostic of other lesions within the differential diagnosis for nonspecific extremity complaints [3,15]. Ultimately, this modality is limited in regards to tissue penetration and a small imaging field of view, which may lead to suboptimal evaluation of lesion extent and size, particularly if located deep in the extremity or adjacent to bone [2,8,13]. As a result, cross-sectional imaging, such as MRI, may be needed for more complete evaluation and definitive diagnosis [1,3,13], especially in cases without a targetable focal abnormality. US Extremity Area of Interest with IV Contrast There is limited evidence regarding the utility of contrast-enhanced US (CEUS) in evaluating suspected peripheral vascular malformations, but this modality may be considered in select cases. The addition of microbubble contrast may enhance visualization of small arteriovenous shunts and low-flow vessels compared with US with Doppler [16].

Clinically Suspected Vascular Malformation of the Extremities. Phleboliths may also be seen and provide a clue to the diagnosis of venous malformations and hemangiomas, which are reported to contain phleboliths in 20% to 67% of cases [3,12]. Lesions located adjacent to bone may be associated with bone changes, including periosteal reaction, remodeling, or signs of destruction, such as cortical scalloping and lucencies. Although such findings may be visible radiographically, they are not specific for the diagnosis of vascular malformations [6,12]. US Duplex Doppler Extremity Ultrasound (US) with duplex Doppler imaging can be useful for the initial assessment of suspected vascular malformation, particularly if a focal mass or other targetable superficial symptomatology is present. US with Doppler can differentiate high-flow from low-flow malformations, and often provides a specific diagnosis in cases where characteristic vascular malformation features are present [3,5,13,14]. US may also be diagnostic of other lesions within the differential diagnosis for nonspecific extremity complaints [3,15]. Ultimately, this modality is limited in regards to tissue penetration and a small imaging field of view, which may lead to suboptimal evaluation of lesion extent and size, particularly if located deep in the extremity or adjacent to bone [2,8,13]. As a result, cross-sectional imaging, such as MRI, may be needed for more complete evaluation and definitive diagnosis [1,3,13], especially in cases without a targetable focal abnormality. US Extremity Area of Interest with IV Contrast There is limited evidence regarding the utility of contrast-enhanced US (CEUS) in evaluating suspected peripheral vascular malformations, but this modality may be considered in select cases. The addition of microbubble contrast may enhance visualization of small arteriovenous shunts and low-flow vessels compared with US with Doppler [16].

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Clinically Suspected Vascular Malformation of the Extremities

CEUS also has potential for quantifying perfusion in vascular malformations, which could be helpful in assessing treatment response [17]. CT Extremity CT offers the benefit of high spatial resolution and provides comprehensive anatomic detail in the workup of extremity complaints. For the specific purpose of vascular malformation imaging, CT may reveal a soft-tissue mass with or without phleboliths, as well as provide information about size and lesion extent [12]. Bone involvement and acute complications like hemorrhage can also be assessed with CT [3,6]. Intravenous (IV) Vascular Malformation of the Extremities contrast administration improves lesion delineation and allows for the assessment of enhancement patterns, which may help narrow the differential diagnosis of a focal finding [3]. However, in general, MRI is the preferred imaging modality when evaluating suspected vascular malformations that are due to its greater soft-tissue contrast and ability to obtain dynamic flow information with MR angiography (MRA) [10]. CTA Extremity CTA with IV contrast can be used to evaluate a suspected vascular malformation. It is generally of greater utility for a high-flow lesion, such as an arteriovenous malformation, as CTA is capable of delineating the feeding arteries, nidus, and draining veins, which typically characterize these lesions [10]. CTA may also provide some information in regards to lesion extent and invasion into muscular compartments and bones [10]. However, MRI or MRA is the preferred method for suspected vascular malformation imaging that is due to superior soft-tissue contrast and potential for dynamic blood flow imaging [2,10,18]. MRI Extremity MRI offers superior soft-tissue contrast compared with CT and plays an important role in the workup of suspected vascular malformations and soft-tissue masses. Lesion morphology and internal signal characteristics can be assessed, often allowing for definitive diagnosis [2-4,6,7,9,19].

Clinically Suspected Vascular Malformation of the Extremities. CEUS also has potential for quantifying perfusion in vascular malformations, which could be helpful in assessing treatment response [17]. CT Extremity CT offers the benefit of high spatial resolution and provides comprehensive anatomic detail in the workup of extremity complaints. For the specific purpose of vascular malformation imaging, CT may reveal a soft-tissue mass with or without phleboliths, as well as provide information about size and lesion extent [12]. Bone involvement and acute complications like hemorrhage can also be assessed with CT [3,6]. Intravenous (IV) Vascular Malformation of the Extremities contrast administration improves lesion delineation and allows for the assessment of enhancement patterns, which may help narrow the differential diagnosis of a focal finding [3]. However, in general, MRI is the preferred imaging modality when evaluating suspected vascular malformations that are due to its greater soft-tissue contrast and ability to obtain dynamic flow information with MR angiography (MRA) [10]. CTA Extremity CTA with IV contrast can be used to evaluate a suspected vascular malformation. It is generally of greater utility for a high-flow lesion, such as an arteriovenous malformation, as CTA is capable of delineating the feeding arteries, nidus, and draining veins, which typically characterize these lesions [10]. CTA may also provide some information in regards to lesion extent and invasion into muscular compartments and bones [10]. However, MRI or MRA is the preferred method for suspected vascular malformation imaging that is due to superior soft-tissue contrast and potential for dynamic blood flow imaging [2,10,18]. MRI Extremity MRI offers superior soft-tissue contrast compared with CT and plays an important role in the workup of suspected vascular malformations and soft-tissue masses. Lesion morphology and internal signal characteristics can be assessed, often allowing for definitive diagnosis [2-4,6,7,9,19].

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Clinically Suspected Vascular Malformation of the Extremities

MRI accurately determines lesion extent and involvement of surrounding structures, both of which are underestimated clinically in up to 76% of cases [2,5,7]. Contrast-enhanced sequences may not be necessary if typical features, such as flow voids, are present; however, the use of IV contrast is preferred for improved specificity and more complete characterization [3,6,8,20]. IV contrast also allows better visualization of the feeding and draining vessels in high-flow lesions, although this is best performed using an MRA protocol [1-3,6,7,21]. Additionally, MRI is useful for the evaluation of other soft- tissue lesions and musculoskeletal pathologies that might be considered in the differential diagnosis for a mass, enlarged extremity, or pain [19,20,22,23]. MRA Extremity MRA is an excellent imaging option when a vascular malformation is suspected. The typical MRA protocol includes conventional T1 and T2 sequences, which provide anatomic information including lesion size, extent, and internal morphology [6,7]. Dynamic contrast-enhanced MRA, when combined with conventional MRI, has a reported sensitivity of 83% and specificity of 95% for the differentiation of venous and nonvenous malformations [24]. Time-resolved MRA has been shown to be nearly equivalent to arteriography for evaluating dynamic perfusion, allowing for accurate differentiation of feeding arteries and draining veins in high-flow lesions [2,9]. MRA can also be useful in differentiating vascular malformations from other causes of an extremity mass, such as soft-tissue neoplasms, although there may be some overlap in findings [7]. Although noncontrast time-of-flight techniques can be employed, contrast-enhanced MRA is preferred for improved depiction of smaller vessels and dynamic imaging assessment [6,25]. Arteriography Extremity There is no evidence to support the use of arteriography as the initial imaging evaluation for a suspected vascular malformation because of its invasive nature.

Clinically Suspected Vascular Malformation of the Extremities. MRI accurately determines lesion extent and involvement of surrounding structures, both of which are underestimated clinically in up to 76% of cases [2,5,7]. Contrast-enhanced sequences may not be necessary if typical features, such as flow voids, are present; however, the use of IV contrast is preferred for improved specificity and more complete characterization [3,6,8,20]. IV contrast also allows better visualization of the feeding and draining vessels in high-flow lesions, although this is best performed using an MRA protocol [1-3,6,7,21]. Additionally, MRI is useful for the evaluation of other soft- tissue lesions and musculoskeletal pathologies that might be considered in the differential diagnosis for a mass, enlarged extremity, or pain [19,20,22,23]. MRA Extremity MRA is an excellent imaging option when a vascular malformation is suspected. The typical MRA protocol includes conventional T1 and T2 sequences, which provide anatomic information including lesion size, extent, and internal morphology [6,7]. Dynamic contrast-enhanced MRA, when combined with conventional MRI, has a reported sensitivity of 83% and specificity of 95% for the differentiation of venous and nonvenous malformations [24]. Time-resolved MRA has been shown to be nearly equivalent to arteriography for evaluating dynamic perfusion, allowing for accurate differentiation of feeding arteries and draining veins in high-flow lesions [2,9]. MRA can also be useful in differentiating vascular malformations from other causes of an extremity mass, such as soft-tissue neoplasms, although there may be some overlap in findings [7]. Although noncontrast time-of-flight techniques can be employed, contrast-enhanced MRA is preferred for improved depiction of smaller vessels and dynamic imaging assessment [6,25]. Arteriography Extremity There is no evidence to support the use of arteriography as the initial imaging evaluation for a suspected vascular malformation because of its invasive nature.

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Clinically Suspected Vascular Malformation of the Extremities

MRA is noninvasive and can depict the vascular anatomy of a malformation nearly as well as arteriography [9], making it the preferred initial imaging evaluation. Arteriography does offer the highest resolution imaging of small vessels and superior temporal resolution for assessment of flow dynamics. These advantages may be useful for high-flow lesions when MRA findings are equivocal or when treatment planning requires the highest available vascular detail resolution and/or better estimation of intralesional shunting [5-8]. Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging. The body regions covered in this clinical scenario are shoulder, humerus, elbow, forearm, wrist, hand, hip, femur, knee, tibia/fibula, ankle, and foot. Radiography Extremity Radiographs are of limited benefit for the specific purpose of vascular malformation imaging, especially in regard to lesions presenting with a vascular murmur. Radiographs may be normal or show a soft-tissue mass [5,12]. Venous malformations and hemangiomas may contain radiographically visible phleboliths in 20% to 67% of cases, which provide a clue to the diagnosis; however, these lesions typically do not present with a vascular murmur [3,12]. Lesions located adjacent to bone may be associated with bone changes, such as periosteal reaction, remodeling, or signs of destruction, such as cortical scalloping and lucencies. Although such findings may be visible radiographically, they are not specific for the diagnosis of vascular malformations [6,12]. Vascular Malformation of the Extremities US Duplex Doppler Extremity US is fast and can often provide initial imaging characterization of vascular malformations [5-7,13]. The presence of a vascular murmur is clinically suggestive of a high-flow malformation, and US with Doppler imaging is generally regarded as a good initial option for the confirmation of a high-flow component.

Clinically Suspected Vascular Malformation of the Extremities. MRA is noninvasive and can depict the vascular anatomy of a malformation nearly as well as arteriography [9], making it the preferred initial imaging evaluation. Arteriography does offer the highest resolution imaging of small vessels and superior temporal resolution for assessment of flow dynamics. These advantages may be useful for high-flow lesions when MRA findings are equivocal or when treatment planning requires the highest available vascular detail resolution and/or better estimation of intralesional shunting [5-8]. Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging. The body regions covered in this clinical scenario are shoulder, humerus, elbow, forearm, wrist, hand, hip, femur, knee, tibia/fibula, ankle, and foot. Radiography Extremity Radiographs are of limited benefit for the specific purpose of vascular malformation imaging, especially in regard to lesions presenting with a vascular murmur. Radiographs may be normal or show a soft-tissue mass [5,12]. Venous malformations and hemangiomas may contain radiographically visible phleboliths in 20% to 67% of cases, which provide a clue to the diagnosis; however, these lesions typically do not present with a vascular murmur [3,12]. Lesions located adjacent to bone may be associated with bone changes, such as periosteal reaction, remodeling, or signs of destruction, such as cortical scalloping and lucencies. Although such findings may be visible radiographically, they are not specific for the diagnosis of vascular malformations [6,12]. Vascular Malformation of the Extremities US Duplex Doppler Extremity US is fast and can often provide initial imaging characterization of vascular malformations [5-7,13]. The presence of a vascular murmur is clinically suggestive of a high-flow malformation, and US with Doppler imaging is generally regarded as a good initial option for the confirmation of a high-flow component.

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Clinically Suspected Vascular Malformation of the Extremities

In many cases, US can help differentiate between the various types of vascular malformations and other soft-tissue lesions [5,7,13-15]. However, US has limitations in regard to field of view and tissue penetration that typically limits the ability to completely characterize and delineate the full extent of vascular malformations. Additional cross-sectional imaging, such as MRI, is usually needed for complete characterization [2,8,9]. US Extremity Area of Interest with IV Contrast Limited evidence is available regarding the utility of CEUS specifically for the evaluation of peripheral vascular malformations, but this may be considered in select cases. Visualization of small arteriovenous shunts may be improved with CEUS compared with US with Doppler [16]. The potential for quantifying perfusion in vascular malformations with CEUS could also be useful in assessing treatment response [17]. CTA Extremity CTA features comparatively high spatial resolution, which allows for the characterization of a vascular nidus, enlarged feeding arteries, and draining veins that are frequently encountered in high-flow lesions that often present clinically with a vascular murmur [10]. CTA may also be useful for the assessment of other vascular- related pathologies, such as vasculitis and compression syndromes [10]. However, poor soft-tissue contrast and limited temporal resolution are drawbacks to this modality, and MRA is typically preferred over CTA [10,18]. MRI Extremity The high tissue contrast of MRI makes it a preferred modality to assess the extent and distribution of vascular malformations, which are often underestimated by physical examination alone [2,5]. MRI is also a good option to evaluate for other soft-tissue lesions that may be included within a differential diagnosis of bruit on clinical examination [19,23]. The use of IV contrast improves lesion characterization and optimizes visualization of the surrounding anatomy [3,6,20].

Clinically Suspected Vascular Malformation of the Extremities. In many cases, US can help differentiate between the various types of vascular malformations and other soft-tissue lesions [5,7,13-15]. However, US has limitations in regard to field of view and tissue penetration that typically limits the ability to completely characterize and delineate the full extent of vascular malformations. Additional cross-sectional imaging, such as MRI, is usually needed for complete characterization [2,8,9]. US Extremity Area of Interest with IV Contrast Limited evidence is available regarding the utility of CEUS specifically for the evaluation of peripheral vascular malformations, but this may be considered in select cases. Visualization of small arteriovenous shunts may be improved with CEUS compared with US with Doppler [16]. The potential for quantifying perfusion in vascular malformations with CEUS could also be useful in assessing treatment response [17]. CTA Extremity CTA features comparatively high spatial resolution, which allows for the characterization of a vascular nidus, enlarged feeding arteries, and draining veins that are frequently encountered in high-flow lesions that often present clinically with a vascular murmur [10]. CTA may also be useful for the assessment of other vascular- related pathologies, such as vasculitis and compression syndromes [10]. However, poor soft-tissue contrast and limited temporal resolution are drawbacks to this modality, and MRA is typically preferred over CTA [10,18]. MRI Extremity The high tissue contrast of MRI makes it a preferred modality to assess the extent and distribution of vascular malformations, which are often underestimated by physical examination alone [2,5]. MRI is also a good option to evaluate for other soft-tissue lesions that may be included within a differential diagnosis of bruit on clinical examination [19,23]. The use of IV contrast improves lesion characterization and optimizes visualization of the surrounding anatomy [3,6,20].

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Although MRI is generally considered high yield for evaluating lesion extent and often can distinguish between the various types of vascular malformations and soft-tissues masses, its evaluation of flow dynamics and intralesional vascular anatomy is limited when compared with MRA [3]. MRA Extremity MRA has emerged as the preferred modality for assessing vascular malformations, particularly in patients with a vascular murmur and suspected high-flow malformation, due to its exceptional ability to delineate inflow and outflow anatomy noninvasively. Time-resolved MRA has been reported to rival conventional angiography for the portrayal of both functional flow dynamics and anatomic detail [9]. Combined conventional and dynamic contrast-enhanced MRA has a reported sensitivity of 83% and specificity of 95% for the differentiation of venous and nonvenous malformations [24]. Furthermore, MRA protocols typically include conventional high soft-tissue contrast T1 and T2 sequences which accurately assess the internal characteristics and extent of vascular malformations. This modality can also assess for and characterize other possible soft-tissue masses which may be included in the clinical differential diagnosis [6,7]. One potential weakness of time-resolved MRA is its underestimation of shunt volumes in vascular malformations which may be better evaluated with arteriography [7]. Arteriography Extremity Arteriography offers high temporal and spatial resolution images of the vascular anatomy associated with high- flow lesions such as arteriovenous malformations, including the inflow and outflow vessels as well as intralesional shunting [5,7]. Although the presence of a vascular murmur increases the suspicion of a high-flow Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch. acr.org/list.

Clinically Suspected Vascular Malformation of the Extremities. Although MRI is generally considered high yield for evaluating lesion extent and often can distinguish between the various types of vascular malformations and soft-tissues masses, its evaluation of flow dynamics and intralesional vascular anatomy is limited when compared with MRA [3]. MRA Extremity MRA has emerged as the preferred modality for assessing vascular malformations, particularly in patients with a vascular murmur and suspected high-flow malformation, due to its exceptional ability to delineate inflow and outflow anatomy noninvasively. Time-resolved MRA has been reported to rival conventional angiography for the portrayal of both functional flow dynamics and anatomic detail [9]. Combined conventional and dynamic contrast-enhanced MRA has a reported sensitivity of 83% and specificity of 95% for the differentiation of venous and nonvenous malformations [24]. Furthermore, MRA protocols typically include conventional high soft-tissue contrast T1 and T2 sequences which accurately assess the internal characteristics and extent of vascular malformations. This modality can also assess for and characterize other possible soft-tissue masses which may be included in the clinical differential diagnosis [6,7]. One potential weakness of time-resolved MRA is its underestimation of shunt volumes in vascular malformations which may be better evaluated with arteriography [7]. Arteriography Extremity Arteriography offers high temporal and spatial resolution images of the vascular anatomy associated with high- flow lesions such as arteriovenous malformations, including the inflow and outflow vessels as well as intralesional shunting [5,7]. Although the presence of a vascular murmur increases the suspicion of a high-flow Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch. acr.org/list.

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Headache Child PCAs

Introduction/Background Headache is a common complaint, even in early childhood. The prevalence of headaches increases with age and ranges from 37% to 51% for children 7 years of age and gradually increases to 57% to 82% by 15 years of age [1]. Prepubertal boys were found to commonly experience more headaches than girls, whereas after puberty, girls were more affected [2]. Headaches can be either primary or secondary in nature. Primary headaches result from the headache condition itself and not from another cause. A secondary headache is a headache that is present because of another condition. Diagnosis of primary headache disorders of children rests principally on clinical criteria as defined by the International Headache Society [3]. The evaluation of a child with headache begins with acquiring a thorough medical history and performing a physical examination with measurement of vital signs, including blood pressure, a complete neurologic examination, and examination of the optic discs. Secondary headache is more common in younger children [7,8]. Most of the secondary headaches have benign etiologies. A single episode of acute headache usually results from an acute infection ranging from viral upper respiratory illness to acute meningitis. Chronic progressive headaches often indicate a serious underlying abnormality, such as a brain tumor, and children with abnormal neurological findings should undergo neuroimaging. The clinical experiences of primary care physicians, pediatricians, and neurologists indicate that neuroimaging studies have a limited role in children with primary headaches [1]. The high prevalence of headaches and the low yield of imaging in pediatric patients presenting with headaches alone bring into question the value of screening for patients with primary headaches. Pediatric headache literature has repeatedly reported that the value of neuroimaging in children with headache is generally low [9-12].

Headache Child PCAs. Introduction/Background Headache is a common complaint, even in early childhood. The prevalence of headaches increases with age and ranges from 37% to 51% for children 7 years of age and gradually increases to 57% to 82% by 15 years of age [1]. Prepubertal boys were found to commonly experience more headaches than girls, whereas after puberty, girls were more affected [2]. Headaches can be either primary or secondary in nature. Primary headaches result from the headache condition itself and not from another cause. A secondary headache is a headache that is present because of another condition. Diagnosis of primary headache disorders of children rests principally on clinical criteria as defined by the International Headache Society [3]. The evaluation of a child with headache begins with acquiring a thorough medical history and performing a physical examination with measurement of vital signs, including blood pressure, a complete neurologic examination, and examination of the optic discs. Secondary headache is more common in younger children [7,8]. Most of the secondary headaches have benign etiologies. A single episode of acute headache usually results from an acute infection ranging from viral upper respiratory illness to acute meningitis. Chronic progressive headaches often indicate a serious underlying abnormality, such as a brain tumor, and children with abnormal neurological findings should undergo neuroimaging. The clinical experiences of primary care physicians, pediatricians, and neurologists indicate that neuroimaging studies have a limited role in children with primary headaches [1]. The high prevalence of headaches and the low yield of imaging in pediatric patients presenting with headaches alone bring into question the value of screening for patients with primary headaches. Pediatric headache literature has repeatedly reported that the value of neuroimaging in children with headache is generally low [9-12].

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acrac_69439_1

Headache Child PCAs

In a study by Yilmaz et al [12] of 449 children with headache, approximately 55% of children had migraine, 30% had tension-type headaches, 10% had secondary headaches, and 5% were unspecified. Twenty-one percent of imaged children (n = 324) had abnormalities identified on their magnetic resonance imaging (MRI) examinations, largely incidental findings, with <1% having relevant findings to explain the headache, namely tumor with hydrocephalus. Similarly, Martens et al [11] found that even though some neurological signs were present in a substantial number of children with primary headaches, mostly migraines, the yield of brain MRI scans was still low. Therefore, the yield of brain MRI is not contributory to the diagnostic and therapeutic approach in children with primary headaches. Based on analysis of a large body of evidence, the practice parameters authored by the American Academy of Neurology and Child Neurology Society recommend considering neuroimaging in children with an abnormal neurologic examination (eg, focal findings, signs of increased intracranial pressure, significant alteration of Reprint requests to: [email protected] Advanced imaging modalities such as computed tomography (CT) and MRI are preferred when neuroimaging in children is considered. CT exposes children to radiation, whereas MRI sometimes requires sedation or general anesthesia, especially in children <6 years of age. Therefore, neuroimaging should be reserved for children with a suspicious clinical history, abnormal neurological findings, or other physical signs suggestive of significant intracranial pathology [7,9]. CT In most cases, CT is usually not the study of choice for imaging children with headaches. However, there are some cases when a CT scan of the head is indicated because of its speed and sensitivity for detecting acute blood products.

Headache Child PCAs. In a study by Yilmaz et al [12] of 449 children with headache, approximately 55% of children had migraine, 30% had tension-type headaches, 10% had secondary headaches, and 5% were unspecified. Twenty-one percent of imaged children (n = 324) had abnormalities identified on their magnetic resonance imaging (MRI) examinations, largely incidental findings, with <1% having relevant findings to explain the headache, namely tumor with hydrocephalus. Similarly, Martens et al [11] found that even though some neurological signs were present in a substantial number of children with primary headaches, mostly migraines, the yield of brain MRI scans was still low. Therefore, the yield of brain MRI is not contributory to the diagnostic and therapeutic approach in children with primary headaches. Based on analysis of a large body of evidence, the practice parameters authored by the American Academy of Neurology and Child Neurology Society recommend considering neuroimaging in children with an abnormal neurologic examination (eg, focal findings, signs of increased intracranial pressure, significant alteration of Reprint requests to: [email protected] Advanced imaging modalities such as computed tomography (CT) and MRI are preferred when neuroimaging in children is considered. CT exposes children to radiation, whereas MRI sometimes requires sedation or general anesthesia, especially in children <6 years of age. Therefore, neuroimaging should be reserved for children with a suspicious clinical history, abnormal neurological findings, or other physical signs suggestive of significant intracranial pathology [7,9]. CT In most cases, CT is usually not the study of choice for imaging children with headaches. However, there are some cases when a CT scan of the head is indicated because of its speed and sensitivity for detecting acute blood products.

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acrac_69439_2

Headache Child PCAs

In the emergency setting, if a brain tumor is suspected, CT without IV contrast can be performed initially; however, a contrast-enhanced study may be indicated if it is not possible to perform an MRI scan of the brain. In patients with thunderclap headache, subarachnoid hemorrhage (SAH) from a ruptured aneurysm or arteriovenous malformation must be excluded; therefore, a noncontrast CT scan of the head is the imaging modality of choice as it is superior to MRI in detecting acute SAH [20]. If subarachnoid or parenchymal hemorrhage is detected, further evaluation for aneurysm or vascular malformation must be performed. This evaluation can be accomplished by CT angiography (CTA), conventional arteriography, or MR angiography (MRA) [21,22]. MRV MR venography (MRV) is the study of choice in children with suspected venous outflow stenosis, such as those with pseudotumor cerebri, or those with venous sinus thrombosis, such as mastoiditis. MRV can be performed with or without IV contrast. MRV with contrast can be helpful in the detection of intracranial sinovenous stenosis that can go undetected because of artifactual flow voids in the transverse sinuses on traditional noncontrast (time- of-flight) MRV [24]. CTV If MRV is not possible, or in cases in which the results of MRV are ambiguous, imaging with contrast-enhanced CT venography (CTV) has been found to be a fast, widely accessible alternative approach with high sensitivity and specificity in detecting venous sinus thrombosis [25]. MRV is generally preferred over CTV because of radiation concerns. MRA If subarachnoid or parenchymal hemorrhage is detected, further evaluation for aneurysm or vascular malformation must be performed. This evaluation can be accomplished by MRA, CTA, or conventional arteriography [21,22]. MRA can be performed without IV contrast and is easily added to a standard MRI study if a stroke or hemorrhage is detected.

Headache Child PCAs. In the emergency setting, if a brain tumor is suspected, CT without IV contrast can be performed initially; however, a contrast-enhanced study may be indicated if it is not possible to perform an MRI scan of the brain. In patients with thunderclap headache, subarachnoid hemorrhage (SAH) from a ruptured aneurysm or arteriovenous malformation must be excluded; therefore, a noncontrast CT scan of the head is the imaging modality of choice as it is superior to MRI in detecting acute SAH [20]. If subarachnoid or parenchymal hemorrhage is detected, further evaluation for aneurysm or vascular malformation must be performed. This evaluation can be accomplished by CT angiography (CTA), conventional arteriography, or MR angiography (MRA) [21,22]. MRV MR venography (MRV) is the study of choice in children with suspected venous outflow stenosis, such as those with pseudotumor cerebri, or those with venous sinus thrombosis, such as mastoiditis. MRV can be performed with or without IV contrast. MRV with contrast can be helpful in the detection of intracranial sinovenous stenosis that can go undetected because of artifactual flow voids in the transverse sinuses on traditional noncontrast (time- of-flight) MRV [24]. CTV If MRV is not possible, or in cases in which the results of MRV are ambiguous, imaging with contrast-enhanced CT venography (CTV) has been found to be a fast, widely accessible alternative approach with high sensitivity and specificity in detecting venous sinus thrombosis [25]. MRV is generally preferred over CTV because of radiation concerns. MRA If subarachnoid or parenchymal hemorrhage is detected, further evaluation for aneurysm or vascular malformation must be performed. This evaluation can be accomplished by MRA, CTA, or conventional arteriography [21,22]. MRA can be performed without IV contrast and is easily added to a standard MRI study if a stroke or hemorrhage is detected.

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Headache Child PCAs

If there is strong concern for arterial dissection within the head and/or neck, the diagnosis is generally made by MRI or MRA [26]. Arteriography In children with sudden onset of severe headache and a positive MRI or CT study demonstrating intracranial hemorrhage or stroke, digital subtraction arteriography can be performed. Arteriography is an invasive procedure that requires a skilled angiographer to be available emergently. Discussion of Procedures by Variant Variant 1: Child. Primary headache. Initial imaging. Radiography There is no role for radiography in patients with primary headache. MRI In a study by Yilmaz et al [12] of 449 children with headache, approximately 55% of children had migraine, 30% had tension-type headaches, 10% had secondary headaches, and 5% were unspecified. Twenty-one percent of imaged children (n = 324) had abnormalities identified on their MRI examinations, largely incidental findings, with <1% having relevant findings to explain the headache, namely tumor with hydrocephalus. Similarly, Martens et al [11] found that despite findings on neurological/physical examinations in a substantial number of children with headaches, mostly migraines, the yield of brain MRI scans was low. Therefore, the yield of brain MRI is not contributory to the diagnostic and therapeutic approach. In unusual circumstances when a complete physical examination is not possible or a thorough history is not available MRI could be considered. CTA There is no role for CTA in patients with primary headache and no concerning findings on clinical or physical examination. CTV There is no role for CTV in patients with primary headache and no concerning findings on clinical or physical examination. MRA There is no role for MRA in patients with primary headache and no concerning findings on clinical or physical examination. MRV There is no role for MRV in patients with primary headache and no concerning findings on clinical or physical examination.

Headache Child PCAs. If there is strong concern for arterial dissection within the head and/or neck, the diagnosis is generally made by MRI or MRA [26]. Arteriography In children with sudden onset of severe headache and a positive MRI or CT study demonstrating intracranial hemorrhage or stroke, digital subtraction arteriography can be performed. Arteriography is an invasive procedure that requires a skilled angiographer to be available emergently. Discussion of Procedures by Variant Variant 1: Child. Primary headache. Initial imaging. Radiography There is no role for radiography in patients with primary headache. MRI In a study by Yilmaz et al [12] of 449 children with headache, approximately 55% of children had migraine, 30% had tension-type headaches, 10% had secondary headaches, and 5% were unspecified. Twenty-one percent of imaged children (n = 324) had abnormalities identified on their MRI examinations, largely incidental findings, with <1% having relevant findings to explain the headache, namely tumor with hydrocephalus. Similarly, Martens et al [11] found that despite findings on neurological/physical examinations in a substantial number of children with headaches, mostly migraines, the yield of brain MRI scans was low. Therefore, the yield of brain MRI is not contributory to the diagnostic and therapeutic approach. In unusual circumstances when a complete physical examination is not possible or a thorough history is not available MRI could be considered. CTA There is no role for CTA in patients with primary headache and no concerning findings on clinical or physical examination. CTV There is no role for CTV in patients with primary headache and no concerning findings on clinical or physical examination. MRA There is no role for MRA in patients with primary headache and no concerning findings on clinical or physical examination. MRV There is no role for MRV in patients with primary headache and no concerning findings on clinical or physical examination.

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Headache Child PCAs

The use of contrast in MRV depends on institutional preferences. Arteriography There is no role for arteriography in patients with primary headache and no concerning findings on clinical or physical examination. Another diagnosis to consider in patients with headache and papilledema is pseudotumor cerebri, also known as pseudotumor cerebri syndrome (PTCS). Primary PTCS is also known as idiopathic intracranial hypertension. This disorder typically manifests as severe headaches and visual impairments and prevails in overweight females of childbearing age but can occur in obese males and prepubertal thin girls and boys. Its incidence is rising in parallel with the obesity epidemic. The etiology of pseudotumor cerebri is unclear, with impaired cerebrospinal fluid (CSF) homeostasis and altered venous hemodynamics the proposed mechanisms for elevated intracranial pressure. A study by Alperin et al [33] supported these mechanisms by demonstrating a reduced relative cerebral drainage through the internal jugular vein with an increased intracranial CSF volume that accumulates in the subarachnoid space. Secondary PTCS is a result of cerebral venous abnormalities such as thrombosis, medications such as vitamin A, and medical disorders such as endocrinopathies [34]. In cases of suspected PTCS, MRI of the brain with and without contrast should be performed as MRI is more sensitive for detection of secondary signs of increased intracranial pressure such as an empty sella, dilated optic sheaths, tortuous or enhancing optic nerves, and flattening of the posterior aspects of the globes. MRI reveals more details of the intracranial structures without radiation and is better able to evaluate for meningeal infiltration and isodense tumors over CT. In patients without PTCS, MRI should reveal normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion and no abnormal meningeal enhancement.

Headache Child PCAs. The use of contrast in MRV depends on institutional preferences. Arteriography There is no role for arteriography in patients with primary headache and no concerning findings on clinical or physical examination. Another diagnosis to consider in patients with headache and papilledema is pseudotumor cerebri, also known as pseudotumor cerebri syndrome (PTCS). Primary PTCS is also known as idiopathic intracranial hypertension. This disorder typically manifests as severe headaches and visual impairments and prevails in overweight females of childbearing age but can occur in obese males and prepubertal thin girls and boys. Its incidence is rising in parallel with the obesity epidemic. The etiology of pseudotumor cerebri is unclear, with impaired cerebrospinal fluid (CSF) homeostasis and altered venous hemodynamics the proposed mechanisms for elevated intracranial pressure. A study by Alperin et al [33] supported these mechanisms by demonstrating a reduced relative cerebral drainage through the internal jugular vein with an increased intracranial CSF volume that accumulates in the subarachnoid space. Secondary PTCS is a result of cerebral venous abnormalities such as thrombosis, medications such as vitamin A, and medical disorders such as endocrinopathies [34]. In cases of suspected PTCS, MRI of the brain with and without contrast should be performed as MRI is more sensitive for detection of secondary signs of increased intracranial pressure such as an empty sella, dilated optic sheaths, tortuous or enhancing optic nerves, and flattening of the posterior aspects of the globes. MRI reveals more details of the intracranial structures without radiation and is better able to evaluate for meningeal infiltration and isodense tumors over CT. In patients without PTCS, MRI should reveal normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion and no abnormal meningeal enhancement.

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Headache Child PCAs

It is important to note that meningeal enhancement can be seen on MRI following lumbar puncture and should not be confused with pathology. Imaging of the orbits including a coronal, fat-saturated T2-weighted sequence is recommended to better evaluate for dilatation of the optic sheaths [24]. In patients in whom there is high suspicion for Chiari I deformity, a noncontrast MRI scan of the brain to include a sagittal T2-weighted sequence of the craniocervical junction with optional phase-contrast CSF flow study at the craniocervical junction is the study of choice. The Chiari I deformity is a condition characterized by the herniation of the cerebellar tonsils through the foramen magnum with headache as its most common symptom in older children [35,36]. In children <3 years of age, abnormal oropharyngeal function is commonly demonstrated. In children >3 years of age, scoliosis (associated with syringohydromyelia) or headache worsened by the Valsalva maneuver are typical findings. Most literature agrees that occipital headache in children is rare and calls for diagnostic caution; however, isolated occipital and cervical pain are not characteristic symptoms of any headache group in the pediatric age group, and their presence or absence does not correspond to changes on conventional brain MRI [37]. Children with sickle cell anemia are a special subgroup of patients who require particular attention as recurrent headaches and migraines in these children are common and undertreated [17]. Low hemoglobin levels and high pain rates are associated with recurrent headaches and migraines, whereas silent cerebral infarction is not. However, acute headache in children with sickle cell anemia is more frequently associated with acute central nervous system events than in the general pediatric population, so the threshold to image these patients should be lower.

Headache Child PCAs. It is important to note that meningeal enhancement can be seen on MRI following lumbar puncture and should not be confused with pathology. Imaging of the orbits including a coronal, fat-saturated T2-weighted sequence is recommended to better evaluate for dilatation of the optic sheaths [24]. In patients in whom there is high suspicion for Chiari I deformity, a noncontrast MRI scan of the brain to include a sagittal T2-weighted sequence of the craniocervical junction with optional phase-contrast CSF flow study at the craniocervical junction is the study of choice. The Chiari I deformity is a condition characterized by the herniation of the cerebellar tonsils through the foramen magnum with headache as its most common symptom in older children [35,36]. In children <3 years of age, abnormal oropharyngeal function is commonly demonstrated. In children >3 years of age, scoliosis (associated with syringohydromyelia) or headache worsened by the Valsalva maneuver are typical findings. Most literature agrees that occipital headache in children is rare and calls for diagnostic caution; however, isolated occipital and cervical pain are not characteristic symptoms of any headache group in the pediatric age group, and their presence or absence does not correspond to changes on conventional brain MRI [37]. Children with sickle cell anemia are a special subgroup of patients who require particular attention as recurrent headaches and migraines in these children are common and undertreated [17]. Low hemoglobin levels and high pain rates are associated with recurrent headaches and migraines, whereas silent cerebral infarction is not. However, acute headache in children with sickle cell anemia is more frequently associated with acute central nervous system events than in the general pediatric population, so the threshold to image these patients should be lower.

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acrac_69439_6

Headache Child PCAs

These children are at risk for posterior reversible encephalopathy syndrome, especially after a bone marrow transplant, and for SAH, especially in the setting of arterial aneurysm. A history of stroke, transient ischemic attack, seizures, neurological symptoms, focal neurological examination, or elevated platelet counts at presentation warrants confirmatory imaging studies [38]. MRI is the imaging modality of choice in these children because of its superior sensitivity for infarction and other parenchymal abnormalities. Seizures are one of the most common secondary etiologies for headache and often have auras similar to some migraines [39]. MRI without IV contrast is indicated in the evaluation of patients with seizures. CTA If an acute stroke is suspected, CTA in conjunction with a noncontrast CT scan of the head is indicated, with MRI/MRA the preferred modality because of its greater sensitivity in detecting acute stroke versus CT. CT should not be delayed if MRI is not available or feasible. CTA of the head and neck are usually indicated if there is strong suspicion for arterial dissection. If MRA is performed initially to evaluate for arterial dissection and is inconclusive, CTA may be helpful for further evaluation. CTV If there is concern for venous outflow obstruction, such as in the setting of venous sinus thrombosis or PTCS, CTV has been found to be an alternative approach with high sensitivity and specificity in detecting venous sinus thrombosis compared with MRV [25]. MRV remains the imaging study of choice over CTV in children. MRA MRI is more sensitive for detecting early changes of a stroke, and a concurrent MRA plays an important role in stroke imaging. MRA is indicated for children with sickle cell anemia in the setting of headache. MRV In conjunction with MRI, MRV is indicated in patients with possible venous sinus abnormalities, such as those with suspected PTCS. Decreased spinal canal compliance has been identified in patients with PTCS [40].

Headache Child PCAs. These children are at risk for posterior reversible encephalopathy syndrome, especially after a bone marrow transplant, and for SAH, especially in the setting of arterial aneurysm. A history of stroke, transient ischemic attack, seizures, neurological symptoms, focal neurological examination, or elevated platelet counts at presentation warrants confirmatory imaging studies [38]. MRI is the imaging modality of choice in these children because of its superior sensitivity for infarction and other parenchymal abnormalities. Seizures are one of the most common secondary etiologies for headache and often have auras similar to some migraines [39]. MRI without IV contrast is indicated in the evaluation of patients with seizures. CTA If an acute stroke is suspected, CTA in conjunction with a noncontrast CT scan of the head is indicated, with MRI/MRA the preferred modality because of its greater sensitivity in detecting acute stroke versus CT. CT should not be delayed if MRI is not available or feasible. CTA of the head and neck are usually indicated if there is strong suspicion for arterial dissection. If MRA is performed initially to evaluate for arterial dissection and is inconclusive, CTA may be helpful for further evaluation. CTV If there is concern for venous outflow obstruction, such as in the setting of venous sinus thrombosis or PTCS, CTV has been found to be an alternative approach with high sensitivity and specificity in detecting venous sinus thrombosis compared with MRV [25]. MRV remains the imaging study of choice over CTV in children. MRA MRI is more sensitive for detecting early changes of a stroke, and a concurrent MRA plays an important role in stroke imaging. MRA is indicated for children with sickle cell anemia in the setting of headache. MRV In conjunction with MRI, MRV is indicated in patients with possible venous sinus abnormalities, such as those with suspected PTCS. Decreased spinal canal compliance has been identified in patients with PTCS [40].

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acrac_69439_7

Headache Child PCAs

A study by Dwyer et al [41] that reviewed more than 200 MRVs in suspected cases of pseudotumor cerebri found that 52% of scans showed evidence of venous obstruction in the dominant side of venous circulation. This was statistically higher than in control groups. It is important to note that reversibility of venous outflow obstruction can be seen on MRV in these patients following lumbar puncture, which argues that the presence of venous outflow obstruction could be secondary to the increased intracranial pressure itself [42]. When cerebellar tonsillar ectopia of >5 mm is identified, imaging and clinical consideration of PTCS are warranted to avoid misdiagnosis as Chiari I [43]. In addition to the initial MRV in patients with suspected PTCS, a second MRV following CSF drainage may be helpful. Venous sinus occlusion and arteriovenous fistulas may produce PTCS. MRV is indicated when there is concern for venous sinus thrombosis, especially in children with intracranial extension of infection. Children with mastoiditis are at a particularly high risk for venous sinus thrombosis. Girls using oral contraceptives are also at increased risk for thrombosis. The use of contrast in MRV depends on institutional preferences. Contrast-enhanced MRV may be helpful when evaluating areas such as the sigmoid venous sinuses, a location often degraded by artifact on noncontrast MRVs. Arteriography In patients with evidence for stroke on CT or MRA, arteriography may be helpful for further evaluation, especially when intervention such as thrombolysis or treatment of vascular malformations is considered. Arteriography is also more sensitive in detecting small vessel disease and arterial dissection and may be a useful examination if results of MRA or CTA are unclear and there is strong suspicion for such. Variant 3: Child. Sudden severe headache (thunderclap headache). Initial imaging. Radiography There is no role for radiography in children with sudden severe headache.

Headache Child PCAs. A study by Dwyer et al [41] that reviewed more than 200 MRVs in suspected cases of pseudotumor cerebri found that 52% of scans showed evidence of venous obstruction in the dominant side of venous circulation. This was statistically higher than in control groups. It is important to note that reversibility of venous outflow obstruction can be seen on MRV in these patients following lumbar puncture, which argues that the presence of venous outflow obstruction could be secondary to the increased intracranial pressure itself [42]. When cerebellar tonsillar ectopia of >5 mm is identified, imaging and clinical consideration of PTCS are warranted to avoid misdiagnosis as Chiari I [43]. In addition to the initial MRV in patients with suspected PTCS, a second MRV following CSF drainage may be helpful. Venous sinus occlusion and arteriovenous fistulas may produce PTCS. MRV is indicated when there is concern for venous sinus thrombosis, especially in children with intracranial extension of infection. Children with mastoiditis are at a particularly high risk for venous sinus thrombosis. Girls using oral contraceptives are also at increased risk for thrombosis. The use of contrast in MRV depends on institutional preferences. Contrast-enhanced MRV may be helpful when evaluating areas such as the sigmoid venous sinuses, a location often degraded by artifact on noncontrast MRVs. Arteriography In patients with evidence for stroke on CT or MRA, arteriography may be helpful for further evaluation, especially when intervention such as thrombolysis or treatment of vascular malformations is considered. Arteriography is also more sensitive in detecting small vessel disease and arterial dissection and may be a useful examination if results of MRA or CTA are unclear and there is strong suspicion for such. Variant 3: Child. Sudden severe headache (thunderclap headache). Initial imaging. Radiography There is no role for radiography in children with sudden severe headache.

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Headache Child PCAs

Severe sudden headaches can be associated with SAH and intracranial hemorrhage that may occur with aneurysms or other vascular malformations, such as AVMs and cavernomas. Neuroimaging of children with severe or unusual head pain who have a first-degree relative with an aneurysm or other vascular abnormality is indicated, as these vascular pathologies can be familial but are otherwise uncommon [39]. The cornerstone for the diagnosis of SAH is a noncontrast CT scan; however, the use of MRI techniques such as proton-density-weighted imaging, susceptibility-weighted imaging (SWI)/gradient-recalled echo (GRE) imaging, or T2-weighted fluid- attenuated inversion recovery (FLAIR) imaging improves the diagnosis of acute SAH, as conventional sequences are insensitive to the finding [21]. A study by Mitchell et al [49] found that sensitivity to SAH varied among MR sequence from 50% to 94% in acute SAH and from 33% to 100% in subacute SAH. The most sensitive sequences were FLAIR and SWI/GRE. It is important to note that signal in the sulci on the FLAIR sequence can be artifactually increased in children receiving propofol and supplemental oxygenation and can mimic SAH. Meningitis can also give this appearance. CT In the acute setting, noncontrast CT is indicated in the evaluation of acute thunderclap headache. The sensitivity of CT for the detection of acute SAH is greater than MRI at 98% with a specificity of 99% [50]. CT is often the initial imaging study of choice because of availability and lack of need for sedation. CTV Except in cases of thunderclap headache related to an AVM, CTV is usually not indicated in patients with thunderclap headache. MRV Except in cases of thunderclap headache related to an AVM, MRV is usually not indicated in patients with thunderclap headache. The use of contrast in MRV depends on institutional preferences.

Headache Child PCAs. Severe sudden headaches can be associated with SAH and intracranial hemorrhage that may occur with aneurysms or other vascular malformations, such as AVMs and cavernomas. Neuroimaging of children with severe or unusual head pain who have a first-degree relative with an aneurysm or other vascular abnormality is indicated, as these vascular pathologies can be familial but are otherwise uncommon [39]. The cornerstone for the diagnosis of SAH is a noncontrast CT scan; however, the use of MRI techniques such as proton-density-weighted imaging, susceptibility-weighted imaging (SWI)/gradient-recalled echo (GRE) imaging, or T2-weighted fluid- attenuated inversion recovery (FLAIR) imaging improves the diagnosis of acute SAH, as conventional sequences are insensitive to the finding [21]. A study by Mitchell et al [49] found that sensitivity to SAH varied among MR sequence from 50% to 94% in acute SAH and from 33% to 100% in subacute SAH. The most sensitive sequences were FLAIR and SWI/GRE. It is important to note that signal in the sulci on the FLAIR sequence can be artifactually increased in children receiving propofol and supplemental oxygenation and can mimic SAH. Meningitis can also give this appearance. CT In the acute setting, noncontrast CT is indicated in the evaluation of acute thunderclap headache. The sensitivity of CT for the detection of acute SAH is greater than MRI at 98% with a specificity of 99% [50]. CT is often the initial imaging study of choice because of availability and lack of need for sedation. CTV Except in cases of thunderclap headache related to an AVM, CTV is usually not indicated in patients with thunderclap headache. MRV Except in cases of thunderclap headache related to an AVM, MRV is usually not indicated in patients with thunderclap headache. The use of contrast in MRV depends on institutional preferences.

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Headache Child PCAs

Arteriography As an invasive and often unavailable study, arteriography is rarely the initial angiographic evaluation performed in children with thunderclap headache. A study in 2011 by Sabri et al [47] evaluated patients that presented with intracranial hemorrhage, predominantly SAH. Their findings showed that the yield from CTA and arteriography are relatively comparable, but that arteriography is superior in detection of aneurysm. Hence, in cases in which the CTA result was found to be normal despite high suspicion for lesion in the setting of SAH, a follow-up CTA or arteriography is considered useful. However, use of CTA over arteriography has been controversial. In 2007, Kallmes et al [45] declared that because both negative and positive CTA scans mandate subsequent conventional angiography, the CTA should be dispensed with and patients should proceed directly to arteriography. Furthermore, Moran et al [46] declared that conventional angiography with arteriography is the ideal method for imaging these patients because of its ability to detect aneurysms quickly, reliably, and safely and that it guides the prompt proper therapy. The applicability of these adult-based studies to the pediatric population is debatable. MRI In a study by Lateef et al [1], the overwhelming majority of acute headaches in children and adolescents were attributable to common, minor, transient conditions, such as upper respiratory illness. Headache is the most common symptom identified with the intracranial spread of infection resulting from dural irritation and localized encephalitis. The headache can be attributed to either intracranial or extracranial infections. In the setting of suspected intracranial infection, the need for neuroimaging is guided by laboratory tests and clinical signs [51]. Clinical signs suggesting intracranial abnormality include high fever and change in mental status with and without focal signs.

Headache Child PCAs. Arteriography As an invasive and often unavailable study, arteriography is rarely the initial angiographic evaluation performed in children with thunderclap headache. A study in 2011 by Sabri et al [47] evaluated patients that presented with intracranial hemorrhage, predominantly SAH. Their findings showed that the yield from CTA and arteriography are relatively comparable, but that arteriography is superior in detection of aneurysm. Hence, in cases in which the CTA result was found to be normal despite high suspicion for lesion in the setting of SAH, a follow-up CTA or arteriography is considered useful. However, use of CTA over arteriography has been controversial. In 2007, Kallmes et al [45] declared that because both negative and positive CTA scans mandate subsequent conventional angiography, the CTA should be dispensed with and patients should proceed directly to arteriography. Furthermore, Moran et al [46] declared that conventional angiography with arteriography is the ideal method for imaging these patients because of its ability to detect aneurysms quickly, reliably, and safely and that it guides the prompt proper therapy. The applicability of these adult-based studies to the pediatric population is debatable. MRI In a study by Lateef et al [1], the overwhelming majority of acute headaches in children and adolescents were attributable to common, minor, transient conditions, such as upper respiratory illness. Headache is the most common symptom identified with the intracranial spread of infection resulting from dural irritation and localized encephalitis. The headache can be attributed to either intracranial or extracranial infections. In the setting of suspected intracranial infection, the need for neuroimaging is guided by laboratory tests and clinical signs [51]. Clinical signs suggesting intracranial abnormality include high fever and change in mental status with and without focal signs.

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Headache Child PCAs

Neurologic signs and symptoms such as nuchal rigidity or alteration in consciousness may be indications for imaging. Symptoms in infants may be nonspecific, including fever, poor feeding, irritability, and lethargy. Seizures are not uncommon in these young children, mostly occurring when the inflammation has progressed to involve the brain parenchyma. Older children may have fever, headache, nausea, vomiting, confusion, stiff neck, and photophobia. Symptoms of viral meningitis can resemble those of the flu. An MRI scan of the brain is indicated in patients with signs of intracranial infection with headache. MRI with and without IV contrast is indicated in the evaluation of intracranial infections that include meningitis, encephalitis, and brain abscess. MRI may improve the sensitivity for detecting encephalitis as T2 FLAIR is sensitive for vasogenic edema, diffusion-weighted imaging is sensitive for cytotoxic edema, and postcontrast T1 and T2 FLAIR sequences are sensitive for meningeal enhancement. The combination of MRI sequences can be very helpful to exclude mimics of encephalitis, identify the extent of inflammation, and confirm if lesion distribution is concordant with symptoms [52]. The distribution of abnormalities on MRI can help guide in determining the pathogen in some cases. For instance, brainstem and spinal cord involvement is common with enterovirus, and basal ganglia/thalamic involvement is common with West Nile virus or Japanese encephalitis. It is important to note that the classic limbic distribution of herpes simplex virus-1 may not always be present, and that extratemporal involvement is not uncommon [52]. Extracranial infections, including subdural empyemas (SDE) and epidural empyemas, can also be well evaluated with MRI. Epidural empyemas are collections of suppurative fluid located between the skull and dura.

Headache Child PCAs. Neurologic signs and symptoms such as nuchal rigidity or alteration in consciousness may be indications for imaging. Symptoms in infants may be nonspecific, including fever, poor feeding, irritability, and lethargy. Seizures are not uncommon in these young children, mostly occurring when the inflammation has progressed to involve the brain parenchyma. Older children may have fever, headache, nausea, vomiting, confusion, stiff neck, and photophobia. Symptoms of viral meningitis can resemble those of the flu. An MRI scan of the brain is indicated in patients with signs of intracranial infection with headache. MRI with and without IV contrast is indicated in the evaluation of intracranial infections that include meningitis, encephalitis, and brain abscess. MRI may improve the sensitivity for detecting encephalitis as T2 FLAIR is sensitive for vasogenic edema, diffusion-weighted imaging is sensitive for cytotoxic edema, and postcontrast T1 and T2 FLAIR sequences are sensitive for meningeal enhancement. The combination of MRI sequences can be very helpful to exclude mimics of encephalitis, identify the extent of inflammation, and confirm if lesion distribution is concordant with symptoms [52]. The distribution of abnormalities on MRI can help guide in determining the pathogen in some cases. For instance, brainstem and spinal cord involvement is common with enterovirus, and basal ganglia/thalamic involvement is common with West Nile virus or Japanese encephalitis. It is important to note that the classic limbic distribution of herpes simplex virus-1 may not always be present, and that extratemporal involvement is not uncommon [52]. Extracranial infections, including subdural empyemas (SDE) and epidural empyemas, can also be well evaluated with MRI. Epidural empyemas are collections of suppurative fluid located between the skull and dura.

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acrac_69439_11

Headache Child PCAs

In infants, SDE is most commonly a complication of purulent meningitis, whereas in older children the source of SDE is typically direct extension of sinusitis or otitis media into the extracranial spaces. MRI can help identify epidural empyemas because of its ability to distinguish between different types of fluid, especially with use of diffusion- weighted imaging. Acute meningitis is a common neurological emergency and the diagnosis is usually made based on clinical and laboratory findings. CT Neuroimaging is reserved for specific adverse features, such as prompt diagnosis of SAH, or underlying causes, such as mastoiditis. Neurologic signs and symptoms such as nuchal rigidity or alteration in consciousness may be indications for imaging with CT. However, the sensitivity of CT in diagnosing pediatric encephalitis in comparison to MRI is generally poor [52]. In the emergency setting, CT may be indicated in evaluating children with suspected intracranial infection, often performed prior to lumbar puncture. IV contrast is recommended in these patients if MRI is not rapidly available. A negative noncontrast CT scan of the head should not conclude the evaluation for suspected encephalitis. In a study by Bykowski et al [52], cranial CTs were the initial study in 94 patients, and abnormal findings were present in 22. An additional 26 children had a normal acute CT and abnormal findings identified on MRI performed within 2 days [52]. CTA The role for CTA is limited in children with headache attributed to infection unless SAH or stroke is suspected and MRI/MRA is not possible. CTV As children with mastoiditis are at particularly high risk for venous sinus thrombosis, CTV may be helpful in the evaluation of these patients. Children with sphenoid sinusitis are also at risk for cavernous sinus thrombosis, and CTV may be helpful in these patients. MRA The role for MRA is limited in children with headache attributed to infection unless SAH or stroke is suspected.

Headache Child PCAs. In infants, SDE is most commonly a complication of purulent meningitis, whereas in older children the source of SDE is typically direct extension of sinusitis or otitis media into the extracranial spaces. MRI can help identify epidural empyemas because of its ability to distinguish between different types of fluid, especially with use of diffusion- weighted imaging. Acute meningitis is a common neurological emergency and the diagnosis is usually made based on clinical and laboratory findings. CT Neuroimaging is reserved for specific adverse features, such as prompt diagnosis of SAH, or underlying causes, such as mastoiditis. Neurologic signs and symptoms such as nuchal rigidity or alteration in consciousness may be indications for imaging with CT. However, the sensitivity of CT in diagnosing pediatric encephalitis in comparison to MRI is generally poor [52]. In the emergency setting, CT may be indicated in evaluating children with suspected intracranial infection, often performed prior to lumbar puncture. IV contrast is recommended in these patients if MRI is not rapidly available. A negative noncontrast CT scan of the head should not conclude the evaluation for suspected encephalitis. In a study by Bykowski et al [52], cranial CTs were the initial study in 94 patients, and abnormal findings were present in 22. An additional 26 children had a normal acute CT and abnormal findings identified on MRI performed within 2 days [52]. CTA The role for CTA is limited in children with headache attributed to infection unless SAH or stroke is suspected and MRI/MRA is not possible. CTV As children with mastoiditis are at particularly high risk for venous sinus thrombosis, CTV may be helpful in the evaluation of these patients. Children with sphenoid sinusitis are also at risk for cavernous sinus thrombosis, and CTV may be helpful in these patients. MRA The role for MRA is limited in children with headache attributed to infection unless SAH or stroke is suspected.

69439

acrac_69439_12

Headache Child PCAs

If arteritis is suspected, as can be seen in the setting of sphenoid sinusitis and skull base osteomyelitis, MRA may be helpful. MRV If venous sinus thrombosis is suspected, MRV is indicated. It should be noted, however, that in some cases of infection-induced venous sinus or cavernous sinus thrombosis, contrast-enhanced MRI could be superior to MRV as it shows the cross-sectional area of the vein with direct delineation of the thrombus itself and not just the absence of flow in the lumen, as seen on MRV [53]. The use of contrast in MRV depends on institutional preferences. Arteriography There is usually no role for arteriography in the evaluation of children with headache related to infection. MRI Patients who have a history of subacute or remote trauma may present with headaches. Post-traumatic headache is defined as a headache that begins within 2 weeks of a closed head injury. A prospective study of children admitted with a closed head injury (minor 79%, major 21%) found that 7% of children reported chronic post- traumatic headaches, 4% had episodic tension-type headaches, and 2.5% had migraine without aura [54]. When neurologic signs or symptoms are positive, when headaches are associated with vomiting, or when headaches are increasing in frequency, duration, or severity, regardless of the severity of the initial trauma, neuroimaging, preferably with noncontrast MRI, is indicated. SWI or GRE imaging is helpful in identifying hemosiderin deposition related to prior hemorrhage and should be included in the MRI examination. These sequences are limited because of susceptibility artifact in children with orthodontic braces or other metallic hardware, especially on higher Tesla strength MRI scanners. CT CT is usually not indicated in children with headaches attributed to remote trauma. A retrospective study by Dayan et al [55] identified 2,462 children who had minor blunt head trauma and headaches as their only symptom.

Headache Child PCAs. If arteritis is suspected, as can be seen in the setting of sphenoid sinusitis and skull base osteomyelitis, MRA may be helpful. MRV If venous sinus thrombosis is suspected, MRV is indicated. It should be noted, however, that in some cases of infection-induced venous sinus or cavernous sinus thrombosis, contrast-enhanced MRI could be superior to MRV as it shows the cross-sectional area of the vein with direct delineation of the thrombus itself and not just the absence of flow in the lumen, as seen on MRV [53]. The use of contrast in MRV depends on institutional preferences. Arteriography There is usually no role for arteriography in the evaluation of children with headache related to infection. MRI Patients who have a history of subacute or remote trauma may present with headaches. Post-traumatic headache is defined as a headache that begins within 2 weeks of a closed head injury. A prospective study of children admitted with a closed head injury (minor 79%, major 21%) found that 7% of children reported chronic post- traumatic headaches, 4% had episodic tension-type headaches, and 2.5% had migraine without aura [54]. When neurologic signs or symptoms are positive, when headaches are associated with vomiting, or when headaches are increasing in frequency, duration, or severity, regardless of the severity of the initial trauma, neuroimaging, preferably with noncontrast MRI, is indicated. SWI or GRE imaging is helpful in identifying hemosiderin deposition related to prior hemorrhage and should be included in the MRI examination. These sequences are limited because of susceptibility artifact in children with orthodontic braces or other metallic hardware, especially on higher Tesla strength MRI scanners. CT CT is usually not indicated in children with headaches attributed to remote trauma. A retrospective study by Dayan et al [55] identified 2,462 children who had minor blunt head trauma and headaches as their only symptom.

69439

acrac_3093336_0

Female Infertility

Introduction/Background Overall, infertility affects about 15.5% of women [1]. Infertility investigations are generally initiated after 12 months of unprotected intercourse without resultant pregnancy in women <35 years of age, whereas they are initiated after 6 months of unprotected intercourse without pregnancy in women >35 years of age. An investigation may commence sooner in couples with a known condition or medical history predisposing to infertility. The most common known causes of infertility are male factor (26%), ovulatory failure (21%), and tubal damage (14%), although in 28% of couples infertility remains unexplained [2]. Female-specific causes of infertility include deterioration of oocyte quality with increasing maternal age; ovulatory disorders, most notably polycystic ovarian syndrome (PCOS); history of salpingitis, such as that caused by chlamydia infection; endometriosis; and uterine cavity abnormalities interfering with implantation causing inability to become pregnant or causing recurrent pregnancy loss [3]. These potential causes of female infertility will be discussed here. Imaging can be used to count ovarian follicles and help determine ovarian reserve, particularly in advanced maternal age. Imaging can also be useful in diagnosing polycystic ovarian morphology (PCOM) in women suspected of having PCOS. PCOS is the leading cause of anovulatory infertility, affecting at least 7% of adult women [4]. Women with PCOS experience hyperandrogenism, infertility, and have PCOM defined as >25 small follicles in at least one ovary or a single ovarian volume >10 mL on transvaginal ultrasound (TVUS) [5]. Because PCOM may be present in up to one-third of reproductive-aged women [6], the imaging findings of PCOM are not sufficient for the diagnosis of PCOS but serve to support the diagnosis of PCOS in women with the clinical features. Endometriosis affects at least one-third of women with infertility and up to 10% of reproductive-aged women [7].

Female Infertility. Introduction/Background Overall, infertility affects about 15.5% of women [1]. Infertility investigations are generally initiated after 12 months of unprotected intercourse without resultant pregnancy in women <35 years of age, whereas they are initiated after 6 months of unprotected intercourse without pregnancy in women >35 years of age. An investigation may commence sooner in couples with a known condition or medical history predisposing to infertility. The most common known causes of infertility are male factor (26%), ovulatory failure (21%), and tubal damage (14%), although in 28% of couples infertility remains unexplained [2]. Female-specific causes of infertility include deterioration of oocyte quality with increasing maternal age; ovulatory disorders, most notably polycystic ovarian syndrome (PCOS); history of salpingitis, such as that caused by chlamydia infection; endometriosis; and uterine cavity abnormalities interfering with implantation causing inability to become pregnant or causing recurrent pregnancy loss [3]. These potential causes of female infertility will be discussed here. Imaging can be used to count ovarian follicles and help determine ovarian reserve, particularly in advanced maternal age. Imaging can also be useful in diagnosing polycystic ovarian morphology (PCOM) in women suspected of having PCOS. PCOS is the leading cause of anovulatory infertility, affecting at least 7% of adult women [4]. Women with PCOS experience hyperandrogenism, infertility, and have PCOM defined as >25 small follicles in at least one ovary or a single ovarian volume >10 mL on transvaginal ultrasound (TVUS) [5]. Because PCOM may be present in up to one-third of reproductive-aged women [6], the imaging findings of PCOM are not sufficient for the diagnosis of PCOS but serve to support the diagnosis of PCOS in women with the clinical features. Endometriosis affects at least one-third of women with infertility and up to 10% of reproductive-aged women [7].

3093336

acrac_3093336_1

Female Infertility

Although endometriosis is associated with infertility, the mechanism is unclear [8]. Imaging is useful in characterizing some features of endometriosis; however, small endometrial implants are not well detected on imaging. Therefore, laparoscopy remains the standard for both diagnosis and staging of endometriosis [9,10]. Women with a history of pelvic infection or surgery may develop intrauterine synechiae, fallopian tube abnormalities that include occlusion, and peritubular adhesions [11], potentially causing infertility. Imaging evaluation in these women focuses primarily on the fallopian tubes. Reprint requests to: [email protected] Female Infertility adhesions cause the loss of pregnancy [17]. Although hysteroscopy is the reference standard for visualizing intrauterine adhesions [18], imaging examinations can aid in making this diagnosis with a less invasive approach. Uterine fibroids are suspected to be related to recurrent pregnancy loss; however, there is insufficient evidence to confirm a causal relationship. Uterine adenomyosis, although previously felt to be more common in multiparous women, has now been associated with increased rates of spontaneous abortion and implantation failure [19]. In approximately 18% of women, a uterine abnormality such as fibroids or MDA is identified as a causative factor for recurrent pregnancy loss [20]. Special Imaging Considerations MR hysterosalpingography (HSG) is an additional technique that can demonstrate tubal patency and may be useful in women in whom both MRI and HSG need to be performed [23]; however, it remains an investigational tool [24]. US Pelvis Transabdominal Although transabdominal US and TVUS may be performed together for evaluation of the ovaries, transabdominal US should be relied upon only if the ovaries are not adequately evaluated via a transvaginal approach. US Pelvis Transvaginal TVUS can be used to monitor follicle development [27], perform antral follicle counts [28], and measure ovarian volume.

Female Infertility. Although endometriosis is associated with infertility, the mechanism is unclear [8]. Imaging is useful in characterizing some features of endometriosis; however, small endometrial implants are not well detected on imaging. Therefore, laparoscopy remains the standard for both diagnosis and staging of endometriosis [9,10]. Women with a history of pelvic infection or surgery may develop intrauterine synechiae, fallopian tube abnormalities that include occlusion, and peritubular adhesions [11], potentially causing infertility. Imaging evaluation in these women focuses primarily on the fallopian tubes. Reprint requests to: [email protected] Female Infertility adhesions cause the loss of pregnancy [17]. Although hysteroscopy is the reference standard for visualizing intrauterine adhesions [18], imaging examinations can aid in making this diagnosis with a less invasive approach. Uterine fibroids are suspected to be related to recurrent pregnancy loss; however, there is insufficient evidence to confirm a causal relationship. Uterine adenomyosis, although previously felt to be more common in multiparous women, has now been associated with increased rates of spontaneous abortion and implantation failure [19]. In approximately 18% of women, a uterine abnormality such as fibroids or MDA is identified as a causative factor for recurrent pregnancy loss [20]. Special Imaging Considerations MR hysterosalpingography (HSG) is an additional technique that can demonstrate tubal patency and may be useful in women in whom both MRI and HSG need to be performed [23]; however, it remains an investigational tool [24]. US Pelvis Transabdominal Although transabdominal US and TVUS may be performed together for evaluation of the ovaries, transabdominal US should be relied upon only if the ovaries are not adequately evaluated via a transvaginal approach. US Pelvis Transvaginal TVUS can be used to monitor follicle development [27], perform antral follicle counts [28], and measure ovarian volume.

3093336

acrac_3093336_2

Female Infertility

When the ovarian volume is <3 cm3 and <5 antral follicles are present, this suggests diminished ovarian reserve [29]. MRI Pelvis A recent study in obese adolescents with suspected PCOS demonstrated that MRI without IV contrast can provide reproducible and reliable ovarian volume assessment; however, ovarian follicle counts had only moderate interobserver agreement. Nonetheless, in the obese adolescent or patient in whom TVUS is unacceptable and transabdominal US limited, MRI may provide additional information on PCOM [25,26,30]. US Pelvis Color Doppler Although increased central stromal vascularity has been demonstrated in the ovaries of women with PCOS, there is limited evidence to indicate color Doppler should be performed routinely in the evaluation of PCOM [31]. Female Infertility US Pelvis Transabdominal Transabdominal US is often performed in conjunction with TVUS; however, in some settings it may be performed in isolation. The transabdominal approach is generally not suitable to record an accurate follicle count but is considered reliable to determine if the ovarian volume is >10 mL. On occasion, with a high superficial location, the ovary may be better seen for follicle counts than via the TVUS route but remains less reliable because of lower transducer frequency. Variant 3: Female infertility. History or clinical suspicion of endometriosis. Initial imaging. Fluoroscopy Hysterosalpingography HSG to assess tubal patency and the uterine cavity has been proposed as part of an infertility workup in women with endometriosis [8]. However, in one study, 21% of women undergoing infertility evaluation were found to have endometriosis at surgery despite a normal HSG [33]. Although HSG may be helpful in diagnosing tubal occlusion or patency in patients with endometriosis, other imaging modalities are more sensitive in detecting endometriosis.

Female Infertility. When the ovarian volume is <3 cm3 and <5 antral follicles are present, this suggests diminished ovarian reserve [29]. MRI Pelvis A recent study in obese adolescents with suspected PCOS demonstrated that MRI without IV contrast can provide reproducible and reliable ovarian volume assessment; however, ovarian follicle counts had only moderate interobserver agreement. Nonetheless, in the obese adolescent or patient in whom TVUS is unacceptable and transabdominal US limited, MRI may provide additional information on PCOM [25,26,30]. US Pelvis Color Doppler Although increased central stromal vascularity has been demonstrated in the ovaries of women with PCOS, there is limited evidence to indicate color Doppler should be performed routinely in the evaluation of PCOM [31]. Female Infertility US Pelvis Transabdominal Transabdominal US is often performed in conjunction with TVUS; however, in some settings it may be performed in isolation. The transabdominal approach is generally not suitable to record an accurate follicle count but is considered reliable to determine if the ovarian volume is >10 mL. On occasion, with a high superficial location, the ovary may be better seen for follicle counts than via the TVUS route but remains less reliable because of lower transducer frequency. Variant 3: Female infertility. History or clinical suspicion of endometriosis. Initial imaging. Fluoroscopy Hysterosalpingography HSG to assess tubal patency and the uterine cavity has been proposed as part of an infertility workup in women with endometriosis [8]. However, in one study, 21% of women undergoing infertility evaluation were found to have endometriosis at surgery despite a normal HSG [33]. Although HSG may be helpful in diagnosing tubal occlusion or patency in patients with endometriosis, other imaging modalities are more sensitive in detecting endometriosis.

3093336

acrac_3093336_3

Female Infertility

MRI Pelvis MRI has been shown to have a sensitivity of 82% to 90% and specificity of 91% to 98% for the diagnosis of endometriomas and can be used when the findings on TVUS are indeterminate [34-36] or when assessment for deep infiltrating endometriosis is required prior to fertility-sparing surgery. Although IV contrast is useful for the assessment of PID and for determining if ovarian masses have enhancing components, IV contrast is not routinely used for the assessment of endometriosis [34]. The typical MRI features of an endometrioma are high signal on T1- weighted with low signal on T2-weighted images (T2 shading) from intracellular methemoglobin, crosslinking of proteins, and iron. Additionally, the T2 dark spot sign has 93% specificity in differentiating endometriomas from hemorrhagic cysts, whereas T2 shading is sensitive but not specific for endometriomas [37]. Deep infiltrating endometriosis presents as low signal intensity regions with or without hyperintense foci on T2- and/or T1-weighted images. Despite administration of gadolinium, studies do not attempt to demonstrate a difference in detection of deep infiltrating endometriosis with gadolinium as compared to without [38,39]. Adhesions are frequently present in endometriosis and can be suspected, in the presence of a uterus, fixed in retroversion, low-signal intensity bands, obliteration of organ interfaces, and/or obliteration of the cul-de-sac [40]. In a study of 159 women who underwent surgery for clinical suspicion of deep infiltrating endometriosis, MRI was 92.4% sensitive and 94.6% specific in detecting intestinal endometriosis, 88% sensitive and 83.3% specific in detecting deep infiltrating endometriosis in posterior locations (uterosacral ligament, retrocervical, rectovaginal septum, vaginal fornix), and 50% sensitive and 97.3% specific in detecting bladder wall endometriosis [38].

Female Infertility. MRI Pelvis MRI has been shown to have a sensitivity of 82% to 90% and specificity of 91% to 98% for the diagnosis of endometriomas and can be used when the findings on TVUS are indeterminate [34-36] or when assessment for deep infiltrating endometriosis is required prior to fertility-sparing surgery. Although IV contrast is useful for the assessment of PID and for determining if ovarian masses have enhancing components, IV contrast is not routinely used for the assessment of endometriosis [34]. The typical MRI features of an endometrioma are high signal on T1- weighted with low signal on T2-weighted images (T2 shading) from intracellular methemoglobin, crosslinking of proteins, and iron. Additionally, the T2 dark spot sign has 93% specificity in differentiating endometriomas from hemorrhagic cysts, whereas T2 shading is sensitive but not specific for endometriomas [37]. Deep infiltrating endometriosis presents as low signal intensity regions with or without hyperintense foci on T2- and/or T1-weighted images. Despite administration of gadolinium, studies do not attempt to demonstrate a difference in detection of deep infiltrating endometriosis with gadolinium as compared to without [38,39]. Adhesions are frequently present in endometriosis and can be suspected, in the presence of a uterus, fixed in retroversion, low-signal intensity bands, obliteration of organ interfaces, and/or obliteration of the cul-de-sac [40]. In a study of 159 women who underwent surgery for clinical suspicion of deep infiltrating endometriosis, MRI was 92.4% sensitive and 94.6% specific in detecting intestinal endometriosis, 88% sensitive and 83.3% specific in detecting deep infiltrating endometriosis in posterior locations (uterosacral ligament, retrocervical, rectovaginal septum, vaginal fornix), and 50% sensitive and 97.3% specific in detecting bladder wall endometriosis [38].

3093336

acrac_3093336_4

Female Infertility

Adherence to or angulation of bowel loops toward the posterior surface of the uterus has been shown to be 83.7% sensitive to diagnose cul-de-sac obliteration, whereas displacement of pelvic free fluid was 95% sensitive and presence of a retrouterine fibrous mass was 97.1% sensitive [40]. These findings may have been enhanced in this study because of the administration of vaginal and rectal sterile US gel as well as an IV antispasmodic. Another study similarly identified serosal uterine fibrotic plaques as having the best accuracy for posterior cul-de-sac obliteration [41] but did not assess displacement of free pelvic fluid. Overall, MRI has value in assessing for endometriomas and other signs of endometriosis, such as angulation of bowel loops toward the posterior surface of the uterus, displacement of pelvic free fluid, and detection of retro-uterine fibrous masses. US Pelvis Color Doppler Endometrial implants have limited vascularity. The presence of Doppler blood flow in a suspected endometrial implant should prompt investigation for neoplasm [42]. Doppler can also be useful in differentiating endometriomas and endometrial implants, which are typically avascular, from other ovarian masses and normal structures [43]. Female Infertility US Pelvis Transrectal Transrectal US can also be useful in the detection of deep infiltrating endometriosis. Compared with surgery, transrectal US was shown to be 97% sensitive and 96% specific for the detection of rectovaginal endometriosis and was 80% sensitive and 97% specific in diagnosing uterosacral ligament implants [44]. In another study, 317 patients underwent pelvic MRI, TVUS, and transrectal US prior to laparoscopy. This study showed transrectal US had the highest sensitivity at 82.8%, but MRI was the most specific at 93.9% for evaluation of the uterosacral ligament, and the three techniques were similar for assessing the rectovaginal septum, bladder, and distal ureters.

Female Infertility. Adherence to or angulation of bowel loops toward the posterior surface of the uterus has been shown to be 83.7% sensitive to diagnose cul-de-sac obliteration, whereas displacement of pelvic free fluid was 95% sensitive and presence of a retrouterine fibrous mass was 97.1% sensitive [40]. These findings may have been enhanced in this study because of the administration of vaginal and rectal sterile US gel as well as an IV antispasmodic. Another study similarly identified serosal uterine fibrotic plaques as having the best accuracy for posterior cul-de-sac obliteration [41] but did not assess displacement of free pelvic fluid. Overall, MRI has value in assessing for endometriomas and other signs of endometriosis, such as angulation of bowel loops toward the posterior surface of the uterus, displacement of pelvic free fluid, and detection of retro-uterine fibrous masses. US Pelvis Color Doppler Endometrial implants have limited vascularity. The presence of Doppler blood flow in a suspected endometrial implant should prompt investigation for neoplasm [42]. Doppler can also be useful in differentiating endometriomas and endometrial implants, which are typically avascular, from other ovarian masses and normal structures [43]. Female Infertility US Pelvis Transrectal Transrectal US can also be useful in the detection of deep infiltrating endometriosis. Compared with surgery, transrectal US was shown to be 97% sensitive and 96% specific for the detection of rectovaginal endometriosis and was 80% sensitive and 97% specific in diagnosing uterosacral ligament implants [44]. In another study, 317 patients underwent pelvic MRI, TVUS, and transrectal US prior to laparoscopy. This study showed transrectal US had the highest sensitivity at 82.8%, but MRI was the most specific at 93.9% for evaluation of the uterosacral ligament, and the three techniques were similar for assessing the rectovaginal septum, bladder, and distal ureters.

3093336

acrac_3093336_5

Female Infertility

MRI was superior to TVUS and transrectal US in diagnosing retrocervical endometriosis [45]. Transrectal US is limited to a small anatomic area [43] but can be useful in some patients, especially those unable to undergo TVUS [45]. US Pelvis Transvaginal TVUS, especially when combined with real-time physical examination, can be useful in the detection of both ovarian and nonovarian endometriosis. Some studies have demonstrated superiority of TVUS over MRI in the detection of rectosigmoid and retrocervical endometriosis [46-49]. TVUS can demonstrate macroscopic endometriomas that are often bilateral. On US, an endometrioma typically appears as an adnexal or ovarian mass with homogenous low-level internal echoes. The presence of echogenic foci in the wall (hemosiderin deposits) or multilocularity further increases the likelihood that a mass with this appearance is an endometrioma [50]. Nonovarian endometriosis can be assessed best with dynamic US by looking for the uterine sliding sign, assessing for nodules at sites of tenderness, assessing ovarian mobility, and looking for hypoechoic nodules outside of the ovaries [51]. US Sonohysterography To our knowledge, there is no relevant literature to support performing isolated US sonohysterography in patients with a history or clinical suspicion of endometriosis. Contrast sonohysterography or saline-infusion sonohysterography (SIS) provides an assessment of the uterine cavity. Although the endometrium can be assessed by TVUS, SIS is particularly useful in assessing potential causes of infertility, including intrauterine adhesions, endometrial polyps, and leiomyomas [52]. Antibiotic administration or prophylactic use of antibiotics is at the discretion of the referring physician if there is a prior history of PID or if hydrosalpinx is noted at the time of the study.

Female Infertility. MRI was superior to TVUS and transrectal US in diagnosing retrocervical endometriosis [45]. Transrectal US is limited to a small anatomic area [43] but can be useful in some patients, especially those unable to undergo TVUS [45]. US Pelvis Transvaginal TVUS, especially when combined with real-time physical examination, can be useful in the detection of both ovarian and nonovarian endometriosis. Some studies have demonstrated superiority of TVUS over MRI in the detection of rectosigmoid and retrocervical endometriosis [46-49]. TVUS can demonstrate macroscopic endometriomas that are often bilateral. On US, an endometrioma typically appears as an adnexal or ovarian mass with homogenous low-level internal echoes. The presence of echogenic foci in the wall (hemosiderin deposits) or multilocularity further increases the likelihood that a mass with this appearance is an endometrioma [50]. Nonovarian endometriosis can be assessed best with dynamic US by looking for the uterine sliding sign, assessing for nodules at sites of tenderness, assessing ovarian mobility, and looking for hypoechoic nodules outside of the ovaries [51]. US Sonohysterography To our knowledge, there is no relevant literature to support performing isolated US sonohysterography in patients with a history or clinical suspicion of endometriosis. Contrast sonohysterography or saline-infusion sonohysterography (SIS) provides an assessment of the uterine cavity. Although the endometrium can be assessed by TVUS, SIS is particularly useful in assessing potential causes of infertility, including intrauterine adhesions, endometrial polyps, and leiomyomas [52]. Antibiotic administration or prophylactic use of antibiotics is at the discretion of the referring physician if there is a prior history of PID or if hydrosalpinx is noted at the time of the study.

3093336

acrac_3093336_6

Female Infertility

US Sonohysterography with Tubal Contrast Agent Hysterosalpingo-contrast sonography (HyCoSy) involves instilling echogenic contrast, typically an agitated air and saline mixture, into the uterus with real-time US to observe the material distending the uterine cavity, filling the fallopian tubes, and spilling out over the adjacent ovary [53]. Accuracy of HyCoSy is similar to HSG when compared with laparoscopy with chromopertubation in determining tubal patency with sensitivity of 75% to 96% and specificity of 67% to 100% when compared directly with laparoscopy with chromopertubation [54]. It has been proposed that TVUS with 3-D imaging of the uterus and ovaries followed by SIS and HyCoSy with agitated saline can be performed in one session as a comprehensive infertility examination [42]. In women with endometriosis, HyCoSy has been shown to be 91% accurate compared with laparoscopy in diagnosing tubal patency [55]. This modality can also detect endometriomas as well as pelvic adhesive disease and endometriotic nodules, especially in the hands of well-trained operators. Variant 4: Female infertility. Suspicion of tubal occlusion. Initial imaging. Fluoroscopy Hysterosalpingography HSG allows detection of tubal patency, tubal size, tubal irregularity, and peritubal disease. It can also detect intrauterine synechiae, which typically present as irregular endometrial filling defects [11]. Tubal flushing during HSG has also been shown to increase pregnancy rates up to 38% compared with pregnancy rates of up to 21% in women being investigated for infertility who did not undergo HSG. Pregnancy rate in this study was highest in women who underwent HSG with oil-soluble contrast [56]. However, unlike performance of HSG with water- soluble contrast agents, the use of oil-based contrast material for HSG carries the increased risk of oil emboli if there is myometrial intravasation [57].

Female Infertility. US Sonohysterography with Tubal Contrast Agent Hysterosalpingo-contrast sonography (HyCoSy) involves instilling echogenic contrast, typically an agitated air and saline mixture, into the uterus with real-time US to observe the material distending the uterine cavity, filling the fallopian tubes, and spilling out over the adjacent ovary [53]. Accuracy of HyCoSy is similar to HSG when compared with laparoscopy with chromopertubation in determining tubal patency with sensitivity of 75% to 96% and specificity of 67% to 100% when compared directly with laparoscopy with chromopertubation [54]. It has been proposed that TVUS with 3-D imaging of the uterus and ovaries followed by SIS and HyCoSy with agitated saline can be performed in one session as a comprehensive infertility examination [42]. In women with endometriosis, HyCoSy has been shown to be 91% accurate compared with laparoscopy in diagnosing tubal patency [55]. This modality can also detect endometriomas as well as pelvic adhesive disease and endometriotic nodules, especially in the hands of well-trained operators. Variant 4: Female infertility. Suspicion of tubal occlusion. Initial imaging. Fluoroscopy Hysterosalpingography HSG allows detection of tubal patency, tubal size, tubal irregularity, and peritubal disease. It can also detect intrauterine synechiae, which typically present as irregular endometrial filling defects [11]. Tubal flushing during HSG has also been shown to increase pregnancy rates up to 38% compared with pregnancy rates of up to 21% in women being investigated for infertility who did not undergo HSG. Pregnancy rate in this study was highest in women who underwent HSG with oil-soluble contrast [56]. However, unlike performance of HSG with water- soluble contrast agents, the use of oil-based contrast material for HSG carries the increased risk of oil emboli if there is myometrial intravasation [57].

3093336

acrac_3093336_7

Female Infertility

Although HSG has been historically regarded as the imaging study of choice in assessing tubal patency, it is only 65% sensitive and 85% specific for diagnosing tubal patency when compared Female Infertility with laparoscopy with chromopertubation [58], which is widely accepted as the reference standard for evaluating tubal patency. MRI Pelvis MRI is useful in the detection of hydrosalpinges, most commonly due to prior PID, and is superior to TVUS in the assessment of PID, although this refers to acute PID, which is outside of the scope of this topic (95% sensitive and 89% specific compared to 81% sensitive and 78% specific) [59]. Hydrosalpinges are detected in subclinical or chronic PID secondary to scarring of the fallopian tubes or tubal obstruction by peritoneal bands from previous inflammation [60]. In a blinded study of 41 patients with hydrosalpinx at surgery, hydrosalpinx was accurately diagnosed in 31 patients (75.6%) on MRI [61]. Although IV contrast is useful in assessing additional complications of PID, it is not necessary for the evaluation of hydrosalpinges [34,60]. US Pelvis Transvaginal Hydrosalpinx may occur in the setting of distal tubal occlusion, most commonly due to prior PID [62]. The finding of hydrosalpinx has implications for patients who may undergo in vitro fertilization [63]. TVUS has been shown to be 86% sensitive in detecting hydrosalpinx [64]. Apart from detection of hydrosalpinges, TVUS has not been shown to be effective in documenting tubal patency. US Sonohysterography To our knowledge, there is no relevant literature to support performing isolated US sonohysterography in patients with suspicion of tubal occlusion; however, the presence of increased fluid in the posterior cul-de-sac following sonohysterography may indicate tubal patency.

Female Infertility. Although HSG has been historically regarded as the imaging study of choice in assessing tubal patency, it is only 65% sensitive and 85% specific for diagnosing tubal patency when compared Female Infertility with laparoscopy with chromopertubation [58], which is widely accepted as the reference standard for evaluating tubal patency. MRI Pelvis MRI is useful in the detection of hydrosalpinges, most commonly due to prior PID, and is superior to TVUS in the assessment of PID, although this refers to acute PID, which is outside of the scope of this topic (95% sensitive and 89% specific compared to 81% sensitive and 78% specific) [59]. Hydrosalpinges are detected in subclinical or chronic PID secondary to scarring of the fallopian tubes or tubal obstruction by peritoneal bands from previous inflammation [60]. In a blinded study of 41 patients with hydrosalpinx at surgery, hydrosalpinx was accurately diagnosed in 31 patients (75.6%) on MRI [61]. Although IV contrast is useful in assessing additional complications of PID, it is not necessary for the evaluation of hydrosalpinges [34,60]. US Pelvis Transvaginal Hydrosalpinx may occur in the setting of distal tubal occlusion, most commonly due to prior PID [62]. The finding of hydrosalpinx has implications for patients who may undergo in vitro fertilization [63]. TVUS has been shown to be 86% sensitive in detecting hydrosalpinx [64]. Apart from detection of hydrosalpinges, TVUS has not been shown to be effective in documenting tubal patency. US Sonohysterography To our knowledge, there is no relevant literature to support performing isolated US sonohysterography in patients with suspicion of tubal occlusion; however, the presence of increased fluid in the posterior cul-de-sac following sonohysterography may indicate tubal patency.

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Female Infertility

US Sonohysterography with Tubal Contrast Agent HyCoSy involves instilling echogenic contrast, typically an agitated air and saline mixture, into the uterus with real- time US to observe the material distending the uterine cavity, filling the fallopian tubes, and spilling out over the adjacent ovary [53]. HyCoSy is similar to HSG when compared with laparoscopy with chromopertubation in determining tubal patency [54]. It has been proposed that TVUS with 3-D imaging of the uterus and ovaries followed by SIS and HyCoSy with agitated saline can be performed in one session as a comprehensive infertility examination [42]. HyCoSy has been shown to be 91% accurate compared with laparoscopy in diagnosing tubal patency in women with endometriosis [55]. Hysterosalpingo-foam sonography in combination with 2-D or 3-D imaging has demonstrated improved accuracy of 93.7% and better concordance with laparoscopy compared with 2-D or 3-D air or saline HyCoSy [65,66] for assessment of tubal patency. The addition of high-definition flow (bidirectional Doppler feature) achieved even higher accuracy at 96.9%, comparable with the reference method of laparoscopic chromopertubation with dye [65]. However, high-definition flow still needs to be validated by other groups. Variant 5: Female infertility. Recurrent pregnancy loss. Initial imaging. Fluoroscopy Hysterosalpingography In 24 cases of surgically proven MDA, MRI was 100% accurate, 2-D TVUS was 92% accurate, and hysterosalpingogram was only 16.7% accurate in characterizing MDAs [67]. Although HSG can visualize the uterine cavity, it cannot always provide complete information about the external uterine contour, preventing accurate distinction between a septate and a bicornuate uterus. A study of 54 women with suspected Asherman syndrome discovered 3-D US was 100% sensitive, and HSG was 66.7% sensitive in grading intrauterine adhesions compared with hysteroscopy [68].

Female Infertility. US Sonohysterography with Tubal Contrast Agent HyCoSy involves instilling echogenic contrast, typically an agitated air and saline mixture, into the uterus with real- time US to observe the material distending the uterine cavity, filling the fallopian tubes, and spilling out over the adjacent ovary [53]. HyCoSy is similar to HSG when compared with laparoscopy with chromopertubation in determining tubal patency [54]. It has been proposed that TVUS with 3-D imaging of the uterus and ovaries followed by SIS and HyCoSy with agitated saline can be performed in one session as a comprehensive infertility examination [42]. HyCoSy has been shown to be 91% accurate compared with laparoscopy in diagnosing tubal patency in women with endometriosis [55]. Hysterosalpingo-foam sonography in combination with 2-D or 3-D imaging has demonstrated improved accuracy of 93.7% and better concordance with laparoscopy compared with 2-D or 3-D air or saline HyCoSy [65,66] for assessment of tubal patency. The addition of high-definition flow (bidirectional Doppler feature) achieved even higher accuracy at 96.9%, comparable with the reference method of laparoscopic chromopertubation with dye [65]. However, high-definition flow still needs to be validated by other groups. Variant 5: Female infertility. Recurrent pregnancy loss. Initial imaging. Fluoroscopy Hysterosalpingography In 24 cases of surgically proven MDA, MRI was 100% accurate, 2-D TVUS was 92% accurate, and hysterosalpingogram was only 16.7% accurate in characterizing MDAs [67]. Although HSG can visualize the uterine cavity, it cannot always provide complete information about the external uterine contour, preventing accurate distinction between a septate and a bicornuate uterus. A study of 54 women with suspected Asherman syndrome discovered 3-D US was 100% sensitive, and HSG was 66.7% sensitive in grading intrauterine adhesions compared with hysteroscopy [68].

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Female Infertility

Another study of only 19 women discovered HSG and sonohysterography were both 100% sensitive, and conventional TVUS was only 52% sensitive for detecting intrauterine adhesions compared with hysteroscopy [69]. Additional studies have shown HSG to be 75% to 81% sensitive and 80% specific compared with hysteroscopy in diagnosing intrauterine adhesions [70,71]. Antibiotic administration or prophylactic use of antibiotics is at the discretion of the referring physician if there is a prior history of PID or if hydrosalpinx is noted at the time of the study. Although HSG may provide useful information about the uterine cavity, such as the presence of adhesions, it is not reliable in categorizing MDAs and has been largely replaced by MRI and 3-D US for assessment of the uterine cavity in recurrent pregnancy loss. Female Infertility MRI Pelvis MRI provides accurate assessment of uterine abnormalities potentially contributing to infertility such as MDA [67], adenomyosis [72], and leiomyomas [73]. On both TVUS and MRI, a fundal cleft >1 cm can be used to diagnose a bicornuate uterus and differentiate it from a septate uterus (fundal cleft <1 cm) [67]. A fundal indentation <5 mm above the interostial line can also be used for identification of a bicornuate uterus [74]. In 24 cases of surgically proven MDA, MRI was 100% accurate, 2-D TVUS was 92% accurate, and hysterosalpingogram was only 16.7% accurate in the classification of MDA [67]. MRI demonstrates 78% to 88% sensitivity and 67% to 93% specificity in detecting adenomyosis, typically thickening of the junctional zone, often with T2 hyperintense foci [72]. Nondegenerated fibroids are classically well circumscribed with low T2 signal. The imaging features of degenerated fibroids can vary greatly on MRI, however [73]. Although MRI may be useful in detecting intrauterine adhesions, no large studies comparing its efficacy to hysteroscopy have been performed [75].

Female Infertility. Another study of only 19 women discovered HSG and sonohysterography were both 100% sensitive, and conventional TVUS was only 52% sensitive for detecting intrauterine adhesions compared with hysteroscopy [69]. Additional studies have shown HSG to be 75% to 81% sensitive and 80% specific compared with hysteroscopy in diagnosing intrauterine adhesions [70,71]. Antibiotic administration or prophylactic use of antibiotics is at the discretion of the referring physician if there is a prior history of PID or if hydrosalpinx is noted at the time of the study. Although HSG may provide useful information about the uterine cavity, such as the presence of adhesions, it is not reliable in categorizing MDAs and has been largely replaced by MRI and 3-D US for assessment of the uterine cavity in recurrent pregnancy loss. Female Infertility MRI Pelvis MRI provides accurate assessment of uterine abnormalities potentially contributing to infertility such as MDA [67], adenomyosis [72], and leiomyomas [73]. On both TVUS and MRI, a fundal cleft >1 cm can be used to diagnose a bicornuate uterus and differentiate it from a septate uterus (fundal cleft <1 cm) [67]. A fundal indentation <5 mm above the interostial line can also be used for identification of a bicornuate uterus [74]. In 24 cases of surgically proven MDA, MRI was 100% accurate, 2-D TVUS was 92% accurate, and hysterosalpingogram was only 16.7% accurate in the classification of MDA [67]. MRI demonstrates 78% to 88% sensitivity and 67% to 93% specificity in detecting adenomyosis, typically thickening of the junctional zone, often with T2 hyperintense foci [72]. Nondegenerated fibroids are classically well circumscribed with low T2 signal. The imaging features of degenerated fibroids can vary greatly on MRI, however [73]. Although MRI may be useful in detecting intrauterine adhesions, no large studies comparing its efficacy to hysteroscopy have been performed [75].

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Female Infertility

When comparing diagnostic modalities to hysterectomy for the detection of intracavitary abnormalities, MRI, SIS, and hysteroscopy were equally effective and superior to TVUS [76]. US Pelvis Color Doppler Power Doppler has been shown to be useful in detecting stalks within endometrial polyps and peripheral or rim vascularity in submucosal fibroids [77]. Color Doppler has also been shown to be useful in differentiating the central vascular pattern of adenomyosis from the peripheral vascularity of fibroids [78]. This procedure is not performed in isolation but in conjunction with TVUS. US Pelvis Transvaginal On both TVUS and MRI, an external fundal cleft >1 cm can be used to diagnose a bicornuate uterus and differentiate it from a septate uterus (fundal cleft <1 cm) [67]. A fundal indentation <5 mm above the interostial line can also be used for identification of a bicornuate uterus [74]. In 24 cases of surgically proven MDA, MRI was 100% accurate, 2-D TVUS was 92% accurate, and hysterosalpingogram was only 16.7% accurate [67]. For detecting intrauterine adhesions, a study of 19 women showed that HSG and sonohysterography were both 100% sensitive, and conventional TVUS was only 52% sensitive compared with hysteroscopy [69]. In another study of 133 women undergoing infertility evaluation who underwent both hysteroscopy and TVUS, TVUS detected submucosal fibroids in 10 of the 11 patients diagnosed with submucosal fibroids at hysteroscopy, yielding a sensitivity of 91% and specificity of 100% [79]. When comparing diagnostic modalities with hysterectomy for the detection of intracavitary abnormalities, MRI, SIS, and hysteroscopy were equally effective and superior to TVUS [76]. US Sonohysterography Contrast sonohysterography or SIS provides an assessment of the uterine cavity. Although the endometrium can be assessed by TVUS, SIS is particularly useful in assessing potential causes of infertility, including intrauterine adhesions, endometrial polyps, and leiomyomas [52].

Female Infertility. When comparing diagnostic modalities to hysterectomy for the detection of intracavitary abnormalities, MRI, SIS, and hysteroscopy were equally effective and superior to TVUS [76]. US Pelvis Color Doppler Power Doppler has been shown to be useful in detecting stalks within endometrial polyps and peripheral or rim vascularity in submucosal fibroids [77]. Color Doppler has also been shown to be useful in differentiating the central vascular pattern of adenomyosis from the peripheral vascularity of fibroids [78]. This procedure is not performed in isolation but in conjunction with TVUS. US Pelvis Transvaginal On both TVUS and MRI, an external fundal cleft >1 cm can be used to diagnose a bicornuate uterus and differentiate it from a septate uterus (fundal cleft <1 cm) [67]. A fundal indentation <5 mm above the interostial line can also be used for identification of a bicornuate uterus [74]. In 24 cases of surgically proven MDA, MRI was 100% accurate, 2-D TVUS was 92% accurate, and hysterosalpingogram was only 16.7% accurate [67]. For detecting intrauterine adhesions, a study of 19 women showed that HSG and sonohysterography were both 100% sensitive, and conventional TVUS was only 52% sensitive compared with hysteroscopy [69]. In another study of 133 women undergoing infertility evaluation who underwent both hysteroscopy and TVUS, TVUS detected submucosal fibroids in 10 of the 11 patients diagnosed with submucosal fibroids at hysteroscopy, yielding a sensitivity of 91% and specificity of 100% [79]. When comparing diagnostic modalities with hysterectomy for the detection of intracavitary abnormalities, MRI, SIS, and hysteroscopy were equally effective and superior to TVUS [76]. US Sonohysterography Contrast sonohysterography or SIS provides an assessment of the uterine cavity. Although the endometrium can be assessed by TVUS, SIS is particularly useful in assessing potential causes of infertility, including intrauterine adhesions, endometrial polyps, and leiomyomas [52].

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Staging and Follow up of Vulvar Cancer

Introduction/Background Vulvar cancer is a rare gynecologic malignancy. In the United States, it is estimated that approximately 6,120 women will present with vulvar cancer, and 1,350 will succumb to their disease in 2020 [1]. Most patients are diagnosed with early-stage disease, and the majority of tumors originate in the labia majora [2,3]. The 5-year survival rate is 86% for patients with vulvar-confined disease but is reduced to 57% for patients with regional lymph node metastases, and 17% for patients with distant metastases [4]. Squamous cell carcinoma (SCC) is the predominant histotype of vulvar cancer, accounting for 90% of cases; thus, it is the focus of this discussion. Up to 69% of vulvar cancer is attributed to chronic human papillomavirus (HPV) infection, in particular high-risk strains such as HPV-16 and HPV-18 [5]. Other risk factors include older age, tobacco use, chronic inflammation of the vulva, and immune-compromised state [6]. The International Federation of Obstetrics and Gynecology (FIGO) and The American Joint Committee on Cancer (AJCC) TNM systems are both used to stage vulvar cancer and are closely aligned [7-9]. Initial evaluation of patients with vulvar lesions consists of careful clinical examination and punch biopsies of all suspicious vulvar lesions. Care must be taken to include the underlying stroma and to avoid necrotic areas. Lesion size, location relative to the midline, relationship to the adjacent organs (urethra, vagina, anus), and presence of multifocal disease are noted on physical examination. Clinical palpation of groin lymph nodes is performed, although this approach is limited by the high false-negative rate [10]. The status of inguinofemoral lymph nodes (IFLNs) is the most important prognostic factor in vulvar cancer. The likelihood of lymph node metastases is estimated by primary tumor size, depth of stromal invasion, and presence of lymphovascular space invasion [11-15].

Staging and Follow up of Vulvar Cancer. Introduction/Background Vulvar cancer is a rare gynecologic malignancy. In the United States, it is estimated that approximately 6,120 women will present with vulvar cancer, and 1,350 will succumb to their disease in 2020 [1]. Most patients are diagnosed with early-stage disease, and the majority of tumors originate in the labia majora [2,3]. The 5-year survival rate is 86% for patients with vulvar-confined disease but is reduced to 57% for patients with regional lymph node metastases, and 17% for patients with distant metastases [4]. Squamous cell carcinoma (SCC) is the predominant histotype of vulvar cancer, accounting for 90% of cases; thus, it is the focus of this discussion. Up to 69% of vulvar cancer is attributed to chronic human papillomavirus (HPV) infection, in particular high-risk strains such as HPV-16 and HPV-18 [5]. Other risk factors include older age, tobacco use, chronic inflammation of the vulva, and immune-compromised state [6]. The International Federation of Obstetrics and Gynecology (FIGO) and The American Joint Committee on Cancer (AJCC) TNM systems are both used to stage vulvar cancer and are closely aligned [7-9]. Initial evaluation of patients with vulvar lesions consists of careful clinical examination and punch biopsies of all suspicious vulvar lesions. Care must be taken to include the underlying stroma and to avoid necrotic areas. Lesion size, location relative to the midline, relationship to the adjacent organs (urethra, vagina, anus), and presence of multifocal disease are noted on physical examination. Clinical palpation of groin lymph nodes is performed, although this approach is limited by the high false-negative rate [10]. The status of inguinofemoral lymph nodes (IFLNs) is the most important prognostic factor in vulvar cancer. The likelihood of lymph node metastases is estimated by primary tumor size, depth of stromal invasion, and presence of lymphovascular space invasion [11-15].

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Staging and Follow up of Vulvar Cancer

Traditionally, IFLN assessment entailed complete lymphadenectomy. High morbidity of this procedure and the fact that only a third of patients with early-stage disease have lymph node metastases at diagnosis led to a major shift toward less invasive assessment strategies including a combination of imaging, sentinel lymph node (SLN) mapping, and/or biopsy [16-21]. The SLN is the lymph node at the highest risk of metastasis because it is the first lymph node to receive lymphatic drainage from the primary tumor. Several prospective multi-institutional trials established the feasibility, safety, and effectiveness of SLN evaluation in early- stage vulvar cancer [16,19-23]. The peer-reviewed literature about the role of imaging in vulvar cancer is limited, likely because vulvar cancer is uncommon. Many studies report on patients with combined histotypes of vulvar cancer, wide range of disease stages, primary and recurrent disease, and vulvar and vaginal cancers combined. The imaging techniques are not uniform, and the descriptions of imaging studies are often limited with regard to the area imaged and the use of intravenous (IV) contrast. The above limits our ability to draw conclusions and provide evidence-based imaging recommendations. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization. Reprint requests to: [email protected] Staging and Follow-up of Vulvar Cancer Special Imaging Considerations Vaginal gel is optional with MRI pelvis without and with IV contrast or MRI pelvis without IV contrast. Vaginal gel may be useful to delineate the extent of vaginal involvement by primary tumor.

Staging and Follow up of Vulvar Cancer. Traditionally, IFLN assessment entailed complete lymphadenectomy. High morbidity of this procedure and the fact that only a third of patients with early-stage disease have lymph node metastases at diagnosis led to a major shift toward less invasive assessment strategies including a combination of imaging, sentinel lymph node (SLN) mapping, and/or biopsy [16-21]. The SLN is the lymph node at the highest risk of metastasis because it is the first lymph node to receive lymphatic drainage from the primary tumor. Several prospective multi-institutional trials established the feasibility, safety, and effectiveness of SLN evaluation in early- stage vulvar cancer [16,19-23]. The peer-reviewed literature about the role of imaging in vulvar cancer is limited, likely because vulvar cancer is uncommon. Many studies report on patients with combined histotypes of vulvar cancer, wide range of disease stages, primary and recurrent disease, and vulvar and vaginal cancers combined. The imaging techniques are not uniform, and the descriptions of imaging studies are often limited with regard to the area imaged and the use of intravenous (IV) contrast. The above limits our ability to draw conclusions and provide evidence-based imaging recommendations. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization. Reprint requests to: [email protected] Staging and Follow-up of Vulvar Cancer Special Imaging Considerations Vaginal gel is optional with MRI pelvis without and with IV contrast or MRI pelvis without IV contrast. Vaginal gel may be useful to delineate the extent of vaginal involvement by primary tumor.

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Staging and Follow up of Vulvar Cancer

Variant 2: Initial staging of pretreatment vulvar cancer: Primary tumor is less than or equal to 4 cm with greater than 1 mm stromal invasion, confined to vulva or perineum, or with minimal involvement of the urethra, vagina, or anus. For early-stage disease, present-day surgical approach consists of conservative vulvar resection (with at least 1 cm of tumor-free margin) and IFLN assessment carried out via two separate incisions [6]. If no lymph node metastases Staging and Follow-up of Vulvar Cancer are apparent at clinical assessment, the extent of lymph node evaluation is determined by the size of the primary tumor, the depth of stromal invasion, and location of the primary tumor relative to the midline [6]. If clinical and/or imaging evaluation shows no apparent IFLN metastases, SLN mapping is performed as detailed below. If IFLN metastases are suspected based on clinical and/or imaging evaluation, either minimally invasive lymph node sampling with US-guided FNAB and/or IFLN dissection may be performed. CT Abdomen and Pelvis CT of the abdomen and pelvis is not indicated in patients with clinical early-stage disease. There is no relevant literature detailing the role of CT in the evaluation of primary tumor size and extent, which is likely explained by the limited soft-tissue contrast of CT. Lymph node morphologic characteristics including size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. If IFLN metastases are suspected on imaging, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling. CT Chest, Abdomen, and Pelvis CT of the chest, abdomen, and pelvis is not indicated in patients with clinical early-stage disease.

Staging and Follow up of Vulvar Cancer. Variant 2: Initial staging of pretreatment vulvar cancer: Primary tumor is less than or equal to 4 cm with greater than 1 mm stromal invasion, confined to vulva or perineum, or with minimal involvement of the urethra, vagina, or anus. For early-stage disease, present-day surgical approach consists of conservative vulvar resection (with at least 1 cm of tumor-free margin) and IFLN assessment carried out via two separate incisions [6]. If no lymph node metastases Staging and Follow-up of Vulvar Cancer are apparent at clinical assessment, the extent of lymph node evaluation is determined by the size of the primary tumor, the depth of stromal invasion, and location of the primary tumor relative to the midline [6]. If clinical and/or imaging evaluation shows no apparent IFLN metastases, SLN mapping is performed as detailed below. If IFLN metastases are suspected based on clinical and/or imaging evaluation, either minimally invasive lymph node sampling with US-guided FNAB and/or IFLN dissection may be performed. CT Abdomen and Pelvis CT of the abdomen and pelvis is not indicated in patients with clinical early-stage disease. There is no relevant literature detailing the role of CT in the evaluation of primary tumor size and extent, which is likely explained by the limited soft-tissue contrast of CT. Lymph node morphologic characteristics including size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. If IFLN metastases are suspected on imaging, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling. CT Chest, Abdomen, and Pelvis CT of the chest, abdomen, and pelvis is not indicated in patients with clinical early-stage disease.

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Staging and Follow up of Vulvar Cancer

There is no relevant literature detailing the role of CT in the evaluation of primary tumor size and extent, which is likely explained by the limited soft-tissue contrast of CT. Lymph node enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. If IFLN metastases are suspected on imaging, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling. CT Pelvis CT of the pelvis is not indicated in patients with clinical early-stage disease. There is no relevant literature about the role of CT in the evaluation of primary tumor size and extent, likely explained by the limited soft-tissue contrast of CT. Lymph node enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. If IFLN metastases are suspected on imaging, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not Staging and Follow-up of Vulvar Cancer exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is not indicated in patients with clinical early-stage disease. There is no relevant literature about the role of FDG-PET/CT in the evaluation of primary tumor size and extent. If findings on FDG-PET/CT are suspicious for lymph node metastases, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling.

Staging and Follow up of Vulvar Cancer. There is no relevant literature detailing the role of CT in the evaluation of primary tumor size and extent, which is likely explained by the limited soft-tissue contrast of CT. Lymph node enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. If IFLN metastases are suspected on imaging, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling. CT Pelvis CT of the pelvis is not indicated in patients with clinical early-stage disease. There is no relevant literature about the role of CT in the evaluation of primary tumor size and extent, likely explained by the limited soft-tissue contrast of CT. Lymph node enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. If IFLN metastases are suspected on imaging, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not Staging and Follow-up of Vulvar Cancer exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is not indicated in patients with clinical early-stage disease. There is no relevant literature about the role of FDG-PET/CT in the evaluation of primary tumor size and extent. If findings on FDG-PET/CT are suspicious for lymph node metastases, SLN mapping is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. Normal imaging findings do not exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN mapping and sampling.

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Staging and Follow up of Vulvar Cancer

Several recent studies examined the value of FDG-PET/CT in the detection of lymph node metastases. Kamran et al [27] retrospectively evaluated the performance of FDG-PET/CT prior to lymphadenectomy in 20 patients with primary vulvar SCC (size unspecified) and >1 mm of stromal invasion. They found that on a patient-by-patient basis, FDG-PET/CT demonstrated a sensitivity of 50%, specificity of 100%, PPV of 100%, and NPV of 57.1%. Crivellaro et al [28] retrospectively evaluated 29 patients with clinical early-stage primary vulvar cancer (primary tumors <4 cm and stromal invasion >1 mm) who were imaged with FDG-PET/CT prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV of FDG-PET/CT was 53%, 85%, 73%, 76%, and 67% on a groin-based analysis, and 50%, 67%, 59%, 59% on a patient-based analysis, respectively. The authors concluded that FDG- PET/CT had low sensitivity and moderate specificity for detecting IFLN metastases in early-stage disease. Combination of radiocolloid (usually Tc-99m sulfur colloid) and blue dye (most commonly isosulfan blue 1%) has a superior SLN detection rate (97.7%) compared with either Tc-99m sulfur colloid (94%) or blue dye (68.7%) alone [21,23]. Radiocolloid is injected intradermally around the tumor 2 to 4 hours prior to surgery. Preoperative lymphoscintigraphy may help with approximate SLN localization, whereas the intraoperative handheld gamma probe allows more precise SLN detection and localization. Blue dye is injected in the operating room 15 to 30 Staging and Follow-up of Vulvar Cancer minutes prior the procedure and localizes transiently to SLN aiding intraoperative visualization and removal of blue-stained lymph nodes [6]. If the SLN is negative for metastatic spread on pathologic evaluation, lymphadenectomy is omitted [6]. If an ipsilateral SLN is not identified, complete lymphadenectomy is recommended [6].

Staging and Follow up of Vulvar Cancer. Several recent studies examined the value of FDG-PET/CT in the detection of lymph node metastases. Kamran et al [27] retrospectively evaluated the performance of FDG-PET/CT prior to lymphadenectomy in 20 patients with primary vulvar SCC (size unspecified) and >1 mm of stromal invasion. They found that on a patient-by-patient basis, FDG-PET/CT demonstrated a sensitivity of 50%, specificity of 100%, PPV of 100%, and NPV of 57.1%. Crivellaro et al [28] retrospectively evaluated 29 patients with clinical early-stage primary vulvar cancer (primary tumors <4 cm and stromal invasion >1 mm) who were imaged with FDG-PET/CT prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV of FDG-PET/CT was 53%, 85%, 73%, 76%, and 67% on a groin-based analysis, and 50%, 67%, 59%, 59% on a patient-based analysis, respectively. The authors concluded that FDG- PET/CT had low sensitivity and moderate specificity for detecting IFLN metastases in early-stage disease. Combination of radiocolloid (usually Tc-99m sulfur colloid) and blue dye (most commonly isosulfan blue 1%) has a superior SLN detection rate (97.7%) compared with either Tc-99m sulfur colloid (94%) or blue dye (68.7%) alone [21,23]. Radiocolloid is injected intradermally around the tumor 2 to 4 hours prior to surgery. Preoperative lymphoscintigraphy may help with approximate SLN localization, whereas the intraoperative handheld gamma probe allows more precise SLN detection and localization. Blue dye is injected in the operating room 15 to 30 Staging and Follow-up of Vulvar Cancer minutes prior the procedure and localizes transiently to SLN aiding intraoperative visualization and removal of blue-stained lymph nodes [6]. If the SLN is negative for metastatic spread on pathologic evaluation, lymphadenectomy is omitted [6]. If an ipsilateral SLN is not identified, complete lymphadenectomy is recommended [6].

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acrac_3102402_5

Staging and Follow up of Vulvar Cancer

If SLN is involved by tumor, management options include bilateral lymphadenectomy or external beam radiation therapy (EBRT) with or without chemotherapy [6]. In situations when lymph node metastases are suspected because of imaging, SLN is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. MRI Pelvis MRI with IV contrast is the best available imaging modality to define local extent of primary tumor because MRI has superior soft-tissue contrast and multiplanar capability. MRI is considered for patients with primary tumors >2 cm and >1 mm of stromal invasion, or primary tumors with close proximity to or involvement of the urethra, vagina, and anus because MRI can aid primary treatment planning [6]. In addition to primary tumor size/extent, MRI can assess IFLN basins [32-34]. Similar to CT and FDG-PET/CT, if findings on MRI are suspicious for lymph node metastases, SLN is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is indicated [6]. Normal findings do not reliably exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN sampling. Hawnaur et al [35] used unenhanced MRI to evaluate IFLN in 10 patients with primary vulvar cancer. Lymph node metastases were diagnosed if any of the following criteria were present: long-axis diameter >21 mm, short-axis diameter >10 mm, long- to short-axis diameter ratio <1.3:1, irregular contour, and cystic changes within a lymph node. On a groin-based analysis, MRI had the sensitivity of 89%, specificity of 91%, PPV of 89%, NPV of 91%, and accuracy of 90%. Staging and Follow-up of Vulvar Cancer Radiography Chest There is no relevant literature to support the use of routine pretreatment radiographs in patients with clinical early- stage vulvar cancer. There is only one report regarding the role of chest radiography for the initial staging of patients with vulvar cancer.

Staging and Follow up of Vulvar Cancer. If SLN is involved by tumor, management options include bilateral lymphadenectomy or external beam radiation therapy (EBRT) with or without chemotherapy [6]. In situations when lymph node metastases are suspected because of imaging, SLN is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is performed [6]. MRI Pelvis MRI with IV contrast is the best available imaging modality to define local extent of primary tumor because MRI has superior soft-tissue contrast and multiplanar capability. MRI is considered for patients with primary tumors >2 cm and >1 mm of stromal invasion, or primary tumors with close proximity to or involvement of the urethra, vagina, and anus because MRI can aid primary treatment planning [6]. In addition to primary tumor size/extent, MRI can assess IFLN basins [32-34]. Similar to CT and FDG-PET/CT, if findings on MRI are suspicious for lymph node metastases, SLN is not recommended. Instead, complete lymphadenectomy or, alternatively, US-guided FNAB is indicated [6]. Normal findings do not reliably exclude IFLN metastases (because of inadequate sensitivity) and do not alleviate the need for SLN sampling. Hawnaur et al [35] used unenhanced MRI to evaluate IFLN in 10 patients with primary vulvar cancer. Lymph node metastases were diagnosed if any of the following criteria were present: long-axis diameter >21 mm, short-axis diameter >10 mm, long- to short-axis diameter ratio <1.3:1, irregular contour, and cystic changes within a lymph node. On a groin-based analysis, MRI had the sensitivity of 89%, specificity of 91%, PPV of 89%, NPV of 91%, and accuracy of 90%. Staging and Follow-up of Vulvar Cancer Radiography Chest There is no relevant literature to support the use of routine pretreatment radiographs in patients with clinical early- stage vulvar cancer. There is only one report regarding the role of chest radiography for the initial staging of patients with vulvar cancer.

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acrac_3102402_6

Staging and Follow up of Vulvar Cancer

Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent). Chest radiographs were performed in 24 of 27 patients, and contrast-enhanced CT scan of the chest, abdomen, and pelvis was obtained in all 27 patients. Only 1 of 24 chest radiographs revealed a clinically important finding (pulmonary metastases) from an asymptomatic and at that time unknown adenocarcinoma of the cecum. Chest radiographs did not alter initial gynecologic management in any of the patients. US Duplex Doppler and US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US with Doppler and US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who underwent imaging with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US- guided FNAB it was 80%, 100%, 93%, and 100%, respectively. US Duplex Doppler Groin There is no relevant literature to support the use of US with duplex Doppler for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36- 38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment.

Staging and Follow up of Vulvar Cancer. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent). Chest radiographs were performed in 24 of 27 patients, and contrast-enhanced CT scan of the chest, abdomen, and pelvis was obtained in all 27 patients. Only 1 of 24 chest radiographs revealed a clinically important finding (pulmonary metastases) from an asymptomatic and at that time unknown adenocarcinoma of the cecum. Chest radiographs did not alter initial gynecologic management in any of the patients. US Duplex Doppler and US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US with Doppler and US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who underwent imaging with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US- guided FNAB it was 80%, 100%, 93%, and 100%, respectively. US Duplex Doppler Groin There is no relevant literature to support the use of US with duplex Doppler for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36- 38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment.

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acrac_3102402_7

Staging and Follow up of Vulvar Cancer

The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB it was 80%, 100%, 93%, and 100%, respectively. Staging and Follow-up of Vulvar Cancer US-Guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. Sampling is minimally invasive. Limitations of US include its risk of undersampling of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. Variant 3: Initial staging of pretreatment vulvar cancer: Primary tumor is greater than 4 cm or tumor of any size with more than minimal involvement of the urethra, vagina, or anus. Patients with primary tumors >4 cm or tumors of any size with more than minimal involvement of urethra, vagina, or anus (TNM larger T2/T3 or larger FIGO II, FIGO III/IVA) are treated primarily with concurrent EBRT and chemotherapy [39,40]. This group of patients has >8% risk of IFLN basin metastases and are not candidates for SLN mapping and biopsy.

Staging and Follow up of Vulvar Cancer. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB it was 80%, 100%, 93%, and 100%, respectively. Staging and Follow-up of Vulvar Cancer US-Guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. Sampling is minimally invasive. Limitations of US include its risk of undersampling of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. Variant 3: Initial staging of pretreatment vulvar cancer: Primary tumor is greater than 4 cm or tumor of any size with more than minimal involvement of the urethra, vagina, or anus. Patients with primary tumors >4 cm or tumors of any size with more than minimal involvement of urethra, vagina, or anus (TNM larger T2/T3 or larger FIGO II, FIGO III/IVA) are treated primarily with concurrent EBRT and chemotherapy [39,40]. This group of patients has >8% risk of IFLN basin metastases and are not candidates for SLN mapping and biopsy.

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Staging and Follow up of Vulvar Cancer

For radiation planning, IFLN assessment with imaging and subsequent IFLN lymphadenectomy or US-guided FNAB is recommended. If lymph node metastases are identified at imaging and confirmed at lymphadenectomy or US-guided FNAB, the radiation field should encompass primary tumor, pelvis, and groin nodal basin. If no lymph node metastases are detected at imaging and subsequent lymphadenectomy, reduced EBRT coverage may be considered. Patients presenting with distant metastases beyond the pelvis (TNM any T or N designation and M1 beyond pelvis or FIGO IVB) are treated primarily with chemotherapy and, when appropriate, EBRT for locoregional disease control and symptom palliation. CT Abdomen and Pelvis Contrast-enhanced CT of abdomen and pelvis may be considered in patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. However, CT of chest, abdomen, and pelvis or FDG-PET/CT is preferred in this group. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. CT Chest, Abdomen, and Pelvis Contrast-enhanced CT of chest, abdomen, and pelvis may be considered for patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. Staging and Follow-up of Vulvar Cancer and/or abnormal pattern of contrast enhancement were observed. CT had sensitivity of 60%, specificity of 90%, PPV of 37.5%, and NPV of 95.7% for detection of IFLN metastases.

Staging and Follow up of Vulvar Cancer. For radiation planning, IFLN assessment with imaging and subsequent IFLN lymphadenectomy or US-guided FNAB is recommended. If lymph node metastases are identified at imaging and confirmed at lymphadenectomy or US-guided FNAB, the radiation field should encompass primary tumor, pelvis, and groin nodal basin. If no lymph node metastases are detected at imaging and subsequent lymphadenectomy, reduced EBRT coverage may be considered. Patients presenting with distant metastases beyond the pelvis (TNM any T or N designation and M1 beyond pelvis or FIGO IVB) are treated primarily with chemotherapy and, when appropriate, EBRT for locoregional disease control and symptom palliation. CT Abdomen and Pelvis Contrast-enhanced CT of abdomen and pelvis may be considered in patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. However, CT of chest, abdomen, and pelvis or FDG-PET/CT is preferred in this group. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. CT Chest, Abdomen, and Pelvis Contrast-enhanced CT of chest, abdomen, and pelvis may be considered for patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. Staging and Follow-up of Vulvar Cancer and/or abnormal pattern of contrast enhancement were observed. CT had sensitivity of 60%, specificity of 90%, PPV of 37.5%, and NPV of 95.7% for detection of IFLN metastases.

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acrac_3102402_9

Staging and Follow up of Vulvar Cancer

CT did not reveal distant metastases from vulvar cancer or alter original treatment plan in any patients. Incidental synchronous cancers were detected in 2 patients. CT Pelvis Contrast-enhanced CT of pelvis may be considered in patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. However, CT of chest, abdomen, and pelvis or FDG- PET/CT is preferred in this group. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is considered in patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. Several recent studies examined the value of FDG-PET/CT in the detection of lymph node metastases. Kamran et al [27] retrospectively evaluated the performance of FDG-PET/CT prior to lymphadenectomy in 20 patients with primary vulvar SCC (size unspecified) and >1 mm of stromal invasion. He found that on a patient-by-patients basis, FDG-PET/CT demonstrated a sensitivity of 50%, specificity of 100%, PPV of 100%, and NPV of 57.1%. Garganese et al [41] reported on FDG-PET/CT in 47 patients with vulvar cancer (44 primary and 3 recurrent) who underwent inguinofemoral lymphadenectomy because they were not candidates for SLN evaluation because of primary tumor >4 cm (n = 12), multifocal tumor (n = 9), prior surgery (n = 16), IFLN involvement (n = 7) or recurrent disease (n = 3). On a groin-based analysis, FDG-PET/CT demonstrated a sensitivity of 56%, specificity of 88%, PPV of 38%, NPV of 93%, and accuracy of 84% in detecting nodal metastases.

Staging and Follow up of Vulvar Cancer. CT did not reveal distant metastases from vulvar cancer or alter original treatment plan in any patients. Incidental synchronous cancers were detected in 2 patients. CT Pelvis Contrast-enhanced CT of pelvis may be considered in patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. However, CT of chest, abdomen, and pelvis or FDG- PET/CT is preferred in this group. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is considered in patients with primary tumors >4 cm; urethral, vaginal, or anal involvement; or clinical suspicion for lymph node metastases. Complete lymphadenectomy or, alternatively, US-guided FNAB is performed if imaging findings are suspicious for lymph node metastases [6]. Several recent studies examined the value of FDG-PET/CT in the detection of lymph node metastases. Kamran et al [27] retrospectively evaluated the performance of FDG-PET/CT prior to lymphadenectomy in 20 patients with primary vulvar SCC (size unspecified) and >1 mm of stromal invasion. He found that on a patient-by-patients basis, FDG-PET/CT demonstrated a sensitivity of 50%, specificity of 100%, PPV of 100%, and NPV of 57.1%. Garganese et al [41] reported on FDG-PET/CT in 47 patients with vulvar cancer (44 primary and 3 recurrent) who underwent inguinofemoral lymphadenectomy because they were not candidates for SLN evaluation because of primary tumor >4 cm (n = 12), multifocal tumor (n = 9), prior surgery (n = 16), IFLN involvement (n = 7) or recurrent disease (n = 3). On a groin-based analysis, FDG-PET/CT demonstrated a sensitivity of 56%, specificity of 88%, PPV of 38%, NPV of 93%, and accuracy of 84% in detecting nodal metastases.

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acrac_3102402_10

Staging and Follow up of Vulvar Cancer

Robertson et al [30] reported on 50 patients who were enrolled in the National Oncologic PET Registry and underwent 83 FDG-PET/CT studies for suspected or known primary or recurrent vulvar or vaginal cancer. Fifty- four of 83 (65%) studies were performed in patients with vulvar cancer, the remaining 29 of 83 (35%) studies in patients with vaginal cancer. The authors did not specify numbers of patients with vulvar cancer (including primary Lymphoscintigraphy Groin Lymphoscintigraphy and SLN mapping are not indicated in patients with primary tumor >4 cm or tumor of any size with more than minimal involvement of the urethra, vagina, or anus. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. MRI Pelvis MRI with IV contrast is the best available imaging modality to define local extent of primary tumor because MRI has superior soft-tissue contrast and multiplanar capability. MRI is considered for patients with primary tumors >2 cm and >1 mm of stromal invasion, or primary tumors with close proximity to or involvement of the urethra, vagina, and anus because MRI can aid primary treatment planning [6]. In addition to primary tumor, MRI can assess IFLN basins [32-34]. Similar to CT and FDG-PET/CT, if findings on MRI are suspicious for lymph node metastases, complete lymphadenectomy or, alternatively, US-guided FNAB is indicated [6]. Hawnaur et al [35] used unenhanced MRI to evaluate IFLN in 10 patients with primary vulvar cancer. Lymph node metastases were diagnosed if any of the following criteria were present: long-axis diameter >21 mm, short-axis diameter >10 mm, long- to short-axis diameter ratio <1.3:1, irregular contour, and cystic changes within a lymph node.

Staging and Follow up of Vulvar Cancer. Robertson et al [30] reported on 50 patients who were enrolled in the National Oncologic PET Registry and underwent 83 FDG-PET/CT studies for suspected or known primary or recurrent vulvar or vaginal cancer. Fifty- four of 83 (65%) studies were performed in patients with vulvar cancer, the remaining 29 of 83 (35%) studies in patients with vaginal cancer. The authors did not specify numbers of patients with vulvar cancer (including primary Lymphoscintigraphy Groin Lymphoscintigraphy and SLN mapping are not indicated in patients with primary tumor >4 cm or tumor of any size with more than minimal involvement of the urethra, vagina, or anus. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. MRI Pelvis MRI with IV contrast is the best available imaging modality to define local extent of primary tumor because MRI has superior soft-tissue contrast and multiplanar capability. MRI is considered for patients with primary tumors >2 cm and >1 mm of stromal invasion, or primary tumors with close proximity to or involvement of the urethra, vagina, and anus because MRI can aid primary treatment planning [6]. In addition to primary tumor, MRI can assess IFLN basins [32-34]. Similar to CT and FDG-PET/CT, if findings on MRI are suspicious for lymph node metastases, complete lymphadenectomy or, alternatively, US-guided FNAB is indicated [6]. Hawnaur et al [35] used unenhanced MRI to evaluate IFLN in 10 patients with primary vulvar cancer. Lymph node metastases were diagnosed if any of the following criteria were present: long-axis diameter >21 mm, short-axis diameter >10 mm, long- to short-axis diameter ratio <1.3:1, irregular contour, and cystic changes within a lymph node.

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acrac_3102402_11

Staging and Follow up of Vulvar Cancer

On a groin-based analysis, MRI had the sensitivity of 89%, specificity of 91%, PPV of 89%, NPV of 91%, and accuracy of 90%. Staging and Follow-up of Vulvar Cancer with unenhanced MRI prior to lymphadenectomy. Two of the three criteria had to be met to diagnose lymph node metastases: 1) short-axis diameter >10 mm; 2) irregular or rounded shape; or 3) increased signal intensity on short tau inversion recovery imaging or heterogeneous signal intensity on T2-weighted imaging. Using this approach, on a groin-by-groin basis, unenhanced MRI had sensitivity of 85.7%, specificity of 82.1%, PPV of 64.3%, and NPV of 93.9%. Radiography Chest There is only one report regarding the role of chest radiography for the initial staging of patients with vulvar cancer. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent). Chest radiographs was performed in 24 of 27 patients and contrast-enhanced CT scan of the chest, abdomen, and pelvis was performed in all 27 patients. Only 1 of 24 chest radiographs revealed a clinically important finding (ie, pulmonary metastases) from an asymptomatic and at that time unknown adenocarcinoma of the cecum. Chest radiographs did not alter initial gynecologic management in any of the patients. US Duplex Doppler and US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US with Doppler and US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases.

Staging and Follow up of Vulvar Cancer. On a groin-based analysis, MRI had the sensitivity of 89%, specificity of 91%, PPV of 89%, NPV of 91%, and accuracy of 90%. Staging and Follow-up of Vulvar Cancer with unenhanced MRI prior to lymphadenectomy. Two of the three criteria had to be met to diagnose lymph node metastases: 1) short-axis diameter >10 mm; 2) irregular or rounded shape; or 3) increased signal intensity on short tau inversion recovery imaging or heterogeneous signal intensity on T2-weighted imaging. Using this approach, on a groin-by-groin basis, unenhanced MRI had sensitivity of 85.7%, specificity of 82.1%, PPV of 64.3%, and NPV of 93.9%. Radiography Chest There is only one report regarding the role of chest radiography for the initial staging of patients with vulvar cancer. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent). Chest radiographs was performed in 24 of 27 patients and contrast-enhanced CT scan of the chest, abdomen, and pelvis was performed in all 27 patients. Only 1 of 24 chest radiographs revealed a clinically important finding (ie, pulmonary metastases) from an asymptomatic and at that time unknown adenocarcinoma of the cecum. Chest radiographs did not alter initial gynecologic management in any of the patients. US Duplex Doppler and US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US with Doppler and US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases.

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acrac_3102402_12

Staging and Follow up of Vulvar Cancer

US Duplex Doppler Groin There is no relevant literature to support the use of US with Doppler for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. US-Guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. Staging and Follow-up of Vulvar Cancer the largest or most abnormal lymph node in each groin findings were found on US, US-guided FNAB prior to surgical management. The sensitivity and specificity for detecting metastatic involvement were 86% and 96% for US with Doppler alone but increased to 93% and 100% for US-guided FNAB, respectively.

Staging and Follow up of Vulvar Cancer. US Duplex Doppler Groin There is no relevant literature to support the use of US with Doppler for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. US-Guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support the use of US-guided FNAB for the assessment of primary tumor extent. A number of studies evaluated groin US with Doppler and US-guided FNAB for IFLN assessment [25,36-38]. Combined US and US-guided FNAB is the most accurate approach to confirm IFLN metastases that are suspected based on clinical and/or imaging assessment. The advantage of this sampling approach is that it is minimally invasive; the limitation is the potential risk of undersampling in the setting of micrometastases. Staging and Follow-up of Vulvar Cancer the largest or most abnormal lymph node in each groin findings were found on US, US-guided FNAB prior to surgical management. The sensitivity and specificity for detecting metastatic involvement were 86% and 96% for US with Doppler alone but increased to 93% and 100% for US-guided FNAB, respectively.

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acrac_3102402_13

Staging and Follow up of Vulvar Cancer

The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. Vulvar region, IFLN basins, multisite, and distant metastases are the most common locations of recurrence in decreasing order of frequency [13]. If the relapse is confirmed pathologically, FDG-PET/CT is recommended to detect lymph node and distant metastases, whereas MRI of pelvis aids the assessment of local tumor extent [44-46]. Treatment of recurrent vulvar cancer is determined by the tumor extent (vulva-confined, nodal recurrence, distant recurrence) and history of prior EBRT. Detailed discussion is beyond the scope of this guideline and is addressed comprehensively elsewhere [6]. In general, nonirradiated vulva-confined recurrent tumors are approached with multimodal strategy including surgical resection or IFLN dissection, and/or chemoradiotherapy. Surgery is the only potentially curative option in previously irradiated vulva-confined tumors. Similarly, if there is no prior EBRT, isolated lymph node or distant metastases are managed with multimodal approach; surgical resection and systemic therapy may be considered if there was prior EBRT. If there are multiple lymph node or distant metastases and prior EBRT, systemic therapy and best supporting care are advised [6]. CT Abdomen and Pelvis Contrast-enhanced CT of the abdomen and pelvis may be considered in patients with suspected vulvar cancer recurrence. However, contrast-enhanced CT of the chest, abdomen, and pelvis or FDG-PET/CT is preferred in this group. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT.

Staging and Follow up of Vulvar Cancer. The aforementioned study by Land et al [25] reported on 44 patients with primary vulvar SCC who were imaged with one or more of the following modalities: CT, US, and/or US-guided FNAB prior to lymphadenectomy. The sensitivity, specificity, PPV, and NPV for US alone was 87%, 69%, 48%, and 94%, respectively, and for US-guided FNAB 80%, 100%, 93%, and 100%, respectively. Vulvar region, IFLN basins, multisite, and distant metastases are the most common locations of recurrence in decreasing order of frequency [13]. If the relapse is confirmed pathologically, FDG-PET/CT is recommended to detect lymph node and distant metastases, whereas MRI of pelvis aids the assessment of local tumor extent [44-46]. Treatment of recurrent vulvar cancer is determined by the tumor extent (vulva-confined, nodal recurrence, distant recurrence) and history of prior EBRT. Detailed discussion is beyond the scope of this guideline and is addressed comprehensively elsewhere [6]. In general, nonirradiated vulva-confined recurrent tumors are approached with multimodal strategy including surgical resection or IFLN dissection, and/or chemoradiotherapy. Surgery is the only potentially curative option in previously irradiated vulva-confined tumors. Similarly, if there is no prior EBRT, isolated lymph node or distant metastases are managed with multimodal approach; surgical resection and systemic therapy may be considered if there was prior EBRT. If there are multiple lymph node or distant metastases and prior EBRT, systemic therapy and best supporting care are advised [6]. CT Abdomen and Pelvis Contrast-enhanced CT of the abdomen and pelvis may be considered in patients with suspected vulvar cancer recurrence. However, contrast-enhanced CT of the chest, abdomen, and pelvis or FDG-PET/CT is preferred in this group. Size enlargement and abnormal pattern of enhancement are the main criteria used to detect lymph node metastases on CT.

3102402

acrac_3102402_14

Staging and Follow up of Vulvar Cancer

Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent) who underwent contrast-enhanced CT scan of the chest, abdomen, and pelvis prior to treatment. IFLN metastases were diagnosed if short-axis diameter was >10 mm and/or abnormal pattern of contrast enhancement were observed. CT had sensitivity of 60%, specificity of 90%, PPV of 37.5%, and NPV of 95.7% for detection of IFLN metastases. CT did not reveal distant metastases from vulvar cancer or alter original treatment plan in any patients. Incidental synchronous cancers were detected in two patients. CT Chest, Abdomen, and Pelvis Contrast-enhanced CT of the chest, abdomen, and pelvis is usually appropriate in patients with suspected vulvar cancer recurrence. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent) who underwent contrast-enhanced CT scan of the chest, abdomen, and pelvis prior to treatment. IFLN metastases were diagnosed if short-axis diameter was >10 mm and/or abnormal pattern of contrast enhancement were observed. CT had sensitivity of 60%, specificity of 90%, PPV of 37.5%, and NPV of 95.7% for detection of IFLN metastases. CT Pelvis Contrast-enhanced CT of the pelvis may be considered in patients with suspected vulvar cancer recurrence. However, contrast-enhanced CT of chest, abdomen, and pelvis or FDG-PET/CT is preferred in this group. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is usually appropriate in patients with suspected vulvar cancer recurrence. FDG-PET/CT, alone or in combination with MRI, can facilitate treatment planning prior to pelvic exenteration for recurrent gynecologic cancers.

Staging and Follow up of Vulvar Cancer. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent) who underwent contrast-enhanced CT scan of the chest, abdomen, and pelvis prior to treatment. IFLN metastases were diagnosed if short-axis diameter was >10 mm and/or abnormal pattern of contrast enhancement were observed. CT had sensitivity of 60%, specificity of 90%, PPV of 37.5%, and NPV of 95.7% for detection of IFLN metastases. CT did not reveal distant metastases from vulvar cancer or alter original treatment plan in any patients. Incidental synchronous cancers were detected in two patients. CT Chest, Abdomen, and Pelvis Contrast-enhanced CT of the chest, abdomen, and pelvis is usually appropriate in patients with suspected vulvar cancer recurrence. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent) who underwent contrast-enhanced CT scan of the chest, abdomen, and pelvis prior to treatment. IFLN metastases were diagnosed if short-axis diameter was >10 mm and/or abnormal pattern of contrast enhancement were observed. CT had sensitivity of 60%, specificity of 90%, PPV of 37.5%, and NPV of 95.7% for detection of IFLN metastases. CT Pelvis Contrast-enhanced CT of the pelvis may be considered in patients with suspected vulvar cancer recurrence. However, contrast-enhanced CT of chest, abdomen, and pelvis or FDG-PET/CT is preferred in this group. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is usually appropriate in patients with suspected vulvar cancer recurrence. FDG-PET/CT, alone or in combination with MRI, can facilitate treatment planning prior to pelvic exenteration for recurrent gynecologic cancers.

3102402

acrac_3102402_15

Staging and Follow up of Vulvar Cancer

Garganese et al [41] reported on FDG-PET/CT in 47 patients with vulvar cancer (44 primary and 3 recurrent) who underwent inguinofemoral lymphadenectomy because they were not candidates for SLN evaluation because of primary tumor >4 cm (n = 12), multifocal tumor (n = 9), prior surgery (n = 16), IFLN involvement (n = 7) or recurrent disease (n = 3). On a groin-based analysis, FDG-PET/CT demonstrated a sensitivity of 56%, specificity of 88%, PPV of 38%, NPV of 93%, and accuracy of 84% in detecting nodal metastases. MRI Pelvis MRI of the pelvis has superior soft-tissue contrast and multiplanar capability. Thus, MRI is the preferred imaging modality to define local extent of suspected or confirmed recurrent vulvar cancers, especially those in close proximity to or involving the urethra, vagina, and anus as it can aid treatment planning [6]. Contrast-enhanced imaging is advised [6]. Radiography Chest Routine radiography is usually not indicated in the evaluation of clinically suspected recurrence of vulvar cancer. There is only one report regarding the role of chest radiography for the initial staging of patients with vulvar cancer. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent). Chest radiographs was performed in 24 of 27 patients and contrast-enhanced CT scan of the chest, abdomen, and pelvis was performed in all 27 patients. Only 1 of 24 chest radiographs revealed a clinically important finding (pulmonary metastases) from an asymptomatic and at that time unknown adenocarcinoma of the cecum. Chest radiographs did not alter initial gynecologic management in any of the patients. Staging and Follow-up of Vulvar Cancer US Duplex Doppler and US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support or refute the use of US with Doppler and US-guided FNAB for the assessment of suspected vulvar cancer recurrence. Nevertheless, this procedure may be useful to confirm suspected IFLN metastases.

Staging and Follow up of Vulvar Cancer. Garganese et al [41] reported on FDG-PET/CT in 47 patients with vulvar cancer (44 primary and 3 recurrent) who underwent inguinofemoral lymphadenectomy because they were not candidates for SLN evaluation because of primary tumor >4 cm (n = 12), multifocal tumor (n = 9), prior surgery (n = 16), IFLN involvement (n = 7) or recurrent disease (n = 3). On a groin-based analysis, FDG-PET/CT demonstrated a sensitivity of 56%, specificity of 88%, PPV of 38%, NPV of 93%, and accuracy of 84% in detecting nodal metastases. MRI Pelvis MRI of the pelvis has superior soft-tissue contrast and multiplanar capability. Thus, MRI is the preferred imaging modality to define local extent of suspected or confirmed recurrent vulvar cancers, especially those in close proximity to or involving the urethra, vagina, and anus as it can aid treatment planning [6]. Contrast-enhanced imaging is advised [6]. Radiography Chest Routine radiography is usually not indicated in the evaluation of clinically suspected recurrence of vulvar cancer. There is only one report regarding the role of chest radiography for the initial staging of patients with vulvar cancer. Andersen et al [26] prospectively evaluated 27 patients with vulvar cancer (23 primary and 4 recurrent). Chest radiographs was performed in 24 of 27 patients and contrast-enhanced CT scan of the chest, abdomen, and pelvis was performed in all 27 patients. Only 1 of 24 chest radiographs revealed a clinically important finding (pulmonary metastases) from an asymptomatic and at that time unknown adenocarcinoma of the cecum. Chest radiographs did not alter initial gynecologic management in any of the patients. Staging and Follow-up of Vulvar Cancer US Duplex Doppler and US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support or refute the use of US with Doppler and US-guided FNAB for the assessment of suspected vulvar cancer recurrence. Nevertheless, this procedure may be useful to confirm suspected IFLN metastases.

3102402

acrac_70547_0

Hematospermia

Introduction/Background Hematospermia (HS), or hemospermia, the presence of blood in the ejaculate or semen, has been recognized for centuries. Although it is not uncommon to encounter HS in clinical practice, the exact prevalence and incidence are not known. Most men with HS are young (<40 years of age), and HS may occur either as a single episode or repeatedly over time. It is typically a cause of great anxiety to men, mainly because of the imagined possibility of underlying malignancy or venereal disease. HS may be associated with pathology in the prostate gland, seminal tract (seminal vesicles, vasa deferentia, and ejaculatory ducts), verumontanum, urethra, urinary bladder, epididymis, or testes, with cited causes reported to include prior prostatic biopsy, prostatic calculi, inflammatory or infectious conditions such as prostatitis or seminal vesiculitis, ductal obstruction, prostatic cyst formation, and rarely vascular malformations. The majority of cases of HS were thought to be idiopathic in nature; however, as a result of improved imaging techniques, the number of cases labeled as idiopathic has decreased significantly, with one of the main sites of bleeding occurring in the seminal vesicles [1-13]. Of specific etiologies, infectious or inflammatory conditions are the most common, accounting for approximately 40% of HS cases overall. An infectious or inflammatory condition of the urogenital tract is the most common etiology in men <40 years of age [1,6,9,14]. Overview of Imaging Modalities Transrectal ultrasound (ultrasound pelvis [prostate] transrectal) Transrectal ultrasound (TRUS) is a safe, inexpensive, effective, noninvasive, radiation-free imaging technique often used as the primary screening or diagnostic modality in men with HS to evaluate the prostate gland and seminal tract. Patients are typically placed in the left lateral decubitus position, and grayscale images are obtained with a 5.0- to 10-MHz TRUS transducer in axial and sagittal planes [12,13,19,20].

Hematospermia. Introduction/Background Hematospermia (HS), or hemospermia, the presence of blood in the ejaculate or semen, has been recognized for centuries. Although it is not uncommon to encounter HS in clinical practice, the exact prevalence and incidence are not known. Most men with HS are young (<40 years of age), and HS may occur either as a single episode or repeatedly over time. It is typically a cause of great anxiety to men, mainly because of the imagined possibility of underlying malignancy or venereal disease. HS may be associated with pathology in the prostate gland, seminal tract (seminal vesicles, vasa deferentia, and ejaculatory ducts), verumontanum, urethra, urinary bladder, epididymis, or testes, with cited causes reported to include prior prostatic biopsy, prostatic calculi, inflammatory or infectious conditions such as prostatitis or seminal vesiculitis, ductal obstruction, prostatic cyst formation, and rarely vascular malformations. The majority of cases of HS were thought to be idiopathic in nature; however, as a result of improved imaging techniques, the number of cases labeled as idiopathic has decreased significantly, with one of the main sites of bleeding occurring in the seminal vesicles [1-13]. Of specific etiologies, infectious or inflammatory conditions are the most common, accounting for approximately 40% of HS cases overall. An infectious or inflammatory condition of the urogenital tract is the most common etiology in men <40 years of age [1,6,9,14]. Overview of Imaging Modalities Transrectal ultrasound (ultrasound pelvis [prostate] transrectal) Transrectal ultrasound (TRUS) is a safe, inexpensive, effective, noninvasive, radiation-free imaging technique often used as the primary screening or diagnostic modality in men with HS to evaluate the prostate gland and seminal tract. Patients are typically placed in the left lateral decubitus position, and grayscale images are obtained with a 5.0- to 10-MHz TRUS transducer in axial and sagittal planes [12,13,19,20].

70547

acrac_70547_1

Hematospermia

Color and power Doppler images can also be acquired, particularly when prostate cancer is suspected and prostatic biopsy is contemplated [2,8]. TRUS-guided aspiration or biopsy of the seminal vesicles or prostate gland can be performed to further elucidate the site of bleeding, to provide a definitive diagnosis if a lesion is detected, or to confirm the presence of ejaculatory duct obstruction [4,15]. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through society representation on expert panels. Participation by representatives from collaborating societies on the expert panel does not necessarily imply individual or society endorsement of the final document. Reprint requests to: [email protected] Hematospermia Magnetic resonance imaging Magnetic resonance imaging (MRI), with its excellent soft-tissue contrast, provides radiation-free, multiplanar, high-spatial-resolution anatomic evaluation of the prostate gland and seminal tract. Imaging should be performed at either 1.5T or 3T, although there is no consensus at this time on the appropriate coil selection or field strength. The fundamental advantage of 3T over 1.5T is increased signal-to-noise ratio, which improves the spatial, temporal, and spectral resolution. Comparable performance between multichannel phased array coil MRI of the prostate at 3T and endorectal phased array coil MRI at 1.5T has been reported [20]. As opposed to TRUS, MRI is operator independent and can be performed when TRUS is unsatisfactory or nondiagnostic. Subsequently, small field-of-view axial T1-weighted images and axial, sagittal, and coronal T2-weighted images are obtained for high- resolution evaluation of the prostate gland, seminal vesicles, ejaculatory ducts, and ampullary portions of the vasa deferentia, followed by large field-of-view images to evaluate for pelvic lymphadenopathy [3,7,10,21].

Hematospermia. Color and power Doppler images can also be acquired, particularly when prostate cancer is suspected and prostatic biopsy is contemplated [2,8]. TRUS-guided aspiration or biopsy of the seminal vesicles or prostate gland can be performed to further elucidate the site of bleeding, to provide a definitive diagnosis if a lesion is detected, or to confirm the presence of ejaculatory duct obstruction [4,15]. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through society representation on expert panels. Participation by representatives from collaborating societies on the expert panel does not necessarily imply individual or society endorsement of the final document. Reprint requests to: [email protected] Hematospermia Magnetic resonance imaging Magnetic resonance imaging (MRI), with its excellent soft-tissue contrast, provides radiation-free, multiplanar, high-spatial-resolution anatomic evaluation of the prostate gland and seminal tract. Imaging should be performed at either 1.5T or 3T, although there is no consensus at this time on the appropriate coil selection or field strength. The fundamental advantage of 3T over 1.5T is increased signal-to-noise ratio, which improves the spatial, temporal, and spectral resolution. Comparable performance between multichannel phased array coil MRI of the prostate at 3T and endorectal phased array coil MRI at 1.5T has been reported [20]. As opposed to TRUS, MRI is operator independent and can be performed when TRUS is unsatisfactory or nondiagnostic. Subsequently, small field-of-view axial T1-weighted images and axial, sagittal, and coronal T2-weighted images are obtained for high- resolution evaluation of the prostate gland, seminal vesicles, ejaculatory ducts, and ampullary portions of the vasa deferentia, followed by large field-of-view images to evaluate for pelvic lymphadenopathy [3,7,10,21].

70547

acrac_70547_2

Hematospermia

The increasing availability of 3T MRI, which offers a higher signal-to-noise ratio and improved spatial resolution, may preclude the use of an endorectal coil for evaluating the seminal tract [22]. Computed tomography Computed tomography (CT) is a noninvasive imaging modality that uses ionizing radiation to identify calcifications, gross soft-tissue masses, or cystic lesions of the prostate gland and seminal vesicles. However, it has limited value in the etiologic determination of HS given its lack of soft-tissue contrast and limitation in differentiating structural changes of the prostate and seminal tract [11,21]. Pelvic angiography Pelvic angiography can be useful to evaluate for vascular causes of HS and is mainly reserved for men with intractable HS with or without hematuria when clinical, laboratory, and noninvasive imaging evaluations have not revealed an etiology. If an arterial source of hemorrhage is identified, such as from the internal pudendal artery, transcatheter arterial embolization can be performed in the same session for therapeutic purposes [23]. Discussion of Imaging Modalities by Variant Factors that determine the extent of investigation are patient age, duration of HS, and associated symptoms and signs. However, a confounding issue is that currently there are no consensus or society guidelines on the distinction between transient or episodic HS and persistent HS. The distinction has been based on either the number of ejaculates or a specific time period, with differing opinions. Ultimately the decision to pursue further investigation will be made by the referring physician, typically a urologist. TRUS Many investigators have reported that TRUS should be used as the first-line imaging tool in this patient population. TRUS is very sensitive for detecting a variety of abnormalities that may involve the prostate gland and seminal tract in the setting of HS, reportedly demonstrating abnormalities in 82% to 95% of men with HS [4,12,13,19,21].

Hematospermia. The increasing availability of 3T MRI, which offers a higher signal-to-noise ratio and improved spatial resolution, may preclude the use of an endorectal coil for evaluating the seminal tract [22]. Computed tomography Computed tomography (CT) is a noninvasive imaging modality that uses ionizing radiation to identify calcifications, gross soft-tissue masses, or cystic lesions of the prostate gland and seminal vesicles. However, it has limited value in the etiologic determination of HS given its lack of soft-tissue contrast and limitation in differentiating structural changes of the prostate and seminal tract [11,21]. Pelvic angiography Pelvic angiography can be useful to evaluate for vascular causes of HS and is mainly reserved for men with intractable HS with or without hematuria when clinical, laboratory, and noninvasive imaging evaluations have not revealed an etiology. If an arterial source of hemorrhage is identified, such as from the internal pudendal artery, transcatheter arterial embolization can be performed in the same session for therapeutic purposes [23]. Discussion of Imaging Modalities by Variant Factors that determine the extent of investigation are patient age, duration of HS, and associated symptoms and signs. However, a confounding issue is that currently there are no consensus or society guidelines on the distinction between transient or episodic HS and persistent HS. The distinction has been based on either the number of ejaculates or a specific time period, with differing opinions. Ultimately the decision to pursue further investigation will be made by the referring physician, typically a urologist. TRUS Many investigators have reported that TRUS should be used as the first-line imaging tool in this patient population. TRUS is very sensitive for detecting a variety of abnormalities that may involve the prostate gland and seminal tract in the setting of HS, reportedly demonstrating abnormalities in 82% to 95% of men with HS [4,12,13,19,21].

70547

acrac_70547_3

Hematospermia

Abnormalities may include calcifications or calculi in the prostate, ejaculatory ducts, or seminal Hematospermia vesicles; seminal vesicle, ejaculatory duct, or prostatic cysts; benign prostatic hypertrophy; prostatitis; and Cowper gland masses. However, it is important to consider that some of these abnormalities can be found in asymptomatic patients, such as benign prostatic hyperplasia and prostatic calcifications, which are age-related changes, and nonobstructing prostatic cysts [10,13,28,29]. TRUS has shown utility in guiding transperineal aspiration of the seminal vesicles [4]. A recent prospective trial enrolled 106 patients with persistent HS and found the diagnostic accuracy of TRUS and transurethral seminal vesiculoscopy was 45.3% and 74.5%, respectively, although the diagnostic accuracy was higher when both modalities were combined. Vesiculoscopy was most useful in the detection of calculi and obstruction/stricture at the level of the verumontanum orifice or ejaculatory duct [19]. Computed tomography CT has very limited value in the etiologic determination of HS for the reasons described above. Pelvic angiography Angiography has been reported sparsely in the literature to be useful for vascular masses when evaluating men with intractable HS with or without hematuria when clinical, laboratory, and noninvasive imaging evaluations have not revealed the etiology. If an arterial source of hemorrhage is identified, transcatheter arterial embolization can be performed during the same session as well [23]. Although there are references that report on studies with design limitations, 3 good-quality studies provide good evidence. Relative Radiation Level Information Potential adverse health effects associated with radiation exposure are an important factor to consider when selecting the appropriate imaging procedure. Because there is a wide range of radiation exposures associated with

Hematospermia. Abnormalities may include calcifications or calculi in the prostate, ejaculatory ducts, or seminal Hematospermia vesicles; seminal vesicle, ejaculatory duct, or prostatic cysts; benign prostatic hypertrophy; prostatitis; and Cowper gland masses. However, it is important to consider that some of these abnormalities can be found in asymptomatic patients, such as benign prostatic hyperplasia and prostatic calcifications, which are age-related changes, and nonobstructing prostatic cysts [10,13,28,29]. TRUS has shown utility in guiding transperineal aspiration of the seminal vesicles [4]. A recent prospective trial enrolled 106 patients with persistent HS and found the diagnostic accuracy of TRUS and transurethral seminal vesiculoscopy was 45.3% and 74.5%, respectively, although the diagnostic accuracy was higher when both modalities were combined. Vesiculoscopy was most useful in the detection of calculi and obstruction/stricture at the level of the verumontanum orifice or ejaculatory duct [19]. Computed tomography CT has very limited value in the etiologic determination of HS for the reasons described above. Pelvic angiography Angiography has been reported sparsely in the literature to be useful for vascular masses when evaluating men with intractable HS with or without hematuria when clinical, laboratory, and noninvasive imaging evaluations have not revealed the etiology. If an arterial source of hemorrhage is identified, transcatheter arterial embolization can be performed during the same session as well [23]. Although there are references that report on studies with design limitations, 3 good-quality studies provide good evidence. Relative Radiation Level Information Potential adverse health effects associated with radiation exposure are an important factor to consider when selecting the appropriate imaging procedure. Because there is a wide range of radiation exposures associated with

70547

acrac_69477_0

Dizziness and Ataxia

aUniversity of Cincinnati Medical Center, Cincinnati, Ohio. bResearch Author, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. cPanel Chair, Uniformed Services University, Bethesda, Maryland. dOhio State University, Columbus, Ohio. eUCONN Health, University of Connecticut, Farmington, Connecticut; Neurosurgery expert. fMontefiore Medical Center, Bronx, New York. gYale University School of Medicine, New Haven, Connecticut; Committee on Emergency Radiology-GSER. hWeill Cornell Medical College, New York, New York. iUniversity of Chicago, Chicago, Illinois. jUniversity of Arizona, Tucson, Arizona; Commission on Nuclear Medicine and Molecular Imaging. kUniversity of California Los Angeles, Los Angeles, California. lUniversity of California San Diego, San Diego, California. mOregon Health & Science University, Portland, Oregon. nUT Southwestern Medical Center, Dallas, Texas; American Academy of Neurology. oSchmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida; American College of Emergency Physicians. pThe University of Vermont Medical Center, Burlington, Vermont. qNaval Medical Center Portsmouth, Portsmouth, Virginia. rColumbia University Medical Center, New York, New York. sAssociation for Utah Community Health, Salt Lake City, Utah; American Academy of Family Physicians. tSpecialty Chair, University of Iowa Hospitals and Clinics, Iowa City, Iowa. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization. Reprint requests to: [email protected] Dizziness and Ataxia

Dizziness and Ataxia. aUniversity of Cincinnati Medical Center, Cincinnati, Ohio. bResearch Author, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. cPanel Chair, Uniformed Services University, Bethesda, Maryland. dOhio State University, Columbus, Ohio. eUCONN Health, University of Connecticut, Farmington, Connecticut; Neurosurgery expert. fMontefiore Medical Center, Bronx, New York. gYale University School of Medicine, New Haven, Connecticut; Committee on Emergency Radiology-GSER. hWeill Cornell Medical College, New York, New York. iUniversity of Chicago, Chicago, Illinois. jUniversity of Arizona, Tucson, Arizona; Commission on Nuclear Medicine and Molecular Imaging. kUniversity of California Los Angeles, Los Angeles, California. lUniversity of California San Diego, San Diego, California. mOregon Health & Science University, Portland, Oregon. nUT Southwestern Medical Center, Dallas, Texas; American Academy of Neurology. oSchmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida; American College of Emergency Physicians. pThe University of Vermont Medical Center, Burlington, Vermont. qNaval Medical Center Portsmouth, Portsmouth, Virginia. rColumbia University Medical Center, New York, New York. sAssociation for Utah Community Health, Salt Lake City, Utah; American Academy of Family Physicians. tSpecialty Chair, University of Iowa Hospitals and Clinics, Iowa City, Iowa. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization. Reprint requests to: [email protected] Dizziness and Ataxia

69477

acrac_69477_1

Dizziness and Ataxia

Special Imaging Considerations Conventional diagnostic angiography (DA) may be used in a specific subset of patients presenting with episodic vertigo if there is clinical concern for positional insufficiency of the posterior circulation. DA allows for real-time dynamic evaluation of vessel patency in various neck positions, which is difficult to accomplish via conventional CT angiography (CTA) or MR angiography (MRA) imaging. This information may inform subsequent surgical management in these patients [12]. DA also remains the reference standard for confirmation of clinically suspected VBI as well as vertebral artery dissection, both of which can present with vertigo [13,14]. In cases of vertebral artery dissection documented by DA, however, CTA demonstrated a similar sensitivity approaching 100% [14]. The risks of DA include the potential for rare, severe, or life-threatening allergic-like reactions to contrast media, renal injury related to iodinated contrast administration, potential vascular injury, or infarct due to catheter manipulation in the vessels, as well as local complications at the vascular access site to include infection, hematoma, pseudoaneurysm, or arterial occlusion. Transcranial Doppler ultrasound (US) has demonstrated differences in vascular flow parameters between patients with vertigo related to VBI, patients with vertigo unrelated to VBI, and asymptomatic controls, making this a potential diagnostic tool in the characterization of vertigo [13,15]. Brain PET using various radiotracers has shown promise in detecting presymptomatic neuronal dysfunction in patients with certain types of spinocerebellar ataxia (SCA) and in asymptomatic mutation carriers. Changes on PET were detectable sooner than alterations in morphology and signal intensity on conventional MRI. As such, PET may be a valuable adjunct to conventional brain imaging in this patient population [16,17]. OR Discussion of Procedures by Variant Variant 1: Adult. Brief episodic vertigo.

Dizziness and Ataxia. Special Imaging Considerations Conventional diagnostic angiography (DA) may be used in a specific subset of patients presenting with episodic vertigo if there is clinical concern for positional insufficiency of the posterior circulation. DA allows for real-time dynamic evaluation of vessel patency in various neck positions, which is difficult to accomplish via conventional CT angiography (CTA) or MR angiography (MRA) imaging. This information may inform subsequent surgical management in these patients [12]. DA also remains the reference standard for confirmation of clinically suspected VBI as well as vertebral artery dissection, both of which can present with vertigo [13,14]. In cases of vertebral artery dissection documented by DA, however, CTA demonstrated a similar sensitivity approaching 100% [14]. The risks of DA include the potential for rare, severe, or life-threatening allergic-like reactions to contrast media, renal injury related to iodinated contrast administration, potential vascular injury, or infarct due to catheter manipulation in the vessels, as well as local complications at the vascular access site to include infection, hematoma, pseudoaneurysm, or arterial occlusion. Transcranial Doppler ultrasound (US) has demonstrated differences in vascular flow parameters between patients with vertigo related to VBI, patients with vertigo unrelated to VBI, and asymptomatic controls, making this a potential diagnostic tool in the characterization of vertigo [13,15]. Brain PET using various radiotracers has shown promise in detecting presymptomatic neuronal dysfunction in patients with certain types of spinocerebellar ataxia (SCA) and in asymptomatic mutation carriers. Changes on PET were detectable sooner than alterations in morphology and signal intensity on conventional MRI. As such, PET may be a valuable adjunct to conventional brain imaging in this patient population [16,17]. OR Discussion of Procedures by Variant Variant 1: Adult. Brief episodic vertigo.

69477

acrac_69477_2

Dizziness and Ataxia

Triggered by specific head movements (eg, Dix-Hallpike maneuver). Initial imaging. Brief episodic vertigo triggered by specific head movements (eg, Dix-Hallpike maneuver) is referred to as triggered episodic vestibular syndrome (t-EVS) and is most commonly due to BPPV; however, more ominous central causes may clinically mimic BPPV and are collectively referred to as CPPV [1]. BPPV results from mobile debris (canaliths) in the vestibular labyrinth. The diagnostic criteria for BPPV are clinical and well established [18-20]. Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. In contrast, patients with CPPV present with symptoms of t-EVS but negative or atypical Dix-Hallpike testing are at an increased risk of having an acute central cause of vertigo identified on imaging [6]. Potential causes of CPPV include mass lesions, hemorrhage, infarct, and demyelinating disease, among others [1]. Other specific t-EVS subgroups may also be at increased risk of incidental but potentially actionable findings on neuroimaging, including those with subjective symptoms but no objective signs, younger patients with post-traumatic onset of symptoms, elderly patients, those unresponsive to repositioning maneuvers, and those with short-term recurrence of symptoms [21]. Vestibular migraine may mimic a variety of causes of dizziness and vertigo, including t-EVS, but often has associated headache and other migrainous features (ie, photophobia, phonophobia, visual aura) [20,22]. Imaging is not required to Dizziness and Ataxia diagnose vestibular migraine. To summarize, most patients with t-EVS will not require imaging unless there are atypical features such as lack of nystagmus on provoking maneuvers (eg, Dix-Hallpike) or lack of response to treatment maneuvers (eg, Epley). CT Head With IV Contrast There is no relevant literature regarding the use of CT head with IV contrast in the evaluation of t-EVS.

Dizziness and Ataxia. Triggered by specific head movements (eg, Dix-Hallpike maneuver). Initial imaging. Brief episodic vertigo triggered by specific head movements (eg, Dix-Hallpike maneuver) is referred to as triggered episodic vestibular syndrome (t-EVS) and is most commonly due to BPPV; however, more ominous central causes may clinically mimic BPPV and are collectively referred to as CPPV [1]. BPPV results from mobile debris (canaliths) in the vestibular labyrinth. The diagnostic criteria for BPPV are clinical and well established [18-20]. Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. In contrast, patients with CPPV present with symptoms of t-EVS but negative or atypical Dix-Hallpike testing are at an increased risk of having an acute central cause of vertigo identified on imaging [6]. Potential causes of CPPV include mass lesions, hemorrhage, infarct, and demyelinating disease, among others [1]. Other specific t-EVS subgroups may also be at increased risk of incidental but potentially actionable findings on neuroimaging, including those with subjective symptoms but no objective signs, younger patients with post-traumatic onset of symptoms, elderly patients, those unresponsive to repositioning maneuvers, and those with short-term recurrence of symptoms [21]. Vestibular migraine may mimic a variety of causes of dizziness and vertigo, including t-EVS, but often has associated headache and other migrainous features (ie, photophobia, phonophobia, visual aura) [20,22]. Imaging is not required to Dizziness and Ataxia diagnose vestibular migraine. To summarize, most patients with t-EVS will not require imaging unless there are atypical features such as lack of nystagmus on provoking maneuvers (eg, Dix-Hallpike) or lack of response to treatment maneuvers (eg, Epley). CT Head With IV Contrast There is no relevant literature regarding the use of CT head with IV contrast in the evaluation of t-EVS.

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CT Head Without and With IV Contrast There is no relevant literature regarding the use of CT head without and with IV contrast in the evaluation of t-EVS. CT Head Without IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. Head CT allows for the evaluation of the intracranial structures; however, soft tissue contrast is inferior to MRI. In a single-center retrospective study of patients with episodic vertigo triggered by specific head movements but negative or atypical Dix-Hallpike testing (CPPV), CT detected acute brain lesions in 6% of cases compared with 11% with MRI [6]. CT Temporal Bone With IV Contrast There is no relevant literature regarding the use of CT temporal bone with IV contrast in the evaluation of t-EVS. CT Temporal Bone Without and With IV Contrast There is no relevant literature regarding the use of CT temporal bone without and with IV contrast in the evaluation of t-EVS. CT Temporal Bone Without IV Contrast There is no relevant literature regarding the use of CT temporal bone without IV contrast in the evaluation of t- EVS. CTA Head and Neck With IV Contrast There is no relevant literature regarding the use of CTA head and neck with IV contrast in the evaluation of t-EVS. MRA Head and Neck With IV Contrast There is no relevant literature regarding the use of MRA head and neck with IV contrast in the evaluation of t-EVS. MRA Head and Neck Without and With IV Contrast There is no relevant literature regarding the use of MRA head and neck without and with IV contrast in the evaluation of t-EVS. MRA Head and Neck Without IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRA allows for evaluation of the course and luminal caliber of the arteries. MRA can also detect luminal filling defects, which may include thrombus, embolus, atherosclerotic plaque, dissection flap, or vascular web.

Dizziness and Ataxia. CT Head Without and With IV Contrast There is no relevant literature regarding the use of CT head without and with IV contrast in the evaluation of t-EVS. CT Head Without IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. Head CT allows for the evaluation of the intracranial structures; however, soft tissue contrast is inferior to MRI. In a single-center retrospective study of patients with episodic vertigo triggered by specific head movements but negative or atypical Dix-Hallpike testing (CPPV), CT detected acute brain lesions in 6% of cases compared with 11% with MRI [6]. CT Temporal Bone With IV Contrast There is no relevant literature regarding the use of CT temporal bone with IV contrast in the evaluation of t-EVS. CT Temporal Bone Without and With IV Contrast There is no relevant literature regarding the use of CT temporal bone without and with IV contrast in the evaluation of t-EVS. CT Temporal Bone Without IV Contrast There is no relevant literature regarding the use of CT temporal bone without IV contrast in the evaluation of t- EVS. CTA Head and Neck With IV Contrast There is no relevant literature regarding the use of CTA head and neck with IV contrast in the evaluation of t-EVS. MRA Head and Neck With IV Contrast There is no relevant literature regarding the use of MRA head and neck with IV contrast in the evaluation of t-EVS. MRA Head and Neck Without and With IV Contrast There is no relevant literature regarding the use of MRA head and neck without and with IV contrast in the evaluation of t-EVS. MRA Head and Neck Without IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRA allows for evaluation of the course and luminal caliber of the arteries. MRA can also detect luminal filling defects, which may include thrombus, embolus, atherosclerotic plaque, dissection flap, or vascular web.

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MRA of the head and neck can be performed either without intravenous (IV) contrast, with IV contrast, or as a combination of without and with IV contrast. Although imaging is not required in the setting of typical BPPV, MRA of the head and neck has been used to detect abnormalities of the carotid or vertebrobasilar arteries, which may be associated with symptoms of BPPV. The prevalence of vascular changes is higher in the elderly population with underlying comorbidities such as diabetes, hypertension, and hyperlipidemia in a small prospective study of 126 patients [23]. MRI Cervical and Thoracic Spine With IV Contrast There is no relevant literature regarding the use of MRI cervical and thoracic spine with IV contrast in the evaluation of t-EVS. MRI Cervical and Thoracic Spine Without and With IV Contrast There is no relevant literature regarding the use of MRI cervical and thoracic spine without and with IV contrast in the evaluation of t-EVS. MRI Cervical and Thoracic Spine Without IV Contrast There is no relevant literature regarding the use of MRI cervical and thoracic spine without IV contrast in the evaluation of t-EVS. MRI Head and Internal Auditory Canal With IV Contrast There is no relevant literature regarding the use of MRI head and internal auditory canal (IAC) with IV contrast in the evaluation of t-EVS. Dizziness and Ataxia MRI Head and Internal Auditory Canal Without and With IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRI of the head and IACs provides evaluation of the entire brain with the addition of specific sequences tailored toward assessment of the skull base and associated cranial nerves. The addition of these sequences allows improved detection of small masses and compressive vascular lesions (ie, aneurysm, vessel tortuosity) in and around the IACs.

Dizziness and Ataxia. MRA of the head and neck can be performed either without intravenous (IV) contrast, with IV contrast, or as a combination of without and with IV contrast. Although imaging is not required in the setting of typical BPPV, MRA of the head and neck has been used to detect abnormalities of the carotid or vertebrobasilar arteries, which may be associated with symptoms of BPPV. The prevalence of vascular changes is higher in the elderly population with underlying comorbidities such as diabetes, hypertension, and hyperlipidemia in a small prospective study of 126 patients [23]. MRI Cervical and Thoracic Spine With IV Contrast There is no relevant literature regarding the use of MRI cervical and thoracic spine with IV contrast in the evaluation of t-EVS. MRI Cervical and Thoracic Spine Without and With IV Contrast There is no relevant literature regarding the use of MRI cervical and thoracic spine without and with IV contrast in the evaluation of t-EVS. MRI Cervical and Thoracic Spine Without IV Contrast There is no relevant literature regarding the use of MRI cervical and thoracic spine without IV contrast in the evaluation of t-EVS. MRI Head and Internal Auditory Canal With IV Contrast There is no relevant literature regarding the use of MRI head and internal auditory canal (IAC) with IV contrast in the evaluation of t-EVS. Dizziness and Ataxia MRI Head and Internal Auditory Canal Without and With IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRI of the head and IACs provides evaluation of the entire brain with the addition of specific sequences tailored toward assessment of the skull base and associated cranial nerves. The addition of these sequences allows improved detection of small masses and compressive vascular lesions (ie, aneurysm, vessel tortuosity) in and around the IACs.

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The addition of gadolinium-based IV contrast agents also improves the detection and characterization of masses as well as inflammatory, infectious, or demyelinating processes, which have been described in the setting of CPPV in a retrospective study of 500 patients [21]. MRI Head and Internal Auditory Canal Without IV Contrast There is no relevant literature regarding the use of MRI head and IAC without IV contrast in the evaluation of t- EVS. MRI Head With IV Contrast There is no relevant literature regarding the use of MRI head with IV contrast in the evaluation of t-EVS. MRI Head Without and With IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRI allows detailed evaluation of the intracranial structures with improved soft tissue resolution when compared with CT. The addition of MRI of the brain following the IV administration of gadolinium-based contrast allows further characterization of any detected lesions and improves the detection and characterization of masses as well as inflammatory, infectious, or demyelinating processes, which have been described in the setting of CPPV [20]. Acute brain lesions have been reported in 6% of patients undergoing head CT and 11% of those undergoing MRI for CPPV [6]. MRI Head Without IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRI allows detailed evaluation of the intracranial structures with improved soft tissue resolution when compared with CT. MRI of the head is not indicated for the initial diagnosis of typical BPPV. The presence of coexisting brain atrophy on MRI, however, is associated with a higher risk of prolonged dizziness following a diagnosis of BPPV and so may be of prognostic value in these patients in a retrospective study of 120 patients [24]. Variant 2: Adult. Acute persistent vertigo. Normal neurologic examination and HINTS examination is consistent with peripheral vertigo. Initial imaging.

Dizziness and Ataxia. The addition of gadolinium-based IV contrast agents also improves the detection and characterization of masses as well as inflammatory, infectious, or demyelinating processes, which have been described in the setting of CPPV in a retrospective study of 500 patients [21]. MRI Head and Internal Auditory Canal Without IV Contrast There is no relevant literature regarding the use of MRI head and IAC without IV contrast in the evaluation of t- EVS. MRI Head With IV Contrast There is no relevant literature regarding the use of MRI head with IV contrast in the evaluation of t-EVS. MRI Head Without and With IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRI allows detailed evaluation of the intracranial structures with improved soft tissue resolution when compared with CT. The addition of MRI of the brain following the IV administration of gadolinium-based contrast allows further characterization of any detected lesions and improves the detection and characterization of masses as well as inflammatory, infectious, or demyelinating processes, which have been described in the setting of CPPV [20]. Acute brain lesions have been reported in 6% of patients undergoing head CT and 11% of those undergoing MRI for CPPV [6]. MRI Head Without IV Contrast Imaging evaluation in BPPV with typical nystagmus on Dix-Hallpike testing is unnecessary. MRI allows detailed evaluation of the intracranial structures with improved soft tissue resolution when compared with CT. MRI of the head is not indicated for the initial diagnosis of typical BPPV. The presence of coexisting brain atrophy on MRI, however, is associated with a higher risk of prolonged dizziness following a diagnosis of BPPV and so may be of prognostic value in these patients in a retrospective study of 120 patients [24]. Variant 2: Adult. Acute persistent vertigo. Normal neurologic examination and HINTS examination is consistent with peripheral vertigo. Initial imaging.

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The acute onset of persistent vertigo with associated nausea/vomiting, gait instability, nystagmus, and head-motion intolerance defines AVS. These patients may or may not have associated deficits on neurologic examination [1]. This variant will address the imaging approach to those presenting with AVS but a normal neurologic examination (isolated AVS). The approach to those with associated neurologic deficits will be discussed as a separate variant (Variant 3). Patients with vertigo of any cause are often initially evaluated in the emergency department. Although the use of CT imaging performed for this indication has dramatically increased over the past several decades, the detection rate of contributory central nervous system pathology in those with a normal neurologic examination remains very low (<1% in an emergency department in a single center, which included 3,165 patients); see Variant 8 [25]. This is not surprising because many patients with acute vertigo and a normal neurologic examination will have a benign peripheral etiology. In patients with AVS and a normal neurologic examination, the most common causes of symptoms are vestibular neuritis and labyrinthitis, neither of which has associated findings on CT imaging. The most worrisome cause of AVS to be excluded is a posterior circulation infarct involving the brainstem or cerebellum; see Variant 3. Although the prevalence of cerebrovascular disease in all patients presenting to the emergency department with dizziness or vertigo is approximately 4%, the prevalence of cerebrovascular disease in those presenting with AVS is closer to 25% and may be as high as 75% in the highest vascular risk cohorts [26]. Depending on the skill and training of the examiner, focal neurologic symptoms/signs are reportedly lacking in between one-third and two-thirds of these patients [27].

Dizziness and Ataxia. The acute onset of persistent vertigo with associated nausea/vomiting, gait instability, nystagmus, and head-motion intolerance defines AVS. These patients may or may not have associated deficits on neurologic examination [1]. This variant will address the imaging approach to those presenting with AVS but a normal neurologic examination (isolated AVS). The approach to those with associated neurologic deficits will be discussed as a separate variant (Variant 3). Patients with vertigo of any cause are often initially evaluated in the emergency department. Although the use of CT imaging performed for this indication has dramatically increased over the past several decades, the detection rate of contributory central nervous system pathology in those with a normal neurologic examination remains very low (<1% in an emergency department in a single center, which included 3,165 patients); see Variant 8 [25]. This is not surprising because many patients with acute vertigo and a normal neurologic examination will have a benign peripheral etiology. In patients with AVS and a normal neurologic examination, the most common causes of symptoms are vestibular neuritis and labyrinthitis, neither of which has associated findings on CT imaging. The most worrisome cause of AVS to be excluded is a posterior circulation infarct involving the brainstem or cerebellum; see Variant 3. Although the prevalence of cerebrovascular disease in all patients presenting to the emergency department with dizziness or vertigo is approximately 4%, the prevalence of cerebrovascular disease in those presenting with AVS is closer to 25% and may be as high as 75% in the highest vascular risk cohorts [26]. Depending on the skill and training of the examiner, focal neurologic symptoms/signs are reportedly lacking in between one-third and two-thirds of these patients [27].

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In one study, 11% of patients presenting with acute persistent vertigo but no focal neurologic symptoms/signs were found to have an acute infarct on CT or MRI of the brain [28]. Other studies estimate that 75% to 80% of patients presenting with AVS related to infarct have no associated focal neurologic deficits [29]. Isolated AVS is most commonly due to a benign peripheral cause; however, a lack of associated neurologic deficits does not exclude a central cause such as infarct. To address this, various bedside tests like the HINTS examination Dizziness and Ataxia have been developed to distinguish AVS of benign cause from posterior circulation infarct. When performed by specially trained practitioners, these tests have been shown to be even more sensitive than early MRI for the detection of infarct (100% versus 46%) [30]. Similar testing performed by nonexperts has shown more mixed results [6,31]. In another retrospective study involving 610 patients in an emergency department, not a single patient with an AVS, in whom the complete HINTS triad was consistent with peripheral vertigo, had abnormalities on CT/MRI [6]. Multiple sclerosis involving the brainstem or cerebellar peduncles is an additional but rare cause of AVS, accounting for approximately 4% of cases. Of note, nearly all of these patients also have additional abnormal neurologic findings suggesting a central lesion [32]. Even rarer causes of isolated AVS include cerebellar hemorrhage and a variety of autoimmune, infectious, and metabolic conditions [25]. Vestibular migraine may mimic a variety of causes of dizziness/vertigo, including AVS, but often has associated headache and other migrainous features (ie, photophobia, phonophobia, visual aura) [22,33]. Imaging is not required to diagnose vestibular migraine. To summarize, patients with isolated AVS may not require imaging if HINTS examination by specially trained providers is available and negative; otherwise, imaging may be required to rule out stroke.

Dizziness and Ataxia. In one study, 11% of patients presenting with acute persistent vertigo but no focal neurologic symptoms/signs were found to have an acute infarct on CT or MRI of the brain [28]. Other studies estimate that 75% to 80% of patients presenting with AVS related to infarct have no associated focal neurologic deficits [29]. Isolated AVS is most commonly due to a benign peripheral cause; however, a lack of associated neurologic deficits does not exclude a central cause such as infarct. To address this, various bedside tests like the HINTS examination Dizziness and Ataxia have been developed to distinguish AVS of benign cause from posterior circulation infarct. When performed by specially trained practitioners, these tests have been shown to be even more sensitive than early MRI for the detection of infarct (100% versus 46%) [30]. Similar testing performed by nonexperts has shown more mixed results [6,31]. In another retrospective study involving 610 patients in an emergency department, not a single patient with an AVS, in whom the complete HINTS triad was consistent with peripheral vertigo, had abnormalities on CT/MRI [6]. Multiple sclerosis involving the brainstem or cerebellar peduncles is an additional but rare cause of AVS, accounting for approximately 4% of cases. Of note, nearly all of these patients also have additional abnormal neurologic findings suggesting a central lesion [32]. Even rarer causes of isolated AVS include cerebellar hemorrhage and a variety of autoimmune, infectious, and metabolic conditions [25]. Vestibular migraine may mimic a variety of causes of dizziness/vertigo, including AVS, but often has associated headache and other migrainous features (ie, photophobia, phonophobia, visual aura) [22,33]. Imaging is not required to diagnose vestibular migraine. To summarize, patients with isolated AVS may not require imaging if HINTS examination by specially trained providers is available and negative; otherwise, imaging may be required to rule out stroke.

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