A systematic multiple stage surgical approach for attainment of satisfactory and favourable surgical results in an extremely severe von Recklinghausen's disease, elephantiasis neurofibromatosa

SummaryThis disease was first described by von Recklinghausen and Festscher and has been known as the von Recklinghausen's disease or neurofibromatosis (NF). Numerous articles have been published on this subject where majority of the authors have stressed their difficulty in achieving favourable surgical results. This disease is pathologically accepted to be of neuroectodermal origin with a positive family history in approximately 50% of the cases, autosomal dominant trait and is known to involve the periorbital regions, orbit (preoperative CT scan: upper left and right photographs), temporal region to a variable extent, mid-facial region to the mandibular region. The indicated treatment for this disease is surgery including cranio-maxillofacial surgery even though re-evaluation of the conventional methods of surgery should be considered. This particular case is an extremely difficult and challenging case for any reconstructive plastic surgeon and required extensive preoperative planning. The systematic multiple stage surgical approach for an extremely severe von Recklinghausen's disease, elephantiasis neurofibromatosa (Fig. 1) is presented and discussed in detail.