Spinal intradural arteriovenous fistula with unusual presentation: case report and literature review.

OBJECT: The authors present their experience with a Cervical Spinal intradural arteriovenous fistula (AVF) with unusual clinical presentation in a pediatric patient and review of the international literature on this condition. METHODS: A 5-year-old girl was found by her parents in bed screaming and talking inappropriately. Later on, she calmed down and complained of occipital, back and neck pain. She was admitted to the CHEO-ICU for work up and observation. The admission head CT scan showed evidence of SAH. Brain MRI showed blood in the third and fourth ventricles. MRA showed a C2 vascular lesion consistent with spinal cord AVF. Conventional angiogram was done which confirmed the AVF fed by a posterior medullary artery originating from the left vertebral artery. An attempt to embolize the AVF with glue was made but proved to be unsuccessful. On December 4, 2002, direct surgery on the AVF was performed, through a midline posterior approach (C2-C3 laminectomies) and microsurgical technique a complete excision of the intradural extramedullary AVF was achieved. The postoperative course was uneventful and the patient remained neurologically intact. Post-operative angiography confirmed complete excision of the AVF. CONCLUSION: The rarity of a pediatric spinal AVF presenting with spontaneous Intracranial SAH, makes this case very peculiar. This is the first reported pediatric case of cervical cord AVF with this clinical presentation found in the literature.