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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, are very rarely diagnosed in uterus, due to a lack of understanding of the condition and because they may be hard to visualize. We report a rare case of a mass effect PAVF diagnosed with fetal MRI, involving the right cerebral hemisphere, fed by a pericallosal artery and associated with a giant venous dilatation. The PAVF was initially managed by the endovascular embolization. The recruitment of a middle cerebral artery (MCA) feeder and the rapidly enlarging size of the venous pouch with mass effect required subsequent surgery. The two-stage multimodal treatment resulted in complete disappearance of the PAVF without complications.
PMID: 28559076 [PubMed - as supplied by publisher]