Intracranial Arteriovenous Malformations in Childhood John J. Kelly, Jr, M D , James F. Mellinger, M D , and Thoralf M. Sundt, Jr, M D

During the 13-year period 1964 through 1976, 37 patients less than 20 years old with an intracranial, parenchymal arteriovenous fistula were seen at the Mayo Clinic. T h e most frequent mode of presentation was hemorrhage or seizure. Other than angiography, computed tomography with contrast enhancement was the most helpful diagnostic test. Surgery was restricted to patients with intraparenchymal hematomas, intractable seizures, or subarachnoid hemorrhage with accessible lesions and to 1infant with amassive, symptomatic malformation. Surgery generally was tolerated well, with reversal o f most acute focal neurological deficits related to hematomas. In the nonsurgical group, follow-up revealed a fairly stable neurological status during the period of the study. Kelly JJ Jr, Mellinger JF, Sundt T M Jr: lntracranial arteriovenous malformations in childhood. Ann Neurol 3:338-343, 1978

Arteriovenous malformations of the brain parenchyma are a rare cause of morbidity and mortality during the first two decades of life. As a result, the natural history of these lesions is unclear. W e reviewed the recent experience at the Mayo Clinic with patients less than 20 years of age who had this malformation and evaluated the presentation and prognosis.

Materials and Methods Medical records were reviewed for the 13-year period January 1, 1964, through December 3 1 , 1976, to identify patients seen during the first two decades of life with symptoms and signs leading to the diagnosis of an intracranial parenchymal arteriovenous malformation. Histopathological o r angiographic proof of the anomaly was required for the patient to be included in the study. To ensure homogeneity in the series, patients with other types of vascular malformation such as Sturge-Weber syndrome, dural arteriovenous malformation, venous angioma, telangiectasis, and all lesions without definite evidence of intraparenchymal arteriovenous shunting were excluded. Follow-up data were obtained by a later examination, correspondence with the local physician, o r communication with the family. Patients were assigned a numerical disability rating at the time of final contact. Follow-up ranged from 1 to 148 months with a mean of 49 months. Thirty-seven patients ( 2 2 males and 15 females) were included in the study. Four patients were seen in the first year of life and most of the others between the ages of 8 and 20 years old (Table 1). Of the 37 lesions, 3 1 were located in the cerebrum (16 right and 15 left), 2 in the midline, 1 in the brainstem, and 3 in the cerebellum.

From the Mayo Clinic and Mayo Foundation, Rochester, MN. Accepted for publication Nov 2, 1977.

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Results Presenting Features Hemorrhage accounted for 5 1$? (19 lesions) of the initial presentations; 3 of these lesions were cerebellar (Table 2). Seizures comprised the next most common category (8 lesions or 22%). Only 6 patients (16%) had mixed presentations (two or more features). Focal neurological deficits were uncommon except when an intraparenchymal hematoma was present or when chronic, fixed, focal motor deficits were initially seen (3 patients). T h e degree of obtundation in our patients with hemorrhage was relatively mild, and only 2 patients were comatose, 1 of whom died. A bruit was identified in 6 of the 2 6 patients in whom i t was sought, and all 6 had large lesions.

Diagnostic Studies Various diagnostic studies were performed (Table 3). Plain skull roentgenograms performed in 29 patients were abnormal in only 5 . Three of these 29 patients had evidence of calcification of the arteriovenous malformation, and 1 additional patient had a marked unilateral increase in the venous channels of the skull; 1 had evidence of macrocephalus only. In 24 patients for whom plain skull roentgenograms were taken, results were either completely normal or nondiagnostic. Twenty-five patients underwent electroencephalography. Focal slowing over the area of the arteriovenous malformation was demonstrated in 1 1,

Address reprint requests to Dr Kelly, Mayo Clinic, Rochester, MN 55701.

0364-5134/78/0003-0410$01.25 @ 1978 by John J. Kelly, Jr

Table I . Distribution of 37 Patients with Arteriovenous Malformation According to A g e at Presentation Age

(v)

Intracranial arteriovenous malformations in childhood.

Intracranial Arteriovenous Malformations in Childhood John J. Kelly, Jr, M D , James F. Mellinger, M D , and Thoralf M. Sundt, Jr, M D During the 13-...
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