Dural arteriovenous fistulae in pediatric patients: associated conditions and treatment outcomes

Abstract

Objective: Dural arteriovenous fistulae (dAVF) are rare vascular lesions of the CNS with potentially significant morbidity and mortality. Our experience in the treatment of children with dAVF is reported.

Design: Our neurosurgical and neurointerventional pediatric database was retrospectively reviewed for patients with dAVF. Brain arteriovenous malformations, pial fistulae, vein of Galen malformations and extracranial fistulae were excluded. Angiography was used to assess radiographic outcome in all cases. Clinical outcome was assessed by the pediatric Functional Status Scale (6=normal, maximal incapacity=30).

Results: Between July 2003 and June 2011, seven patients with dAVF were treated, with a mean age at presentation of 4.8 years. Presenting symptoms included facial venous prominence, headache, macrocephaly and symptoms of elevated intracranial pressure; one patient (14%) had asymptomatic dAVF discovered on imaging follow-up after craniotomy for cavernous malformation. Two cases (28%) were associated with vascular anomaly syndromes, PHACE association and PTEN associated syndrome. Venous sinus thrombosis was identified in four patients (57%) and was associated with a systemic hypercoagulable state or trauma. Complete obliteration was achieved in six of seven patients. Treatment was exclusively endovascular in six patients and by combined surgical/endovascular means in the remaining patient. No treatment complications were experienced. Functional Status Scale scores at an average of 2 years of follow-up were 6 in all patients.

Conclusion: Treatment is effective in the obliteration of dAVF in children and is well tolerated, with patients having excellent prognoses. Genetic screening and hypercoagulable workup are indicated, particularly when other anomalies or sinus thrombosis coexist.