Pediatric extracranial arteriovenous malformations.

PURPOSE OF REVIEW: Arteriovenous malformations (AVMs) are congenital vascular anomalies composed of a 'nidus' of inappropriately connected arteries and veins with an unlimited growth potential. This review aims to explore new information on the natural history, pathophysiology, and treatment for pediatric extracranial AVMs.

RECENT FINDINGS: AVMs are frequently dormant during childhood with only subtle signs of disease. Expansion occurs, on average, during early adolescence, during which hormonal changes are thought to play a role. AVMs have a site predilection for the midface, oral cavity, and limbs. Relentless disease progression with local invasion is the rule. Focal or diffuse disease may be present, with the latter being more difficult to treat. Location and depth of disease affect patient outcomes. Genetic and molecular factors involved in vascular stabilization are aberrant in AVMs. Management techniques include embolization and surgical resection, separately or in combination. Expectant observation is no longer recommended. Pharmacotherapy is currently not an option. Early intervention with supraselective embolization followed by surgical extirpation has the best chance of disease control. Unfortunately, disease margins are difficult to identify, and recurrence rates are high (93%).

SUMMARY: Extracranial AVMs are a dangerous group of vascular birthmarks. Lack of research into AVMs has curtailed development of novel treatment options. Surgical excision and intravascular embolization are effective tools offering control but not necessarily a cure.