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

Abstract Background Pial arteriovenous fistulas (AVFs) are rare intracranial vascular lesions consisting of one or more feeder arteries connecting directly to a venous system without a nidus, in the subpial space. Due to 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 paediatric arterio-venous 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 paediatric 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. Embolisation was undertaken three times before successful surgical disconnection was done. Genetic testing for associated syndromes were all negative. Conclusion Prominence of facial veins could be one of the more uncommon presenting features of pial AVFs. Genetic testing should always be considered in the paediatric population diagnosed with AVFs due to their association to various syndromes. Despite endovascular embolisation being considered the less invasive choice, decision on mode of treatment should be a multifactorial decision.