Comprehensive and Global Approach of Soft-Tissue Deformities in Craniofacial Neurofibromatosis Type 1.

Abstract

To present a single-institution experience in the comprehensive and global soft-tissue surgical approach of patients with craniofacial neurofibromatosis type 1 (NF-1). A retrospective analysis of patients with craniofacial NF-1 (n = 20) who underwent craniofacial soft-tissue reconstruction between 1993 and 2014 was conducted. Surgical treatment was individualized according to age, functional and/or aesthetic impairment, neurofibroma types, anatomical location, size, and patient/family and surgical team preferences, regardless of previously published compartmental grading systems. The surgical results were classified based on 2 previously published outcome rating scales (craniofacial symmetry improvement and need for additional surgery). All patients underwent en bloc translesional surgical excisions, 12 facial suspension, 3 eyebrow suspension, 2 ear suspension, 9 lateral canthopexy, 5 horizontal shortening of the tarsus of lower eyelid, and 1 horizontal shortening of the tarsus of upper eyelid. The degree of craniofacial symmetry improvement was considered "mostly satisfactory" (75%), and the overall rate of surgical results ranked according to the need for additional surgery was 2.4 ± 0.50, with variations according to the spectrum of soft-tissue involvement. According to the experience and surgical outcomes presented in this study, the soft-tissue surgical approach of the craniofacial NF-1 should be global, comprehensive, and individualized.