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  • Spinal cord compression from spinal root neurofibromas in Neurofibromatosis type 1 (NF1)

    Final Number:
    1471

    Authors:
    Jose M De Campos MD, PhD; Maria Elena Kusak MD; Delia Viñas MD; Anna Lo Presti; Sergio Garcia-Urquiza; Beatriz Sobrino; Jose Fortes

    Study Design:
    Other

    Subject Category:

    Meeting: Congress of Neurological Surgeons 2013 Annual Meeting

    Introduction: Neurofibromas are characteristic of NF1, growing along cutaneous and subcutaneous nerves. Although mainly asymptomatic, spinal neurofibromas are present in at least 40% of NF1 patients. Only few small series are available, with limited information about the management of spinal cord compression from spinal neurofibromas. We here review our experience in the management of these tumors, and their particular characteristics.

    Methods: We performed a retrospective clinical reports review in a series of NF1 patients, followed and treated in a neurosurgical NF referral center. Demographic, clinical, anatomical, and pre and postoperative neurological status (McCormick´s classification) result data were analyzed.

    Results: Ten surgeries were performed in a series of 260 patients. Two procedures at distant levels in two patients were evaluated as separated events. Age ranged from 16 to 51 years. Clinical presentation was tri-quadriparesis in 5, paraparesis in 2, and monoparesis in 3 cases. The level was cervical in 8, and thoraco-lumbar in 2. Tumors grew from anterior (7) and/or posterior (9) roots. Tumor was resected in all cases, subtotally in motor eloquent levels, sparing rootlets with small tumor nodules. Most were followed by one clinical level upgrade, with full recovery in some patients. In one patient, with a long standing severe quadriparesis, the deficit persists. No tumor recurred or progressed at 12 m – 12 y follow-up. No kyphotic deformity was observed.

    Conclusions: Spinal cord compression from spinal root neurofibromas is an infrequently reported known complication in NF1 patients. The risk of becoming symptomatic doesn’t decrease with age. Although lesions are more frequently cervical, caudal spinal cord is also affected. Resection of these symptomatic neurofibromas is followed by very good results, except in patients with severe neurological deficit. Although asymptomatic spinal root neurofibromas should not be treated, surgery should be considered and planned as the initial symptoms appear for a best functional result.

    Patient Care: Improving surgical results by providing knowledge on the best timing and surgical technique

    Learning Objectives: Identifying the characteristics and adequate timing for surgical treatment of NF1 spinal root neurofibromas.

    References:

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