Klippel-Trenaunay-Weber Syndrome Associated with Multiple Cerebral Arteriovenous Malformations: Usefulness of Gamma Knife Stereotactic Radiosurgery in This Syndrome.

Abstract

Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by the presence of a combined vascular malformation of capillaries, veins, and lymphatic vessels; congenital venous abnormalities; and limb hypertrophy. Its association with neurovascular abnormalities is infrequent, and the presence of intracranial arteriovenous malformations (AVMs) is extremely rare. We report a case of a 48-year-old male diagnosed with KTWS who spontaneously presented with a cerebral hemorrhage. Computed tomography scan and angio-computed tomography studies revealed bleeding associated with AVM rupture. In the conventional arteriography study, 10 small (<1 cm) AVMs were observed. The patient presented a good clinical recovery. These multiple small lesions were not considered susceptible to surgical or endovascular treatments. Therefore all lesions were treated with Gamma Knife stereotactic radiosurgery since it attains the highest dose drop and minimal irradiation of the healthy parenchyma. One year after the treatment, the lesions have shrunk. Cerebral AVMs are extremely rare in KTWS cases; however, their presence can have serious consequences if they are treated. We find it advisable to include brain imaging tests, such as nuclear magnetic resonance imaging, to diagnose and monitor KTWS. Furthermore, a Gamma Knife may be useful when multiple AVMs are present.