Vascular malformation in the region of the great vein of Galen.

Abstract

T HIS report concerns arteriovenous malformations in the region of the pineal gland. The 5 cases reported here and similar cases reported in the literature have several factors in common. All are of congenital origin, are arteriovenous shunts with venous drainage into the great vein of Galen, and appeared to be amenable to surgery. They often are associated with hydrocephalus. In ~ of the 5 cases the communication was between the anterior cerebral artery group and the great vein of Galen, in ~ it was between the posterior cerebral artery group of vessels and the great vein of Galen, and in 1 it was between the superior cercbellar artery and the great vein of Galen. In each of the cases the diagnosis was established by angiography. When therapy was instituted it was ligation of the vessels on the arterial side and in these instances postoperative angiography revealed failure of filling of the vascular malformations. The first report found in the literature of arteriovenous malformations leading into the great vein of Galen was by Jaeger, Forbes and Dandy 7 in 1937. They described a communication between the circle of Willis and the great vein of Galen in a 4-year-old child whose symptoms began at approximately the age of 8 months. The child had an internal hydrocephalus. This they at t r ibuted to the greatly dilated great vein of Galen pressing on the aqueduct. Because the pat ient had no history of trauma, they concluded the aneurysm was congenital. In 1940 Russell and i e v i n 11 reported ~ cases in children. In one the aneurysm was a complex sacculated structure communicating between the left posterior cerebral artery and the great vein of Galen. There was an associated absence of the sigmoid portions of both lateral sinuses. In their second case the arteries coming over the corpus callosum connected with the great vein of Galen. In both cases there was an internal hydrocephalus. In 1945 A]pers and Forster I reported on an 18-year-old boy with bilateral hydrocephalus secondary to a calcified arteriovenous malformation in the pineal region. The arterial supply came through the posterior cerebral or the choroidal arteries and the venous drainage was through the great vein of Galen. Alpers and Forster doubted that involvement of the great cerebral vein is capable of producing internal hydrocephalus. They mentioned the investigations of Bedford 3,4 indicating that occlusion of this vein in the dog is incapable of producing internal hydrocephalus despite scattered re-