Neonatal superficial cerebral arteriovenous malformations.

The purpose of this report is to determine the role of various imaging modalities, and the outcome of early intervention in neonates with high flow pial arteriovenous malformations (AVM). This report describes 4 neonates with pial AVM associated with congestive cardiac failure, and reviews previous reports. Among the 4 infants in our series, 3 underwent early surgical resection of the AVM, complete in 2 and partial in 1. One infant suffered hemorrhagic infarction postoperatively and has a mild deficit on clinical follow-up; the other 2 patients had no worsening of mild neurologic deficits after surgery, and subsequent development has been normal. The fourth infant had absent deep venous drainage and died from intracranial hemorrhage following transvenous embolization. A single vessel arteriogram in 1 infant led to underestimation of the lesion and unexpected findings at surgery. Complete angiographic study is important in planning treatment, as the vascular anatomy of the lesions may be complex. Cranial ultrasound is useful in early diagnosis, but may miss the superficial lesions. Untreated infants with pial AVM have a poor prognosis due to cardiac failure or progressive cerebral ischemia and neurologic regression. This series illustrates the benefits of complete angiographic investigation and early treatment.