An Intracranial Aneurysm and Dural Arteriovenous Fistula in a Newborn

Abstract

The authors present the case of a newborn with an intracerebral aneurysm and a dural arteriovenous fistula. The patient initially presented with intraventricular hemorrhage and hydrocephalus, with evidence of remote subarachnoid hemorrhage, left hemispheric stroke, and sagittal sinus thrombosis. He was treated with a ventriculoperitoneal shunt and subsequent staged endovascular obliteration of both the aneurysm and fistula. Interestingly, the aneurysm did not appear on an artery feeding the abnormal fistula. Intracerebral aneurysms in the neonatal population are rare, and dural arteriovenous fistulae even more so; we present a case of a 2-month-old infant with both, as well as a review of the literature concerning these rare vascular abnormalities.

Pediatric dural arteriovenous malformations

“…10,11,13 Dural sinus malformations are further divided into two subtypes: 1) those involving the posterior sinuses with or without the torcular herophili, with giant dural lakes and slow-flow mural arteriovenous shunting; and 2) those involving the jugular bulb with otherwise normal sinuses, but associated with a high-flow sigmoid sinus dAVM. Patients with dural sinus malformations typically present with high-output cardiac failure, venous sinus thrombosis, and consumptive coagulopathy in the antenatal or neonatal period.…”

“…Patients with dural sinus malformations typically present with high-output cardiac failure, venous sinus thrombosis, and consumptive coagulopathy in the antenatal or neonatal period. 3,10,11,13 Infantile dAVSs are high-flow, low-pressure systems with patent sinuses and no venous lakes. Meningeal and occipital vessels are often directly involved.…”

“…Clinically, infantile dAVSs manifest in early childhood with macrocrania, seizures, hydrocephalus, and progressive neurological deficits. [7][8][9][10][11]13 Adult dAVSs present in older children and are nearly always located in the cavernous sinus. These are generally secondary to trauma, venous sinus thrombosis, or surgery, and present with venous hypertension, proptosis, and chemosis.…”

“…These are generally secondary to trauma, venous sinus thrombosis, or surgery, and present with venous hypertension, proptosis, and chemosis. 7,9,10,13 Patients may also present with significant intracranial hemorrhage and neurological deficits if cortical venous hypertension is present. 4 The arterial supply, venous drainage, sites of the dAVM, and clinical presentation of the cases in this series were similar to those reported in the literature with respect to infantile dAVSs.…”

“…Embolization has often been performed in stages to achieve gradual closure of the shunt in an attempt to maintain the patency of the abnormally enlarged dural sinus, allowing it time to undergo remodelling. 6,[8][9][10][11][13][14][15] Medical treatment with anticoagulants, diuretics, and inotropic agents have been used in this process to prevent dural sinus thrombosis and hyperdynamic cardiac failure. 9,14 Radiation therapy has been used to treat adult dAVMs, but its role in pediatric dAVMs is less clear due to the young ages of the patients.…”