Intestinal Neurofibromatosis



Hakami M¹ ; Mosavy SH¹ ; Juzdani M¹ ; Vakhshuri P¹ Show Affiliations
Source: Journal of Abdominal Surgery Published:1975

Abstract

Neurofibromatosis (Von Recklinghausen's disease) is characterized by multiple neurofibromas that arise in the trunks of the spinal, cranial, and sympathetic nerves, usually associated with the presence of cutaneous fibromas, areas of skin pigmentation, abnormalities of the bones, and varying malformations. This is an inherited disease (Mendelian dominant) and the tumors arise from the neurilemmal sheath (Schwann cells) and fibroblasts of the peripheral nerve. Neurofibromatosis is not commonly considered in the differential diagnosis of intestinal lesion, and if intestinal involvement does occur in Von Recklinghausen's disease, it is most commonly seen within the small bowel. The occurrence of rather massive colonic neurofibromatosis causing intestinal intussusception in a 44 yr old man with cutaneous Von Recklinghausen's disease forms the base of this report.