Indian Pediatrics 2002; 39:307-308  

An Uncommon Variety of Vein of Galen Malformation

A 10-year-old boy presented with pro-gressively increasing soft, bluish, compressible facial swelling over the nasal bridge and adjoining areas of the face since the age of one year. There had been history of significant bleeding (500 ml) from tortuous veins over the face along with nasal and oral bleeds. Rest of the history was non-contributory. On examination dilated tortuous veins were present over the face with areas of bluish discoloration (Fig. 1). Bruit was audible over these areas and venous hum was present bilaterally in the neck. Systemic examination revealed no abnormality except glaucoma in both eyes.

Investigations revealed hemoglobin 7.5 g/dl with a microcytic hypochromic picture. Coagulation and bleeding dysfunction screen was negative and echocardiography was normal. Contrast enhanced CT of the brain revealed a hyperdense expanded linear opacity in the falx cerebri suggestive of large vein of Galen aneurysm (Fig. 2).

Malformations involving the vein of Galen are rare congenital connections occurring between intracranial arteries (usually thalamo-perforator, choroidal and anterior cerebral arteries) and the vein of Galen or other primitive midline vein. A strong association with venous anomalies and certain cardio-vascular ano-malies, most commonly aortic coarctation, and secundum atrial septal defects has been noted.

Over 90% of the vein of Galen varices fall into choroidal malformations group which are arteriovenous connections in the anterior wall of the prosencephalic vein supplied by a plethora of vessels. They have significant arteriovenous shunting and present as congestive heart failure in neonates. Prenatal diagnosis is possible. They have the poorest prognosis and are usually fatal without treatment.

The second, less common category of vein of Galen malformation is the "mural" malforma-tion which is characterized by fewer (usually one to four) but larger calibre connections with the prosencephalic vein. Patients usually present in infancy with developmental delay, hydro-cephalus and seizures but mild or no signs of congestive heart failure or present in an older child or young adult with hemorrhage as in the case reported.

On neuroimaging, vein of Galen malforma-tions appear as large masses in the posterior incisural region, sometimes extending rostrally and anteriorly displacing the third ventricle. Endovascular therapy has become the method of choice for both and offers a high rate of cure with low morbidity.

Sheffali Gulati,
Veena Kalra,
Department of Pediatrics,
All India Institute of Medical Sciences,
New Delhi 110 029, India.
E-mail: [email protected]