Congenital Arteriovenous Fistula With an Aneurysm of the Great Cerebral Vein and Hydrocephalus Treated Surgically

Abstract

Aneurysms occasionally develop from the great cerebral vein as a result of congenital arteriovenous fistulae. In a recent review Hirano and Terry (1958) referred to 18 cases, nine of which were confirmed at necropsy. The example of this rare condition reported here presented as a case of hydrocephalus showing certain noteworthy features. The condition responded well to surgical treatment, but the patient died after a fall. The connexions of the aneurysm were defined at necropsy. Case ReportA baby girl of 6 months was brought for neurosurgical consultation because of obvious hydrocephalus in February, 1954. The pregnancy and delivery and the early development of the infant had been normal, and five weeks before she was examined she had been able to sit up almost unaided. At the time her balance had become impaired and she no longer raised her head spontaneously. The skull was seen to be expanded and superficial scalp veins were prominent. The skull circumference was 515 cm.; the fontanelle was half closed but tense. Eye movements and pupillary reactions were normal; no other abnormalities were detected. In radiographs of the skull, opening of sutures and finger markings of the vault were seen but there was no evidence of the cause of the increased pressure. The transverse sinuses were in their normal position and the posterior fossa was not unduly shallow. At ventriculography, the intracranial pressure was high and an obstruction of the aqueduct was demonstrated, but not with the flaskshaped outline usually found in aqueduct stenosis. The obstruction (Fig. I) began 2 cm. below the third ventricle and increased caudally giving rise to a "rat tail" shadow. The aqueduct was displaced forwards and downwards suggesting a space-occupying lesion behind the pineal. To define this further, air was subsequently introduced by the lumbar route after the ventricular pressure had been lowered. The radiological appearances then were surprising (Fig. 2). All the subarachnoid cisterns were greatly dilated, particularly the cistema ambiens. The sulci of the cerebral convexities were considerably enlarged up to the midline. These findings were interpreted to indicate defective absorption of fluid at the sagittal sinus as the primary cause of the hydrocephalus. 224It was thought that the resultant rise in fluid pressure in the subarachnoid space had caused dilatation of the cisterns to such a degree that the cistema ambiens had obstructed the aqueduct and so produced a type of hydrocephalus which was at least intermittently noncommunicating. To reverse this sequence an anastomosis between the spinal theca and the peritoneal cavity was made with polythene tubing on February 25, 1954. The immediate result was highly satisfactory: skull expansion ceased, the scalp veins became less distended, and normal development was resumed. At the age of I year the child could stand with support; the skull circumference had increased by only 2 cm. At 18 months she could stand unaided; the fontanelles were closed. She had a normal vocabulary and t...

Vein of Galen Aneurysms: Presentation and Endovascular Management

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Endoscopic Treatment of Hydrocephalus Due to Aneurysm of the Vein of Galen: Case Report and Literature Review

“…We reviewed all the reported cases of vein of Galen malformations in adults and, more specifi cally, those which determined hydrocephalus ( ᭹ ᭤ Table 2 ). The development of hydrocephalus as a late manifestation of vein of Galen malformations in adult patients may be due to obstruction of the cerebral aqueduct as a direct or indirect effect of the aneurysmal mass; communicating hydrocephalus after a subarachnoid haemorrhage [66] ; a dual mechanism (aqueduct obstruction and defective absorption of CSF) [77] ; passive ex vacuo hydrocephalus; thrombosis of an aneurysm, and high venous pressure. Most authors believe that mechanical obstruction of the mesencephalic aqueduct and high venous pressure with consequent impaired CSF absorption are the primary causes of the development of hydrocephalus [78] .…”

Hydrocephalus due to Dilatation of the Dural Sinuses

“…Arteriovenous fistulae at the level of the vein of Galen and ampulla were cited by Gibson,et al,1 as producing internal hydrocephalus due to deficient absorption of Hydrocephalus due to cerebrospinal fluid, because of increased venous pressure in the superior sinus. In our case, we believe the hydrocephalus was secondary to extrinsic compression on the aqueduct and a twisting of the brain stem, related to the large size of the aneurysm.…”