marked suture separtion. The scalp veins, especially on the right, were distended and tortuous. Both eyes were proptosed, this being more evident on the right. The right extremities were spastic and weak, and there was a right reflex preponderance with right ankle clonus and Babinski reaction. The child was discharged following control of the nasal bleeding.

At two and one-half years of age, the child was admitted to another hospital with progressive enlargement of the head. Repeat ventriculograms were unchanged, and the child died shortly after this procedure.

Hirano and Terry (1958) have previously reported on the postmortem findings in this case. The vein of Galen aneurysm measuring 4 x 5 cm. was supplied by the right posterior cerebral artery and drained into a massively dilated straight sinus and torcula Herophili. The aneurysm and sinuses were partially occluded by a clot. The spheno- parietal sinus was connected by a vein with the right deep middle cerebral veins, and this connection probably gave rise to the proptosis. Marked ventricular dilatation re- sulted from compression of the aqueduct by the aneurysm.

Comment: A six month old female with progressive hydrocephalus, bilateral proptosis, right spastic hemiparesis, tortuous scalp veins and epistaxis was diagnosed as a third ventricular tumor by ventriculography. Death occurred at two and one-half years following a diagnostic air contrast study, and the diagnosis of vein of Galen aneurysm was made at necropsy.

Clinical syndromes with review of the literature Evaluation of our eight patients and the case reports from the world literature resulted in a arbitrary classification based on age of onset of signs and symp- toms. It became apparent that there were three major age groups in whom this arteriovenous malformation became symptomatic: early neonatal life, with the major signs resulting from the marked effects on the cardiovascular system; during infancy in which progressive hydrocephalus andlor convulsions were prominent features; and later childhood or adult life, in which head- aches with or without signs of subarachnoid hemorrhage were seen.

Litvak, Yahr, and Ransohoff ( 1 960) have separated the midline arterio- venous malformations into three categories based on pathological differences. One may then reasonably question how the “same” arteriovenous malforma- tion could become symptomatic in so varied a manner. It is our belief that although there may be anatomical similarities, the malformation may differ greatly in its function. The symptomatic neonate has a shunt of arterial blood to the venous circulation of such magnitude as to result in peripheral con- gestion and congestive heart failure. In infancy, it is of lesser magnitude, for although definite physiological alterations of cardiovascular function may be apparent, there is paucity of cardiac manifestations with only abnormal

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roentgenograms or cardiac function studies. In this group, the shunt results in marked dilatation of the vein of Galen and secondary compression of surrounding structures. Clinically, this is most apparent as aqueductal stenosis and secondary hydrocephalus. The older child or adult has a smaller arterio- venous shunt with only a moderate dilatation of the vein of Galen. There is neither cardiovascular dysfunction or hydrocephalus. It becomes clinically symptomatic as headaches by its effect on pain sensitive structures or by rupture with subarachnoid hemorrhage.

Neonatal Period (Chart I )

Newborn children with symptomatic vein of Galen aneurysms were all males, and at, or shortly after birth, invariable presented with cyanosis and1 or respiratory distress. Physical findings were suggestive of congestive heart failure, but the condition was refractory to digitalis therapy in five children. Eight of the nine infants died from myocardial insufficiency.

Hydrocephalus as an unusual secondary complication was reported by Glatt and Rowe (1960) in a nine week old child, and Pollack and Larlett (1958) described a child with an. increased head circumference at birth. Intra- cranial bruits were not present in the two children in whom an attempt was made to delineate this sign. Convulsions were present in three children: one (Claireaux and Newman, 1960) had an associated marked hypoglycemic state; while the others (Glatt and Rowe, 1960; Hirano and Solomon, 1960) had no adequate explanation for the seizures. Subarachnoid hemorrhage was present in two neonates. Claireaux and Newman (1960) reported a small hemorrhage limited to the cerebellum in their child. Three additional neonates had xanthochromic cerebrospinal fluid. Since newborns frequently have xan- thochromic fluid, the presence of such fluid by itself in this age group can- not be utilized as evidence for antecedent hemorrhage.

All the malformations were supplied by one or both posterior cerebral arteries. Five of the nine children showed additional supply from the superior cerebellar artery, and three also had involvement by the anterior and/or middle cerebral arteries. In this age group there was a tendency for these arteries to form a network around the dilated vein of Galen as demonstrated pathologically in Patient 3 and as recorded in the excellent pathological study by Gagnan and Boileau (1960).

None of the neonates survived, and in all cases the diagnosis was con- firmed at necropsy. Death was associated with cardiovascular decompensa- tion in all but the child described by Glatt and Rowe (1960). This neonate initially showed symptoms and signs of cardiac failure, and at nine weeks of age had evidence of hydrocephalus. Following shunting procedures, the child died at five and one-half months of age from a coagulase nega-ive

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Staphylococcus meningitis. Postmortem examination revealed the heart to be of normal size.

Other complicating conditions which greatly influenced the prognosis was the marked hypoglycemia and convulsions in the neonate reported by Claireaux and Newman (1 960), and the transposition of the great vessels, the patent ductus arteriosus and the patent foramen ovale in the case described by Hirano and Solomon (1960). Two neonates described by Glatt and Rowe (1960) had aberrant veins draining from the vein of Galen. One had a vein directed superiorly through the tentorium to the superior longitudinal sinus, while the other had a venous channel to the occipital dural sinus. Patient 3 had a hypoplastic superior sagittal sinus and superior and middle cerebral veins. Anomalies not affecting the ultimate outcome were the cervical cystic hygroma and the hepatic hemangioma in Patient 3, suFcrnumerary digits in the neonate reported by Claireaux and Newman (1 960) and hyFospadius in Hirano and Solomon’s (1960) case.

Infancy (Chart 2)

The infant with a symptomatic vein of Galen aneurysm invariably prezented with hydrocephalus and/or convulsions.

Hydrocephalus present in 18 of the 19 children, was the initial manifesta- tion in eight of them. The process was highly variable in severity, being minimal in some cases and of major proportions in others.

Convulsions occurred in eight children, and was the initial sign of the pathological process in five. Grand ma1 was the outstanding seizure type, but focal motor seizures were also observed. Subarachnoid hemorrhage wai present in six infants and was associated with convulsions in five of them.

Distension and tortuosity of scalp veins was the initial sign in five case:. In most instances this was localized in the region above the glabella.

Although the majority presented with hydrocephalus, convulsions or venous distension of scalp veins as their initial complaints, subsequent signs and symptoms were highly variable. These consisted of psychomotor retardation in seven, pyramidal tract involvement in five, cardiomegaly in five, proptosis in four, recurrent epistaxis in two, a pulsatile retromastoid mass in two and papilledema in another child. An important diagnostic sign in this age group was the intracranial bruit. This was present in ten children. The bruit was a loud, harsh sound, synchronous with cardiac systole, which could be diminished by carotid compression.

Carotid arteriography confirmed the diagnosis in 14 of the 19 children. The remainder were confirmed at necropsy. This is in contrast to the neonatal group, in whom all were diagnosed at autopsy. Air contrast studies were

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never completely diagnostic, but at times demonstrated the presence of a posterior third ventricular mass suggestive of a vein of Galen malformation.

Four infants are alive and two were lost to followup. Surgical clipping of the feeding arteries was performed in three of the living children. The child reported by Boldrey and Miller (1 949) is asymptomatic eight years following surgery with an I.Q. in the “mid-eighties”. French and Peyson’s (1954) patient is markedly retarded, and Patient 2 from our series has done well except for a postoperative subdural requiring surgical drainage. The other living child described by Thomson (1959) did not require surgery and is asymptomatic three years after arteriographic diagnosis.

Seven of the 13 deaths could be directly related to postoperative com- plications that included increased intracranial pressure, hemorrhage, cardiac decompensation and infection.

The remaining deaths, unrelated to surgery, were most frequently as- sociated with a subarachnoid hemorrhage.

Older Child and Adult (Churt 3 )

The older child, adolescent, or adult, generally presented with headaches andlor signs and symptoms of a subarachnoid hemorrhage as the initial manifestations of a symptomatic vein of Galen aneurysm.

The average age of onset was 12.9 years, and the diagnosis was made 14 years later.

Headaches were the initial complaint in 10 of the 13 patients, and this symptom occurred in an additional two during the course of the disease. These two patients, both children, initially presented with convulsions or a behavioral disorder. Excessive fatigue, distension of scalp veins, vertigo, lethargy, epistaxis, hemiparesis and signs of meningeal irritation were also seen.

Sxbarachnoid hemorrhage occurred in 10 patients and was associated with headaches as the initial complaint in four.

Subsequent signs and symptoms indicative of central nervous system dys- function were frequently observed. These consisted of convulsions, paretic phenomena, ataxia of the cerebellar type, apha;ia. cranial nerve palsies, vertical and horizontal nystagmus, vertigo, signs of meningeal irritation, personality aberrations, and mental retardation. Ocular disturbances were seen in eight patients. Paralysis of upward gaze and decreased visual acuity were most frequently observed, but proptosis, eyelid ptosis, photophobia, papilledema, external strabismus and loss of the pupillary reflex were also encountered. I t is of interest that an intracranial bruit was rarely heard, being present in only one child.

Plain skull x-rays were of diagnostic importance in this older age group.

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All five patients with reported studies showed a characteristic concentric calcification in the wall of the vein of Galen aneurysm. Carotid and vertebral angiography confirmed the diagnosis in 1 1 patients, and the remaining two were confirmed at autopsy. The posterior cerebral artery, as noted in the other groups, was the major feeding artery, but in some cases there was also supply by the anterior and middle cerebral and the cerebellar arteries.

Six of the I t older children or adults with sufficient follow-up data are alive. Four are asymptomatic, o,ne has a residual postoperative homonymous hemianopia, and the other developed a psychosis requiring institutionaliza- tion. Carotid artery ligation or clipping of the feeding vessels were performed on all but two of the living patients. The two patients not subjected to surgical intervention are living, one is asymptomatic and the other psychotic. There were five deaths. Four were secondary to operative or postoperative com- plications, while the other (Oscherwitz and Davidoff, 1947) died from a probable subarachnoid hemorrhage four years after the vein of Galen aneu- rysm was punctured.

Discussion Arteriovenous malformations of the vein of Galen differ from other cerebral arteriovenous fistulas by the varying clinical manifestations in different age groups, the frequent early onset of symptoms and signs, and the common co-existence of cardiovascular effects and/or hydrocephalus.

Schmitt (1 920) reported the first case of cardiovascular dysfunction secondary to a cerebral arteriovenous fistula. Subsequent reviews by Cushing and Bailey (1 928), Dandy ( I 928), Schlesinger and Hazen (1954), and Hook, Werko and Ohrberg ( 1958) frequently failed to mention this complication, and questioned whether a cerebral arteriovenous fistula ever caused cardiac embarrassment. It is evident from the cardiac function studies of Shenkin and associates (1948), the clinical reviews of Ray (1941) and Paterson and McKissock (1956), and the data from our cases, that this complications is not rare, and a symptomatic newborn or infant with the vein of Galen mal- formation frequently shows evidence of cardiovascular dysfunction. Cerebral arteriovenous fistula do not differ from vascular malformations in other area: of the body. Cardiovascular alterations may occur when there is a large func- tional arteriovenous shunt. The failure to demonstrate such changes in many older patients was probably due to the presence of a small functional shunt.

The physiological alterations formations can be summarized systemic circulation producing

associated with cerebral arteriovenous mal- as a “shortcircuiting” of a portion of the a lowered peripheral resistence which in

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