Giant Megacolon in Aganglionic Colon Complicated With Bilateral Hydronephrosis

Abstract

Hirschsprung Disease (HSCR) is a congenital disorder resulting from the failure of migration of the neural crest cells to distal intestine leading to functional obstruction of affected aganglionic bowel segment. The incidence of HSCR is approximately 1:5000 live births. Here, we present a -20-year-old HSCR male with giant megacolon complicated with bilateral hydronephrosis. A 20 -year- old male with past medical history of HSCR, status post bowel resection, Botox injection and multiple fecal dis-impactions under anesthesia in recent year presented to the emergency department with 5-6 episodes of bilious vomiting for one day, no flatus for a week and no bowel movements for a month. It was associated with acute onset of severe generalized constant vague abdomen pain. He denies fever, nausea, vomiting, overflow diarrhea or urinary incontinence. He was not compliance with his bowel regimens for almost 2 months and failed to follow up with HSCR specialist. After recent histological report showed aganglionosis, surgical intervention was recommended but he deferred. On physical examination of abdomen disclosed obvious abdomen distension, tympanic on percussion, decreased bowel sounds but no signs of peritonitis. Labs were within normal references. XR (abdomen) showed massive amount of retained stool with no intra-abdominal free air(Figure-1 and 2). CT scan (abdomen and pelvis) showed massive constipation/fecal impaction. Obstructive uropathy pattern was seen possibly related to extrinsic mass effect due to constipation(Figure-3). After fecal dis-impactions under anesthesia for 3 times, the patient can pass flatus followed by normal well- formed stool in 2 days. The patient was educated for medication compliance for bowel regimens and recommended for surgical intervention in the near- future during follow up. Giant megacolon is a potentially life-threatening conditions in the patients with HSCR. It can present with the triad of constipation, serve abdomen pain, and progressive abdomen distension. In our patient, the recurrence of such conditions results from non-compliance with bowel regimens;deferred from surgical intervention and subsequently complicated with obstructive uropathy due to extrinsic mass effect from chronic constipation.2978_A Figure 1. Figure-1: XR (abdomen) ( Supine) showed Massive amount of retained stool consistent with history of Hirschsprung disease. No free intraabdominal air.2978_B Figure 2. XR (abdomen )(Erect view) showed Massive amount of retained stool consistent with history of Hirschsprung disease. No free intra-abdominal air.2978_C Figure 3. CT scan (abdomen and pelvis) showed abnormal colonic distension pattern with large volume feculent material consistent with constipation/fecal impaction. There is no evidence for bowel perforation. Obstructive uropathy pattern was seen due to extrinsic mass effect from chronic constipation in setting of Hirschsprung disease.