Arteriovenous Malformations of the Brain in Children: A Forty Year Experience

Abstract

ABSTRACT:Despite the great capacity for the pediatric brain to recover from stroke, the morbidity and mortality in children who harbor an arteriovenous malformation (AVM) remains high. This study examines the clinical data and management experience with 132 patients with brain AVM from 1949 to 1989. Although the high tendency for a childhood AVM to present with hemorrhage (79%) remained constant for the forty year study period, the associated morbidity and mortality of hemorrhage changed. The mortality rate from hemorrhage for the entire series was 25%, which was reduced from 39% to 16% after the introduction of computed tomography. The mortality from AVM hemorrhage since 1975 was dependent on location; 8 of 14 patients (57%) with a cerebellar AVM died from hemorrhage while only 2 of 44 patients (4.5%) with a cerebral hemisphere AVM died (p < 0.0001). Sixteen children (12%) presented with a chronic seizure disorder. Surgical excision of the malformation resulted in complete seizure control off anti-convulsant medication in 73% of patients. Although 21% of patients were treated non-operatively (many with terminal poor-grade hemorrhage), 79% had a surgical procedure with total AVM excision achieved in 70 patients (53.1%). Complete AVM resection was followed by a normal neurological outcome in 47 children (67%). Most partial excisions (n=9) and clipping of feeding arteries (n=7) were performed in the early years of this study, and did not provide protection from rehe-morrhage. Although conservative management has been advocated for selected non-hemorrhagic AVMs, we conclude that essentially all children with an AVM should be treated in order to eliminate the risk of hemorrhage. Long-term conservative management in pediatric patients is warranted only in patients with large AVMs not amenable to treatment using current multimodality techniques.

Rates and predictors of seizure freedom in resective epilepsy surgery: an update

“…The mechanisms of epileptogenesis in AVMs are not fully known, but have been proposed to include cerebral ischemia from neighboring arteriovenous shunting, hemosiderin deposition, gliosis, demyelination, and kindling of excitatory synapses [125, 129, 191, 211, 216]. Various authors have reported the incidence of epilepsy with brain AVMs as anywhere from 12 to 57 % of patients [39, 107, 110, 118, 123, 143]. Epilepsy is more likely in patients with a history of hemorrhage, with larger lesions, and with AVM location in the temporal or frontal lobes [39, 54, 79, 104, 110, 118, 157].…”

“…Several case series have examined rates of seizure outcomes in the surgical treatment of AVMs causing seizures, with Engel class I outcomes reported in 49–94 % of patients [79, 107, 123, 157, 191, 217]. In a recent observational study of 440 patients with supratentorial AVMs undergoing resection at our institution, 130 (30 %) had preoperative seizures [79].…”

Multimodality management and outcomes of brain arterio-venous malformations (AVMs) in children: personal experience and review of the literature, with specific emphasis on age at first AVM bleed

“…Brain AVMs result in 35%–55% of haemorrhagic strokes in children, with an incidence of 1.4 per 100,000 person-years and significant mortalities and morbidities [4, 5]. In one series, mortality reached 25% when averaged over 40 years, with an improvement from 39 to 16% over this time, coinciding with technological advances [6]. The annual bleeding risk of ruptured AVMs in children was reported as 2.71% and increased when associated with aneurysms and deep venous drainage [7].…”

Radiosurgery of brain arteriovenous malformations in children

“…Brain arteriovenous malformations (bAVM) in children are reported to have a less favourable prognosis than bAVMs in adults, with a mortality rate after hemorrhage above 20 % [17]. Children (< 20 years) with a bAVM were found to present significantly more often with a hemorrhage (56 %) in comparison to adults (43 %), but they were not found to be at an increased risk for a subsequent hemorrhage [8].…”