Diagnosis and treatment of vein of Galen aneurysmal malformations.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAM) are rare but clinically significant intracranial arteriovenous shunt lesions that most often present in neonates and infants.

METHODS: Retrospective clinical data were collected for patients evaluated with a diagnosis of VGAM from 1994 to 2007.

RESULTS: Thirteen patients with VGAM were evaluated from 1994 to 2007. Seven patients presented emergently with medically intractable cardiac failure, and six were treated in the first 2 weeks of life. Five children treated after this period (1.5-31 months of age) manifested enlarging head circumference, abnormal development, or subarachnoid hemorrhage. Eleven patients were managed endovascularly. Four disease or procedure-related complications occurred. Two complications were associated with poor outcome, both of which occurred in patients treated at less than 2 weeks of age. Two other patients experienced transient neurological deficits with no evidence of permanent sequelae. Outcome in the six patients treated emergently in the first 2 weeks of life included two patients who developed normally, one with mild to moderate neurological deficits, one with severe neurological deficits, and two deaths. Outcome in the five older patients (treated between 1.5 and 31 months) was considerably better than in the group treated early and included three with normal outcome and two with mild neurological deficits.

CONCLUSIONS: Contemporary endovascular techniques remain the preferred treatment for VGAM in all age groups. Early diagnosis and multimodality treatment are essential for the best management and treatment of the complex constellation of clinical problems often arising from this disorder.