Adult Onset Hirschsprung's Disease With Overlapping Presentation of Sigmoid Volvulus: Two Case Reports With Literature Review at Tikur Ambessa Specialized Hospital, AAU, Ethiopia

ABSTRACT Adult‐onset Hirschsprung's disease (HSD) is uncommon and frequently leads to diagnostic difficulty after infancy. It is a congenital illness that causes intestinal obstruction due to a lack of ganglion cells. Approximately 1 in 5000 live newborns have this illness, and 95% of HSD cases are identified in infants. This case report will fill a knowledge gap at a low‐resource setting where published papers on this topic are extremely rare, and will provide insight into diagnosis and surgical management options that are feasible in resource‐limited environments with good outcomes. In this case report, we present two cases of adult‐onset HSD having a history of persistent constipation that has been treated by laxatives for a long time, abdominal distention, and intermittent colicky pain. For one of the patients, exploratory laparotomy, sigmoid resection and colostomy was done with suspected diagnosis of sigmoid volvulus due to sign of obstruction. Despite this, the patient's clinical condition was not improved and was referred to our hospital for further work up and management. The patient was diagnosed with adult‐onset HSD at our hospital by biopsy from the rectum and colostomy site and managed as adult‐onset HSD. The other patient was deflated with a rectal tube with suspicion of sigmoid volvulus and later referred to our hospital. She was diagnosed with adult‐onset HSD by full‐thickness rectal biopsy after having a detailed history. She was surgically treated and discharged improved. A detailed history including childhood period was obtained, and a physical examination was performed. Basic investigations were done and within the normal range. Barium enema revealed a grossly distended proximal colon. Rectal biopsy was performed in all cases and showed aganglionosis at the stenotic segment. The Soave pull‐through procedure was performed along with a protective ileostomy. One procedure was laparoscopy assisted and the other one was open. Ileostomy reversal was performed later. In both cases, patient symptoms were completely resolved and had an uneventful condition at one‐year follow‐up at our hospital. Generally, adult HSD should be considered in the differential diagnosis of adult patients with chronic constipation or even acute bowel obstruction with a chronic background of constipation. Full‐thickness rectal biopsy is confirmatory for diagnosis. The Soave pull‐through procedure with protective ileostomy is a feasible surgical option for treating adult HSD.