Prenatal Diagnosis and Multimodal Neonatal Treatment of a Rare Pial Arteriovenous Fistula: Case Report and Review of the Literature

Abstract

Background: Intracranial pial arteriovenous fistulas (PAVFs) are direct communications between the arterial and venous system of the brain, with the characteristic absence of a plexiform nidus, as seen in the classic cerebral arteriovenous malformations. These vascular malformations, usually occurring in the pediatric population, very rarely are diagnosed in utero, because of a lack of understanding of the condition and because they may be hard to visualize.

Case description: We report a rare case of a mass-effect PAVF diagnosed with fetal magnetic resonance imaging, involving the right cerebral hemisphere, fed by a pericallosal artery and associated with a giant venous dilatation. The PAVF initially was managed by the endovascular embolization. The recruitment of a middle cerebral artery feeder and the rapidly enlarging size of the venous pouch with mass effect required subsequent surgery.

Conclusions: The 2-stage multimodal treatment resulted in complete disappearance of the PAVF without complications.

Keywords: Endovascular embolization; Fetal pial arteriovenous fistula; Radical surgery.