Management of Arteriovenous Malformations

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Clinical Features

The most common site of extracranial AVM is the head and neck, followed by the limbs, trunk, and viscera.8 Although present at birth, AVM may not become evident until childhood. Lesions have a pink-red cutaneous stain, are warm, have a palpable thrill or bruit, and may be initially mistaken for a capillary malformation or hemangioma. Arteriovenous shunting reduces capillary oxygen delivery causing ischemia; patients are at risk for pain, ulceration, bleeding, and congestive heart failure. AVM

Clinical Features

The prevalence of capillary malformation–arteriovenous malformation (CM-AVM) is estimated to be 1 in 100,000 Caucasians.6, 50 Patients have atypical capillary malformations (CMs) that are small, multifocal, round, pinkish-red, and surrounded by a pale halo (50%).6, 50 An individual may have as many as 53 CMs, ranging in size from 1 to 15 cm in diameter, although 6% of patients have only a solitary lesion.50 Thirty percent of individuals also have an AVM: Parkes-Weber syndrome (PWS) (12%),

Clinical Features

The PTEN (phosphatase and tensin homolog) gene encodes a tumor suppressor lipid phosphatase involved in the phosphoinositide-3 kinase pathway; it mediates cell-cycle arrest and apoptosis.54 Patients with PTEN mutations have PTEN hamartoma-tumor syndrome (PHTS). This autosomal dominant condition had previously been referred to as Cowden syndrome or Bannayan-Riley-Ruvalcaba syndrome (BRRS).7, 55 Males and females are equally affected, and approximately one-half (54%) of patients have a unique

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