Aneurysm of the vein of Galen.

Abstract

History: This 272 year old female child was admitted to Montefiore Hospital on November 13, 1956. Her birth at full term following a normal pregnancy had been unremarkable but was followed by a brief period of neonatal anoxia. At the age of 5 months the infant had an upper respiratory tract infection lasting 4 weeks. At this time the head was first noted to be enlarging more rapidly than normal. The illness was associated with moderate somnolence and lethargy because of which she was admitted to another hospital where her general condition soon improved. A ventriculogram revealed extensive hydrocephalus, and the probability of a deep thalamic neoplasm was suggested. No treatment was instituted and the child did well at home despite progressive enlargement of the head. At the age of 1 year she was readmitted to the other hospital because of severe epistaxis. The hemorrhage stopped spontaneously and did not recur. The hydrocephalus had increased and a proptosis was noted. During the next 16 months the child was alert and continued to respond to auditory and visual stimuli. The weight of the enlarging head prevented sitting or turning. The mother had noted mild, irregular elevations of the patient's temperature. Examination: On admission to this hospital the fontanelles were found to be large and bulging. The right eye was protuberant with dilated venous channels around it and over the anterior portion of the cranial vault. The head was massively enlarged. Radiographs showed severe osteoporosis of the skull, moderate suture separation, and slight enlargement of the sella turcica. Ventricular fluid was clear, colorless and without cells. Its total protein content was 16 mgm. per 100 cc. Course: The child was persistently febrile throughout her course although no infection could be demonstrated. The temperature was 100-102° F. until the terminal episode. Indigo carmine dye injected into the left lateral ventricle promptly appeared on the opposite side but none was apparent in the lumbar spinal fluid within 20 minutes. A 300 cc. ventriculogram revealed massive hydrocephalus without visualization of the aqueduct of Sylvius or 4th ventricle. The patient responded well after the procedure, but on the following day her temperature rose to 105.6° F. Despite alcohol sponges and antibiotics she expired on November 29,1956, two days after ventriCUlography. Post Mortem Findings: Permission for post mortem examination limited the investigation to the head. The body was well developed without external abnormalities. The calvarium was very thin with widened sutures. The cerebral hemispheres were greatly enlarged with flattening of the gyri and diminution of the depth of the sulci. The floor of the third ventricle was thin and bulged downward. The cranial nerves were intact. A 1.5 x 0.8 cm., thin-walled, venous channel connected the sphenoparietal sinus with the right deep middle cerebral veins which were dilated and tortuous (fig. 2). The superior and inferior longi-