Pediatric nonvertebral paraspinal arteriovenous fistulas along the segmental nerve: clinical, imaging, and therapeutic considerations.

OBJECT: The authors characterize the clinical presentation and imaging features of paraspinal nonvertebral arteriovenous fistulas (AVFs) along the segmental nerve and describe their endovascular treatment.

METHODS: The authors undertook a retrospective review of medical records, imaging, and treatment of patients with endovascular problems spanning the period from 1985 to 2003. Five pediatric patients (2-3 years of age) received diagnoses of nonvertebral paraspinal AVFs along the segmental nerve. All patients presented with an incidentally discovered continuous murmur over the paraspinal or parasternal regions. All patients were neurologically intact; two patients had cardiomegaly. The AVF was found in the midthoracic level in four patients and at L-3 in one patient. All AVFs were high-flow single-hole fistulas at the neural foramen with venous drainage into paraspinal and epidural veins but without intradural reflux. All fistulas were endovascularly occluded in the same session as diagnostic angiography took place. The fistula was completely occluded, with detachable coils in one case and with N-butyl-cyanoacrylate (NBCA) in four cases. Before NBCA injection, the flow through the fistula was decreased either by placing coils distal to the fistula or by inflating a balloon proximally. No signs of recanalization appeared on short-term follow-up magnetic resonance imaging in all patients. All patients remained neurologically intact at the last available follow-up session (mean 6 years).

CONCLUSIONS: Nonvertebral paraspinal AVFs along the segmental nerve are specific disease entities seen in children presenting with bruit and cardiomegaly. Endovascular embolization should be the treatment of choice for this rare disease.