Hypoganglionosis and Isolated Crohn’s Disease of the Terminal Ileum Presenting with Perforation Mimicking Appendicular Mass in a 72-Year-Old Asian Lady

Abstract

A very unusual and interesting case of a 72-year-old Indian female with a background of chronic constipation since early infancy presented in emergency critical condition with acute perforative peritonitis and right lower quadrant fullness, guarding and tenderness. Laboratory studies showed high inflammatory markers and provisional diagnosis of walled off appendicular perforation was made. Radiographs and multi scanner computed tomography (MSCT) was found to have pneumoperitoneum secondary to the terminal ileal stricture with perforation. The patient underwent emergency exploratory laparotomy following initial resuscitation, resection and anastomosis of the ileal stricture, peritoneal toilet and extramucosal biopsies of rectosigmoid colon were performed. Histopathology confirmed isolated Crohn’s disease of terminal ileum with stricture and associated hypoganglionosis. Subsequent colonoscopy and biopsies showed no active Crohn’s disease but had nonspecific colitis of the left colon due to associated hypoganglionosis. The postoperative period was uneventful. The patient is doing well at follow up with conservative management of the hypoganglionosis with laxatives, dietary changes, life style modifications and dietary supplements.