Workup
Laboratory Studies
The workup of vascular anomalies relies heavily on history, physical examination, and overall clinical assessment. Typically, laboratory studies are not required in the workup and diagnosis of these anomalies. However, in patients with multiple venous malformations, Kasabach-Merritt syndrome, and combined malformations, coagulation studies are warranted because coagulopathies are often present.
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Imaging Studies
MRI is the leading imaging modality in the diagnosis and follow-up of patients with vascular malformations. Arteriography is the criterion standard for the evaluation of high-flow vascular anomalies, particularly for arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs). [2]
Hemangiomas
Most hemangiomas can be managed without imaging studies. However, magnetic resonance imaging (MRI) is warranted in the following circumstances:
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Lesions consistent with PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, cardiac defects, and eye anomalies) should undergo imaging to evaluate the carotids and cerebral vasculature
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The presence of multiple cutaneous lesions warrants screening with ultrasonography (US) or MRI to assess concomitant visceral lesions
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Lumbosacral lesions require imaging of the spinal cord (US or MRI) to rule out synchronous cord lesions
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Preoperative imaging may be required at the discretion of the surgeon
Venous malformations
Magnetic resonance angiography (MRA) or computed tomography (CT) angiography (CTA) may be required to delineate the full extent of complex venous malformations. Such information may be useful for assisting in treatment and operative management.
Capillary malformations
Imaging of the spinal cord should be considered in the presence of capillary malformations; developmental defects of the central neural axis are common with these lesions.
Lymphatic malformations
Large lymphatic malformations may be diagnosed in utero with US. [37] Such malformations are classified according to their radiographic and histologic characteristics. Hence, multimodality imaging is often used for proper delineation. MRI and Doppler US yield insight into the extent and flow characteristics, respectively.
Arteriovenous malformations
US with Doppler imaging is a very useful tool to confirm the diagnosis of suspected AVM. Further imaging with MRI can delineate the full extent of the lesion and involvement of other structures. Angiography may also be useful to aid in embolization and preoperative planning. Hardwicke et al described a case in which office-based thermography was used adjunctively in the assessment of an AVM of the hand. [48]
Previous
Media Gallery
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Disfiguring nasal hemangioma at proliferating phase on 2-year-old girl.
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Deep and superficial hemangioma of parotid region in 4-month-old girl.
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Clinical image of 9-year-old boy with axillary mixed malformation. Note increased volume, mostly due to lymphatic malformation, venous ectasia seen as cutaneous bluish varicose-looking venous malformation, and reddish port-wine stain due to capillary malformation.
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Preoperative image of 2-year-old boy who underwent plastification of right-thoracic-wall arteriovenous malformation.
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Surgical removal of AVM of same child described in previous image. Note how malformation can be resected en bloc without bleeding due to previous plastification.
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Clinical image of 10-year-old boy with CLOVES syndrome (congenital lipomatous overgrowth syndrome with vascular anomalies, epidermal nevi, and scoliosis). Severe overgrowth on thorax, abdomen, and extremities, with multiple lipomas.
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Clinical image of previously mentioned patient with CLOVES syndrome, from back. Scoliosis and severe left buttock enlargement are evident.
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Closer clinical image of same patient with CLOVES syndrome, where lymphatic cutaneous infiltrate is seen as red confluent macules on right hemiabdomen.
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Clinical image of 2-month-old boy with large hamartoma of right clavicular-axillary-thoracic region due to Proteus syndrome.
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Surgical image of aforementioned patient with Proteus syndrome after removal of large axillary hamartoma.
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Postoperative appearance of same patient with Proteus syndrome after soft-tissue reconstruction.
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Patient who developed skin necrosis as a complication of sclerosis of a nontarget organ after endovascular injection of a sclerosing agent for a venous malformation.
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Clinical image of 9-year-old girl presenting with cutaneous ulcer on right buttock as complication after endovascular sclerotherapy for venous malformation. Sclerosis of nontarget organ.
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Newborn with massive macrocystic lymphatic malformation of left neck.
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Preoperative image of 1-year-old boy with forehead hemangioma who required surgical resection because of previous episodes of massive bleeding after trauma. Initially, tissue expander was placed under scalp and progressively distended with water to achieve enough native tissue for defect coverage. Lesion was plastified before resection to reduce risk of operative bleeding.
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Postoperative image of previously mentioned patient with forehead hemangioma after surgical removal of hemangioma. Primary closure was achieved satisfactorily after extraction of tissue expander. Since dissection had been considerable, drain was left in place to avoid postoperative fluid collection, avoiding risk of palpebral and facial edema.
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Clinical appearance of same patient with hemangioma at follow-up, aged 5 years.
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Clinical image of 2-month-old boy with diffuse hemangiomatosis who presented with multiple cutaneous as well as hepatic hemangiomas.
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Hemangioma of arm in aforementioned patient with diffuse hemangiomatosis.
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Clinical image of right hemifacial mixed (venous and lymphatic) malformation in 7-year-old girl.
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Clinical image of male newborn with Klippel-Trenaunay syndrome. Note left leg hypertrophy, with evident cutaneous venous lesions.
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Clinical image of 7-year-old girl with Klippel-Trenaunay syndrome. Note left leg hypertrophy, with evident cutaneous venous lesions.
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Clinical image of 3-year-old boy who underwent percutaneous sclerotherapy with absolute alcohol for left-cheek venous malformation. Patient had very severe reaction to sclerosing agent and developed cutaneous lesion that scarred and retracted over time.
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Clinical image of 14-year-old girl with Maffucci syndrome, comprising diffuse venous malformations and enchondromas. Venous malformations are readily visible on lips.
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Clinical image of aforementioned patient with Maffucci syndrome demonstrating multiple enchondromas of wrist and fingers of left hand, combined with vascular malformations on distal phalanx of fifth finger.
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Postoperative image of same patient with Maffucci syndrome after surgical resection of three venous malformations of lips.
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Clinical picture of same patient with Maffucci syndrome with diffuse venous malformations on left foot.
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CT angiogram of patient with Klippel-Trenaunay syndrome demonstrating pathognomonic vein of Servelle. Hypoplasia of deep venous system of lower extremities causes dilatation of saphenous vein.
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3D CT reconstruction of lower extremities in a patient with Klippel-Trenaunay syndrome. Hypoplasia of deep venous system of leg with subsequent tortuous dilatation of superficial venous system is evident.
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Surgical photograph during resection of axillary macrocystic lymphatic malformation that did not respond to sclerotherapy. Topographic anatomy of lesion required simultaneous two-sided (cervical and axillary) approach. Extreme care during axillary neurovascular structures is paramount for adequate postoperative functional outcome.
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Postoperative image of aforementioned patient with axillary macrocystic lymphatic malformation.
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Coronal CT image of abdomen in 2-year-old patient with intestinal macrocystic lymphatic malformation. Surgical treatment was chosen on basis of difficult percutaneous image-guided approach and high risk of complications (intestinal ischemia/perforation).
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Surgical specimen of previously mentioned patient with intestinal macrocystic lymphatic malformation that required intestinal resection and primary anastomosis.
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Clinical image of 3-year-old girl with large macrocystic lymphatic malformation on left neck.
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Clinical image of newborn with large macrocystic lymphatic malformation and acute inflammation after upper respiratory tract infection.
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Clinical image of 2-day-old male with massive mixed lymphatic malformation on left neck. Surgical treatment was chosen because of risk of airway compromise.
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Sagittal T2-weighted MRI image of previously mentioned 3-year-old girl with large macrocystic lymphatic malformation on left neck.
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Hemihypertrophy of right lower extremity on 2-month-old boy.
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Severe mixed vascular malformation of right leg on 5-day-old boy, with massive overgrowth, port-wine stains, and diffuse venolymphatic malformation.
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Newborn boy with Parks-Weber syndrome. Note diffuse left-lower-leg hypertrophy with larger overgrowth; lower abdominal and diffuse leg capillary malformation; tortuous venous dilatation of superficial system on left leg; and skin ischemia with necrosis and bleeding due to arteriovenous malformation.
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Newborn boy with Parks-Weber syndrome. Note severe overgrowth of right leg, with massive and diffuse mixed malformations. Port-wine stains represent capillary malformations, while arterial blood sequestration from arteriovenous malformation creates skin ischemia and necrosis.
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Clinical picture of right hand of 5-day-old boy with Parks-Weber syndrome. Note hypertrophy of fourth finger and port-wine stain (capillary malformation) on hand distal to crease.
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Clinical picture of 5-year-old boy with arteriovenous vascular malformation of tongue. He underwent attempted surgical correction at local hospital, where surgeon attemtped to ligate primary inflow artery but instead ligated venous drainage vessel, provoking sudden and intense outflow obstruction with rapid lesion enlargement, tongue protrusion, and mucosal ischemia.
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Preoperative image of aforementioned patient with arteriovenous vascular malformation of tongue, who initially received tracheostomy for airway control and feeding gastrostomy. Note severe tongue swelling and enlargement and poor mucosal perfusion with ischemia and necrosis.
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Selective lingual artery angiography of aforementioned patient with arteriovenous vascular malformation of tongue demonstrates large, diffuse lingual arteriovenous malformation occupying entire tongue. At hemodynamics suite, with patient under general anesthesia, catheter was introduced through femoral artery and into external carotid artery, and micro-catheter was then passed through former into lingual artery. Under fluoroscopy with image substraction and contrast roadmapping, supraselective lingual artery branches were embolized.
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Preoperative postembolization radiologic image of aforementioned patient with arteriovenous vascular malformation of tongue demonstrates important reduction of blood flow to malformation.
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3D color reconstruction CT angiography depicts upper-lip venous malformation.
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3D color reconstruction CT angiography depicts left-lower-extremity arteriovenous malformation.
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Operative image of 6-year-old boy during postplastification glossectomy for lingual venous malformation.
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Postoperative image after partial anterior glossectomy on previously mentioned 6-year-old boy.
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Preoperative image of previously mentioned 6-year-old boy with marking of anterior partial glossectomy after plastification for treatment of lingual venous malformation.
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Postoperative image after anterior two thirds glossectomy after embolization of lingual arteriovenous malformation.
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Surgical specimen from anterior partial glossectomy after plastification for lingual venous malformation.
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