Surgical treatment of colonic ganglioneuromatosis in neurofibromatosis.

Abstract

Gastrointestinal involvement in neurofibromatosis occurs in three forms: neurofibromatous tumours, visceral vasculopathy and ganglioneuromatosis. A case of colonic ganglioneuromatosis is reported and six similar cases from the literature are reviewed. The clinical and pathological features of this condition are summarized and guidelines for surgical therapy formulated. Subtotal colectomy is recommended for pseudo-obstructive symptoms refractory to medical management.