Although intestinal neurofibromatosis associated with Recklinghausen's disease is a rare condition, it may cause severe gastrointestinal disturbances as a result of hemorrhage or perforation. Shaiken1 and Shaw2 have each reported a single case of exsanguinating gastrointestinal hemorrhage resulting from neurofibromata of the small intestine in Recklinghausen's disease. Warshauer and Nelson3 reported a case in which a neurofibroma in the jejunum perforated. Hartman4 reported a case of fibromyxosarcoma of the stomach in a patient with Recklinghausen's disease which he believed arose from a neurofibroma of the stomach wall. River et al.,5 in a collective review of the world literature on benign tumors of the small intestine, including 1,399 cases, found only 90 cases of neurogenic tumors of all types. In only 14 of these cases were there cutaneous neurofibromata (Recklinghausen's disease).
Report of a Case
A 50-year-old white man was admitted to the surgical service of
FARRAR T, CHAPPELL FW. Severe Gastrointestinal Hemorrhage Resulting from Recklinghausen's Disease. AMA Arch Surg. 1959;79(1):106-108. doi:10.1001/archsurg.1959.04320070110018