Hirschsprung’s Disease in Patients of Advanced Age

Abstract

Hirschsprung’s disease is a congenital motility disorder that is easily overlooked as a cause of chronic refractory constipation in adults. We present a case of Hirschsprung’s disease in a patient 70 years of age with a history of long-standing constipation, chronic use of laxatives, and recurrent episodes of colonic obstruction. Presumptive preoperative diagnosis was chronic ischemic sigmoid stenosis or intermittent sigmoid volvulus before Hirschsprung’s disease was suspected based on intraoperative colonoscopy and surgical findings. The diagnosis was confirmed by the absence of intrinsic ganglion cells on histopathologic examination of the surgical specimen and the absence of the rectoanal inhibitory reflex on postoperative manometry. A conservative surgical approach that limited the resection to the grossly diseased rectum successfully restored normal defecation despite the anastomosis being performed on the distal aganglionic rectum. This approach, which avoids extensive rectal dissection, may be suitable for older or frail patients. Heightened awareness of Hirschsprung’s disease is necessary to ensure its prompt recognition in the elderly.