Craniofacial neurofibromatosis and tissue expansion: long-term results

Abstract

The role of tissue expansion for coverage of soft-tissue defects of the face following neurofibroma resection has been briefly described previously with good results but limited follow-up. Recent scientific evidence has arisen that neurofibromas adversely affect the rheological properties of normal surrounding skin resulting in hyper-extensibility of dermal elements. In this context, we believe that long-term outcomes of tissue expansion for treatment of soft-tissue defects in patients with craniofacial neurofibromatosis would demonstrate suboptimal results because of loss of contour. Between June 1981 and June 2011, two patients underwent five tissue expander placements during the course of treatment for craniofacial neurofibromatosis at our institution. In both patients, tissue expansion was used to recruit tissue for fascio-cutaneous flaps following radical excision of adjacent neurofibromas. Three cervical expansions were performed for cheek and lateral face recontouring. One forehead expansion was used for nasal reconstruction and a second forehead expansion was performed for temporo-orbital recontouring. No perioperative complications occurred related to the tissue expander placement, expansions or radical excisions. Photographic follow-up of 15 years is available in each case. Cervical tissue expanded to provide cheek coverage demonstrated early loss of contour. Loss of lateral cheek contour resulted in increased morbidity from lateral and caudal displacement of the mid-face and orbital tissues requiring multiple revisional procedures. Tissue expansion of the forehead for both nasal reconstruction and temporo-orbital reconstruction demonstrated excellent long-lasting results.