title: Surgical care burden in orbito-temporal neurofibromatosis: Multiple procedures and surgical care duration analysis in 47 consecutive adult patients.
abstract: Patients with orbito-temporal neurofibromatosis (OTNF) bear a heavy burden of surgical care. We studied 47 consecutive patients with OTNF from the French Neurofibromatosis 1 Referral Center cohort (n > 900), over a 15-year period to determine the clinical features most likely to predict repeat surgery and longer duration of surgical care. Forty-seven patients (5.2% of the NF1 patients' cohort) underwent 79 procedures with a 4.8 years average follow-up. Soft-tissue surgery had a high revision rate (19/45 patients), skeletal surgery did not (2/13 patients). Transosseous wire canthopexy and facial aesthetic unit remodeling were associated with stable outcome. Ptosis repair carried an unfavorable outcome, particularly in the presence of sphenoid dysplasia. Stable skeletal remodeling was achieved with polyethylene implants and/or cementoplasty. Multiple procedures were undertaken in 70% of patients and were predicted by the NF volume, canthopexy, skeletal dysplasia, or a Jackson's classification 2 and/or 3; but not by declining visual acuity. A classification based upon predictive risk of repeated procedures is proposed: Group 1: Isolated soft tissue infiltration not requiring levator palpebrae or canthal surgery; Group 2: Soft tissue involvement requiring ptosis repair or canthopexy, or NF great axis over 4.5 cm; Group 3: Presence of sphenoid dysplasia with pulsatile proptosis, regardless of visual acuity.
authors.affiliation: Department of Plastic Surgery, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris (APHP), PRES Sorbonne Paris Cité, Université Paris Descartes, Paris, France.
authors.forename: Rachel
authors.initials: R
authors.lastname: Pessis
authors.affiliation: Department of Plastic Surgery, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris (APHP), PRES Sorbonne Paris Cité, Université Paris Descartes, Paris, France; Neurofibromatosis National Reference Center, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Lantieri
authors.affiliation: Craniofacial Centre, Great Ormond Street Hospital for Children NHS Trust, London, UK.
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Britto
authors.affiliation: Neurofibromatosis National Reference Center, France; Department of Neurosurgery, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Est Creteil, Créteil, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Leguerinel
authors.affiliation: Département of Dermatologie, Hôpital Henri-Mondor, Assistance Publique-Hôpitaux de Paris (APHP) and EA 4393 LIC, Université Paris Est Créteil (UPEC), Créteil, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Wolkenstein
authors.affiliation: Department of Plastic Surgery, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris (APHP), PRES Sorbonne Paris Cité, Université Paris Descartes, Paris, France. Electronic address: mikael.hivelin@egp.aphp.fr.
authors.forename: Mikaël
authors.initials: M
authors.lastname: Hivelin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001763:Blepharoptosis / Q000601:surgery; D001843:Bone Cements / Q000627:therapeutic use; D015331:Cohort Studies; D004954:Esthetics; D005094:Exophthalmos / Q000601:surgery; D005142:Eyelid Neoplasms / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000601:surgery*; D009918:Orbital Neoplasms / Q000601:surgery*; D020959:Polyethylene / Q000737:chemistry; D019736:Prostheses and Implants; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D012878:Skin Neoplasms / Q000601:surgery; D012888:Skull Neoplasms / Q000601:surgery*; D013100:Sphenoid Bone / Q000473:pathology; D013701:Temporal Bone / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001843:Bone Cements; D020959:Polyethylene
keywords: Canthopexy; Neurofibromatosis 1 (NF1); Orbit; Polyethylene; Ptosis; Surgical revisions