Adulthood hirschprung's disease : a report of 4 cases in Ile - Ife. Nigeria

Abstract

Background: Hirschsprung's disease in adulthood is very rare and is often misdiagnosed. We present four cases of adulthood Hirschsprung's disease seen in the last two decades to illustrate challenges accompanying its diagnosis and management. Method: This descriptive case series included cases of histologically proven Hirschsprung's seen in adulthood at the Obafemi Awolowo University Teaching Hospitals Complex in the last two decades (1991 - 2011). The clinical data, radiological investigations, details of surgical treatment, histological diagnosis, outcomes and complications were analyzed. Result: There were 4 adult patients, 3 males and 1 female with age ranging from 17 to 74 years (mean 23 years). Each patient presented with sub-acute intestinal obstruction needing two staged procedures of initial colostomy followed by definitive procedure of low anterior resection (State procedure) in 3 patients and Swenson - Bill procedure in one. There was one mortality and good long term outcome in the remaining three. Conclusion: This review presented the oldest patient presenting with adult Hirschsprung's and the highest mean age of any case series. Four patients with adulthood Hirschsprung's disease managed by two operative procedures enabled comparison of operative outcome with respect to complications and functional outcomes. Mortality seems to correlate with presentation at old age, which is usually due to lifelong self-management of chronic constipation. Though very rare, a high index of suspicion of adulthood Hirschprung's disease should be maintained in adult patients with recurrent chronic constipation needing lifelong laxative, enema or mechanical wash-out.