title: Craniofacial neurofibromatosis: treatment of the midface deformity.
abstract: Craniofacial Neurofibromatosis is a benign but devastating disease. While the most common location of facial involvement is the orbito-temporal region, patients often present with significant mid-face deformities. We reviewed our experience with Craniofacial Neurofibromatosis from June 1981 to June 2011 and included patients with midface soft tissue deformities defined as gross alteration of nasal or upper lip symmetry. Data reviewed included the medical records and photobank. Over 30 years, 52 patients presented to and underwent surgical management for Craniofacial Neurofibromatosis at the Chang Gung Craniofacial Center. 23 patients (43%) demonstrated gross mid-facial deformities at initial evaluation. 55% of patients with lip deformities and 28% of patients with nasal deformities demonstrated no direct tumour involvement. The respective deformity was solely due to secondary gravitational effects from neurofibromas of the cheek subunit. Primary tumour infiltration of the nasal and/or labial subunits was treated with excision followed by various methods of reconstruction including lower lateral cartilage repositioning, forehead flaps, free flaps, and/or oral commissure suspension. Soft tissue deformities of the midface are very common in patients with Craniofacial Neurofibromatosis and profoundly affect overall aesthetic outcomes. Distinguishing primary from secondary involvement of the midface assists in surgical decision making.
authors.affiliation: Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
authors.forename: Dhruv
authors.initials: D
authors.lastname: Singhal
authors.affiliation: Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
authors.forename: Yi-Chieh
authors.initials: YC
authors.lastname: Chen
authors.affiliation: Department of Ophthalmology, Chang Gung Memorial Hospital, Taiwan.
authors.forename: Yueh-Ju
authors.initials: YJ
authors.lastname: Tsai
authors.affiliation: Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
authors.forename: Chung-Chih
authors.initials: CC
authors.lastname: Yu
authors.affiliation: Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
authors.forename: Hung Chang
authors.initials: HC
authors.lastname: Chen
authors.affiliation: Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
authors.forename: Yu-Ray
authors.initials: YR
authors.lastname: Chen
authors.affiliation: Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taiwan. Electronic address: philip.ktchen@gmail.com.
authors.forename: Philip Kuo-Ting
authors.initials: PK
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D000328:Adult; D002610:Cheek / Q000601:surgery; D004954:Esthetics; D005143:Eyelids / Q000601:surgery; D005147:Facial Bones / Q000601:surgery*; D005153:Facial Neoplasms / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D058752:Free Tissue Flaps / Q000637:transplantation; D006801:Humans; D008046:Lip / Q000601:surgery; D008297:Male; D055171:Nasal Cartilages / Q000601:surgery; D009361:Neoplasm Invasiveness; D017253:Neurofibromatoses / Q000601:surgery*; D009667:Nose Deformities, Acquired / Q000601:surgery; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D012888:Skull Neoplasms / Q000601:surgery*; D012983:Soft Tissue Neoplasms / Q000601:surgery*; D013524:Surgical Flaps / Q000601:surgery; D055815:Young Adult
keywords: Craniofacial Neurofibromatosis; Forehead flap; Midface