Craniofacial Neurofibromatosis: Treatment of the midface deformity

Dhruv Singhal, Yi Chieh Chen, Yueh Ju Tsai, Chung Chih Yu, Hung Chang Chen, Yu Ray Chen, Philip Kuo Ting Chen

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)595-600
Number of pages6
JournalJournal of Cranio-Maxillofacial Surgery
Volume42
Issue number5
DOIs
Publication statusPublished - Jan 1 2014
Externally publishedYes

Keywords

  • Craniofacial Neurofibromatosis
  • Forehead flap
  • Midface

ASJC Scopus subject areas

  • Surgery
  • Oral Surgery
  • Otorhinolaryngology

Fingerprint Dive into the research topics of 'Craniofacial Neurofibromatosis: Treatment of the midface deformity'. Together they form a unique fingerprint.

Cite this