Diffuse Intestinal Ganglioneuromatosis in an Infant - A Case Report

Dr. Gopika G Nair, Dr. Laila Raji N

Abstract: Intestinal diffuse ganglioneuromatosis is a rare, benign, neoplastic condition arising from the enteric nervous system. This is a case of 2-month-old female baby, who presented with abdominal distention and decreased stool passage and suspected to have hirschsprung disease. Patient underwent ileostomy with multiple seromuscular biopsies, on histopathological examination and immunohistochemistry found to have Intestinal diffuse ganglioneuromatosis.

Keywords: Ganglioneuromatosis, MEN 2B, NF1, ganglion cells