Antenatal course of a fetal intracranial arteriovenous fistula: a case report

BACKGROUND: Intracranial arteriovenous fistulas (AVFs) are rare in childhood. AVFs in fetuses are very rare and usually not associated with the vein of Galen. These can be solitary or multiple and may be associated with aneurysms or AVMs. The presentation of an AVF depends on the age of the patient, site and size of the fistula, and any accompanying lesion. Neonates can present with cardiac failure and a cranial bruit, infants with cardiomegaly and cardiac failure, children with hydrocephalus, and adolescents and adults with headaches, seizures or intracranial hemorrhage. CASE: Ultrasound and Doppler flow studies revealed a large, vascular lesion in the left cerebral hemisphere that was consistent with an arteriovenous malformation or fistula. The fetus developed cardiomegaly and transient pleural effusion not hydrops. Planned cesarean section without manipulation of thefetal head was performed at 39 weeks. Embolization during the neonatal period completely ceased flow through the arteriovenous fistula. At 28 months of age the child was doing well and showed no developmental or neurologic deficits. CONCLUSION: Prenatal recognition of fistulas and malformations can result in a good outcome as long as thefetus does not develop cardiac failure. Knowledge of the presence of an extensive arteriovenous malformation orfistula prior to birth allows planned delivery and permits the parents and medical team to be prepared for treatment at a tertiary care center.