Pheochromocytoma and intestinal ganglioneuromatosis are rare entities, associated with Multiple Endocrine Neoplasia Type 2B (MEN2B), but rarely associated with colonic pseudo-obstruction and ischemia. We report a 52-year-old patient who presented with colonic perforation and was later found to have all 3 pathologies concurrently. An exploratory laparotomy revealed toxic megacolon with rectosigmoid perforation requiring a total abdominal colectomy. Diffuse intestinal ganglioneuromatosis with no malignancy was reported by histopathology. Perioperative challenges included severe acidosis (pH 6.9) and supraventricular tachycardia requiring electrical and chemical cardioversion. Despite this, the patient was successfully managed with minimal vasopressor support (likely due to endogenous catecholamine release from his pheochromocytoma). This case of gastrointestinal emergency underlines the need for early recognition of comorbidities associated with MEN2B, and the importance of multidisciplinary care. This is the first case report describing bowel perforation in the context of MEN2B, pheochromocytoma, and intestinal ganglioneuromatosis.

Citations

• Megacolon Associated with Multiple Endocrine Neoplasia Type 2B: A Case Successfully Managed with Ileostomy
  Takeshi Ikeda, Nao Hondo, Masato Kitazawa, Satoshi Nakamura, Yuta Yamamoto, Satoru Miyazaki, Masahiro Kataoka, Hirokazu Tanaka, Naoki Ishizaka, Tadaaki Shimizu, Masatsugu Kuroiwa, Naoya Yamamoto, Yuji Soejima
  Surgical Case Reports.2026; 12(1): n/a.     CrossRef

Gallstone ileus is rare complication of cholecystitis or cholelithiasis due to the formation of an aberrant communication between the gallbladder and small bowel. The movement of the gallstone to the small bowel may cause bowel obstruction. A patient presented with acute onset abdominal pain, nausea, vomiting, and tenderness around the umbilical area. Physical exam, laboratory tests, and computed tomography were performed. Within 24 hours the patient had developed peritonitis. He received an emergency exploratory laparotomy, and small bowel resection and anastomosis of the proximal jejunum. The patient had gallstone ileus complicated by the presence of an intraluminal abscess and perforation due to pressure necrosis caused by the gallstone. The patient recovered and was discharged on postoperative day 31. Despite having had a cholecystectomy 20 years prior this case highlights a patient who suffered a rare case of gallstone ileus.