Dural arteriovenous fistulae in a 6-year-old girl with trisomy 21 and congenital heart disease

Abstract

Dural arteriovenous fistula (DAVF) represents a pathological group of intracranial shunts arising from the dural artery to venous sinus and veins. Childhood-onset DAVF is generally considered to be poor in prognosis, whereas only limited information is available for the onset and long-term outcomes. We herein report a Japanese girl with trisomy 21, large ventricular septal defects, and pulmonary vein stenosis, for which a transcatheter stent had been placed after birth. At age 6 years, she developed bacterial meningitis due to S. pneumoniae, leading to the diagnosis of venous sinus thrombosis and multiple intracranial shunts. Cerebral angiography identified multiple shunts arising from the middle meningeal arteries to the superior sagittal sinus and a concurrent reflux to cortical vein. Endovascular embolization successfully occluded the shunts without neurovascular complications over 24 months. This report first demonstrates the favorable outcome of DAVF in a pediatric patient with trisomy 21 after the catheter intervention. For children at a risk for intracranial thrombosis, preemptive neurovascular evaluation and transcatheter intervention provide a chance of early diagnosis of DAVF to improve their survival and neurologic outcome.