Intracranial pial arteriovenous fistula

Intracranial pial arteriovenous fistulas (AVFs) are rare cerebrovascular malformations. Intracranial pial AVFs have single or multiple arterial connections to a single venous channel.

Since 1986 they have been recognized as a distinct pathological entity from other intracranial cerebrovascular malformations ¹⁾.

Because pial AVF has a poor natural history, the clinical suspicion of pial AVF, followed by prompt appropriate treatment is important ²⁾.

Pediatric pial arteriovenous shunts in the brain and spine are challenging to understand because of low incidence, variable presentation, and associations with genetic syndromes. What is known about their natural history comes from reviews of small series. To better understand the natural history and role of intervention, two cases are presented followed by a review of the literature. In the first case, an infant with a prior history of intracranial hemorrhage from a ruptured pial fistula returns for elective embolization for a second pial fistula which was found to be spontaneously thrombosed 2 weeks later. In the second case, a 5-year-old with a vertebra-vertebral fistula, identified on workup for a heart murmur and documented with diagnostic angiography, is brought for elective embolization 6 weeks later where spontaneous thrombosis is identified. In reviewing the literature on pediatric single-hole fistulae of the brain and spine, Chen et al. offer some morphologic considerations for identifying which high-flow fistulae may undergo spontaneous thrombosis to decrease the potentially unnecessary risk associated with interventions in small children ³⁾.

Intracranial pial AVFs are rare cerebrovascular lesions, with less than 100 reported cases since 1970 to 2007 ⁴⁾.

They differ from brain arteriovenous malformations in that they lack a true nidus. They differ from dural arteriovenous fistula in that they derive their arterial supply from pial or cortical artery and are not located within the dura mater.

De novo aneurysm

De novo dural arteriovenous fistula

Intracranial pial arteriovenous fistula case reports.