Surgical Disconnection of a Residual Pediatric Pial Arterio-Venous Fistula Following Partial Embolization: A Case Study

Background: Pial arteriovenous fistulas (AVFs) are rare intracranial vascular lesions consisting of 1 or more feeder arteries connecting directly to a venous system without a nidus, in the subpial space. Because of the high-flow system, they are commonly associated with a large varix. They are thought to represent between 1.6% and 7.3% of all pediatric arteriovenous malformations (AVMs). Morbidity and mortality is high in this condition and surgical or endovascular treatment options are usually considered. There have been limited reports on the clinical features, treatment options, and outcomes of pial AVMs due to its rarity. We present a case study of a pediatric patient in our institution and her clinical course, focusing on her presenting clinical features and management.

Case description: A 1-year-old girl presents with progressively prominent and dilated facial veins and no other features suggestive of pial AVF. She was diagnosed with pial AVF with two feeder arteries and a large varix on imaging. Embolization was undertaken 3 times before successful surgical disconnection was done. Genetic testing for associated syndromes were all negative.

Conclusions: Prominence of facial veins could be 1 of the more uncommon presenting features of pial AVFs. Genetic testing should always be considered in the pediatric population diagnosed with AVFs because of their association to various syndromes. Despite endovascular embolization being considered the less invasive choice, decision on mode of treatment should be a multifactorial decision.