Neurofibromatosis Type 1 or Von Recklinghausen's Disease

Abstract

Introduction: Von Recklinghausen's disease or neurofibromatosis type I is a autosomal dominant inheritance condition with varied clinical expressions, its manifestations range from café-au-lait-type spots on the skin to severe aesthetic and functional complications that can also affect other organs, bone and nerve tissues.

Objective: To preport the aesthetic and functional results achieved with the surgical intervention of a case with neurofibromatosis type I with large lesions.

Clinical case report: We report a case treated in Gaborone, Republic of Botswana by a multidisciplinary team including a plastic surgeon, a neurosurgeon and a pathologist, all from the Republic of Cuba. The patient was treated, on that occasion, for skin neurofibromas and she reported a family history of the disease. Bone changes were found due to an exaggerated syphosis and with no intellect damage. She underwent surgery for a major back injury.

Conclusions: The aesthetic and functional results of surgical treatment in extensive lesions, in the course of neurofibromatosis, can be successful if correct assessment is carried out by a multidisciplinary group. The group can choose the most appropriate surgical treatment regardless of the size of the lesion.