Isolated diffuse intestinal ganglioneuromatosis presented as a redundant sigmoid colon: a case report

Abstract

Introduction and Importance: Diffuse intestinal ganglioneuromtosis is a benign tumor of the enteric nervous system, that almost always occurs in children with systemic syndromes. Whereas isolated cases in adults are exceedingly rare. Case Presentation: A 38-year-old man presented with refractory chronic constipation. An abdominal computed tomography scan revealed a redundant sigmoid colon, then he underwent a sigmoid colectomy. Histopathologic examination showed diffuse ganglioneuromatosis. However, the patient was in good health 18 months after surgery. Clinical Discussion: Intestinal ganglioneuromas commonly occur in children with systemic syndromes such as multiple endocrine neoplasia type 2B and neurofibromatosis type 1. The most frequent symptoms are abdominal discomfort, constipation, ileus, weight loss, appendicitis, and obstruction in more severe cases. surgical resection is the standard management in diffuse ganglioneuromatosis. Conclusion: Although diffuse ganglioneuromatosis is uncommon, it should be considered in patients with refractory constipation.

Ganglioneuromatous polyposis associated with type 2 B multiple endocrine neoplasia (MEN 2B) – case report

“…O'Riordain et al performed a study involving patients with MEN 2B syndrome in which 90% of participants had colonic disturbances, mostly constipation, and all of them had MTC [29]. Ganglioneuromas, especially in the colon, is rare among adults [30,31,32,33]. The diagnosis of ganglioneuromas is based on histopathological analysis, such as a biopsy stained with haematoxylin and eosin, which would show spindle and ganglion cells [23,30].…”