Clinically Suspected Vascular Malformation of the Extremities. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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.


Clinically Suspected Vascular Malformation of the Extremities. 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].


Clinically Suspected Vascular Malformation of the Extremities. Vascular Malformation of the Extremities


Clinically Suspected 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. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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]. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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].


Clinically Suspected Vascular Malformation of the Extremities. 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


Clinically Suspected Vascular Malformation of the Extremities. Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and rating round tabulations for each recommendation.


Clinically Suspected Vascular Malformation of the Extremities. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Headache Child PCAs

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].


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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]. 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.


Headache Child PCAs

Headache Child PCAs. 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


Headache Child PCAs

Headache Child PCAs. Reprint requests to: publications@acr.org


Headache Child PCAs

Headache Child PCAs. 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].


Headache Child PCAs

Headache Child PCAs. 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. 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].


Headache Child PCAs

Headache Child PCAs. 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].


Headache Child PCAs

Headache Child PCAs. 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


Headache Child PCAs

radiation concerns.

Headache Child PCAs. radiation concerns.


Headache Child PCAs

Headache Child PCAs. 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. If there is strong concern for arterial dissection within the head and/or neck, the diagnosis is generally made by MRI or MRA [26].


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. Discussion of Procedures by Variant Variant 1: Child. Primary headache. Initial imaging. Radiography There is no role for radiography in patients with primary headache.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. CTA There is no role for CTA in patients with primary headache and no concerning findings on clinical or physical examination.


Headache Child PCAs

Headache Child PCAs. CTV There is no role for CTV in patients with primary headache and no concerning findings on clinical or physical examination.


Headache Child PCAs

Headache Child PCAs. MRA There is no role for MRA in patients with primary headache and no concerning findings on clinical or physical examination.


Headache Child PCAs

Headache Child PCAs. MRV There is no role for MRV in patients with primary headache and no concerning findings on clinical or physical examination. The use of contrast in MRV depends on institutional preferences.


Headache Child PCAs

Headache Child PCAs. Arteriography There is no role for arteriography in patients with primary headache and no concerning findings on clinical or physical examination.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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. 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].


Headache Child PCAs

Headache Child PCAs. 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


Headache Child PCAs

Headache Child PCAs. 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].


Headache Child PCAs

Headache Child PCAs. 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. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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]. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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

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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. CTV Except in cases of thunderclap headache related to an AVM, CTV is usually not indicated in patients with thunderclap headache.


Headache Child PCAs

Headache Child PCAs. 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

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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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. 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].


Headache Child PCAs

Headache Child PCAs. 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. 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.


Headache Child PCAs

Headache Child PCAs. 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


Headache Child PCAs

Headache Child PCAs. 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].


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. MRA The role for MRA is limited in children with headache attributed to infection unless SAH or stroke is suspected. If arteritis is suspected, as can be seen in the setting of sphenoid sinusitis and skull base osteomyelitis, MRA may be helpful.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. Arteriography There is usually no role for arteriography in the evaluation of children with headache related to infection.


Headache Child PCAs

Headache Child PCAs. 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.


Headache Child PCAs

Headache Child PCAs. CT CT is usually not indicated in children with headaches attributed to remote trauma. A retrospective study by


Headache Child PCAs

Headache Child PCAs. Dayan et al [55] identified 2,462 children who had minor blunt head trauma and headaches as their only symptom. None of these children had clinically important traumatic brain injuries, and only 0.7% had a traumatic brain injury identified on CT scan of the head. CT can be used if there are concerning symptoms and MRI is not possible.


Headache Child PCAs

Headache Child PCAs. CTA Unless there is concern for a post-traumatic arterial abnormality, such as an aneurysm or arteriovenous fistula detected by CT or MRI, CTA is usually not indicated in these patients.


Headache Child PCAs

Headache Child PCAs. CTV CTV is usually not indicated in children with headache secondary to remote trauma.


Headache Child PCAs

Headache Child PCAs. MRA Unless there is concern for a post-traumatic arterial abnormality such as an aneurysm or arteriovenous fistula detected by CT or MRI, MRA is usually not indicated in these patients. MRA is preferred over CTA.


Headache Child PCAs

Headache Child PCAs. MRV MRV is usually not indicated in children with headache secondary to remote trauma. The use of contrast in MRV depends on institutional preferences.


Headache Child PCAs

Headache Child PCAs. Arteriography Arteriography is usually not indicated in children with headache secondary to remote trauma.


Headache Child PCAs

Headache Child PCAs. Summary of Recommendations For the initial imaging of primary headache in children, imaging is usually not appropriate. For the initial imaging of secondary headache in children, MRI head without IV contrast is usually appropriate, and postcontrast imaging is indicated if the noncontrast study is abnormal. For the initial imaging of sudden severe headache (thunderclap headache) in children, either noncontrast CT head or MRI brain is usually appropriate. Noncontrast MRA head is also usually appropriate. For the initial imaging of headache attributed to infection in children, MRI head with and without IV contrast is usually appropriate. For the initial imaging of headache attributed to remote trauma in children, MRI head without contrast is usually appropriate.


Headache Child PCAs

Headache Child PCAs. Although there are references that report on studies with design limitations, 3 well-designed or good-quality studies provide good evidence.


Headache Child PCAs

Headache Child PCAs. Supporting Documents For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Female Infertility

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.


Female Infertility

Female 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.


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. Endometriosis affects at least one-third of women with infertility and up to 10% of reproductive-aged women [7]. 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].


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. Reprint requests to: publications@acr.org


Female Infertility

Female Infertility

Female Infertility. Female Infertility


Female Infertility

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].


Female Infertility

Female Infertility. 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].


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. US Pelvis Transvaginal TVUS can be used to monitor follicle development [27], perform antral follicle counts [28], and measure ovarian volume. When the ovarian volume is <3 cm3 and <5 antral follicles are present, this suggests diminished ovarian reserve [29].


Female Infertility

Female Infertility. 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].


Female Infertility

Female Infertility. 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

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.


Female Infertility

Female Infertility. 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

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].


Female Infertility

Female Infertility. 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

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.


Female Infertility

Female Infertility. 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

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. 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].


Female Infertility

Female Infertility. 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].


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. 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

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.


Female Infertility

Female Infertility. 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]. 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

Female Infertility

Female Infertility. Female Infertility


Female Infertility

Female Infertility. with laparoscopy with chromopertubation [58], which is widely accepted as the reference standard for evaluating tubal patency.


Female Infertility

Female Infertility. 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].


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. 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

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].


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. 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]. 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

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]. When comparing diagnostic modalities to hysterectomy for the detection of intracavitary abnormalities, MRI, SIS, and hysteroscopy were equally effective and superior to TVUS [76].


Female Infertility

Female Infertility. 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.


Female Infertility

Female Infertility. 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].


Female Infertility

Female Infertility. 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].


Female Infertility

Female Infertility. 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]. 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

Female Infertility. In a study of 140 women who underwent hysteroscopy during infertility evaluation, all had an HSG and 93 also underwent SIS. Compared with hysteroscopy, HSG was 52.6% accurate and SIS was 75% accurate in detecting fibroids [85]. A systematic review of studies involving women with abnormal uterine bleeding demonstrated hysteroscopy and SIS to be better than TVUS in detecting submucosal fibroids [86]. When comparing diagnostic modalities with hysteroscopy for the detection of intracavitary abnormalities, MRI, SIS, and hysteroscopy were equally effective and superior to TVUS [76]. In a study comparing HSG, TVUS, and SIS with hysteroscopy, SIS


Female Infertility

Female Infertility. demonstrated 75% sensitivity and 93% specificity in detecting intrauterine adhesions. This was similar to HSG but far superior to TVUS [71].


Female Infertility

Female Infertility. Three-dimensional SIS was shown to be 100% accurate in classification of anomalies in women with bicornuate, septate, and arcuate uteri [83] when compared with hysteroscopy, although this was not statistically different from the performance of 3-D TVUS or 2-D SIS. Another study demonstrated 3-D SIS had similar accuracy to hysteroscopy in detected intracavitary polyps, leiomyomas, adhesions, and uterine septum [87].


Female Infertility

Female Infertility. Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.


Female Infertility

Female Infertility. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Female Infertility

Female Infertility

Female Infertility. Female Infertility


Staging and Follow up of Vulvar Cancer

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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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]. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. Reprint requests to: publications@acr.org


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

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

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. CT Chest, Abdomen, and Pelvis CT of the chest, 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 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

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%.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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]. If SLN is involved by tumor, management options include bilateral lymphadenectomy or external beam radiation therapy (EBRT) with or without chemotherapy [6].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up 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 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.


Staging and Follow up of Vulvar Cancer

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 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

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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. 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

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 it was 80%, 100%, 93%, and 100%, respectively.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

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. 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%.


Staging and Follow up of Vulvar Cancer

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.


Staging and Follow up of Vulvar Cancer

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


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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%.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

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 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

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.


Staging and Follow up of Vulvar Cancer

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.


Staging and Follow up of Vulvar Cancer

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. 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

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

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.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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].


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. 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

Staging and Follow up of Vulvar Cancer. US Duplex Doppler Groin There is no relevant literature to support or refute the use of US with Doppler for the assessment of suspected vulvar cancer recurrence.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. US-guided Fine-Needle Aspiration Biopsy Groin There is no relevant literature to support or refute the use of US-guided FNAB for the assessment of suspected vulvar cancer recurrence.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.


Staging and Follow up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Staging and Follow up of Vulvar Cancer

Staging and Follow-up of Vulvar Cancer

Staging and Follow up of Vulvar Cancer. Staging and Follow-up of Vulvar Cancer