Surgical care burden in orbito-temporal neurofibromatosis: Multiple procedures and surgical care duration analysis in 47 consecutive adult patients.

摘要: Patients with orbito-temporal neurofibromatosis (OTNF) bear a heavy burden of surgical care. We studied 47 consecutive patients OTNF from the French Neurofibromatosis 1 Referral Center cohort (n > 900), over 15-year period to determine clinical features most likely predict repeat surgery and longer duration Forty-seven (5.2% NF1 patients' cohort) underwent 79 procedures 4.8 years average follow-up. Soft-tissue had high revision rate (19/45 patients), skeletal did not (2/13 patients). Transosseous wire canthopexy facial aesthetic unit remodeling were associated stable outcome. Ptosis repair carried an unfavorable outcome, particularly in presence sphenoid dysplasia. Stable was achieved polyethylene implants and/or cementoplasty. Multiple undertaken 70% predicted by NF volume, canthopexy, dysplasia, or Jackson's classification 2 3; but declining visual acuity. A based upon predictive risk repeated is proposed: Group 1: Isolated soft tissue infiltration requiring levator palpebrae canthal surgery; 2: Soft involvement ptosis great axis 4.5 cm; 3: Presence dysplasia pulsatile proptosis, regardless