Intracranial pial arteriovenous fistula in infancy: a case report and literature review

Abstract

Intracranial pial arteriovenous fistulas (AVF) are rare vascular malformation especially in the first 2 years of life. The pathology in this age group is associated with greater morbidity and mortality. We report a rare case of 36-day-old male infant with a pial AVF associated with an arterial aneurysm, who presented with intraventricular hemorrhage and hydrocephalus. In addition, an online review of the literatures on pediatric pial AVF was performed using PubMed on published case reports and articles from 1980 to April 2013.

Intracranial pial fistulas in pediatric population. Clinical features and treatment modalities

“…The shunt was totally occluded with NBCA. This case is similar to the one published by Kraneburg et al [2]. Case no.…”

“…Pial arteriovenous fistulas (PAVFs) are a group of infrequent entities affecting predominantly the pediatric population [2][3][4]14]. They are a high-flow shunt communicating one or more pial arteries with a single vein without an intervening nidus, located in the subpial space [1,3,14].…”

Non-galenic arteriovenous fistulas in adults: transarterial embolization and literature review

“…Unlike patients with AVM, a relatively small proportion presented with hemorrhage (5 of 26; 19.2%). The clinical features of adult patients with NGAVFs were similar to those reported in older children and distinct from neonates and infants, who are more apt to present with cardiac symptoms 2 9 24…”

Nongalenic pial arteriovenous fistula: Prenatal diagnosis

“…In later stages, it may present as hydrocephalus, focal neurologic deficits, headaches, or seizures . Hemorrhagic manifestations are less common . As a result, it is quite likely that prenatal diagnosis is only possible in cases with the most complex and large fistulae, with detectable abnormality in the central nervous and venous systems, and which lead to secondary heart failure.…”

“…3,14 Hemorrhagic manifestations are less common. 15 As a result, it is quite likely that prenatal diagnosis is only possible in cases with the most complex and large fistulae, with detectable abnormality in the central nervous and venous systems, and which lead to secondary heart failure. In our case, the first sonogram did not directly lead to the diagnosis of cerebral fistula, but indirect signs (cardiomegaly, enlarged vena cava and cervical venous vessels, ventriculomegaly, and an enlarged venous sinus) raised the suspicion of a high-flow vascular pathology in the central nervous system.…”