Problems and outcome of Hirschsprung's disease presenting after 1 year of age in a developing country.

Abstract

The purpose of the present study was to evaluate the problems, treatment outcome, and contributory factors to delayed presentation in Hirschsprung's disease (HD) after 1 year of age in a resource-limited setting. This retrospective study included 41 children aged >1 year with HD managed at the University of Nigeria Teaching Hospital, Enugu, in south eastern Nigeria, between January 2000 and June 2009. Complications of HD were evident at presentation in 38 (92.7%) of the 41 children. Late presentation was due to delayed referral in 27 cases (65.9%), parental ignorance in 11 (26.8%), and poverty in 3 (7.3%). The HD was rectosigmoid in 33 patients (80.5%) and was of ultra-short length variety in 8 patients (19.5%). Thirty-five patients (85.4%) required colostomy for decompression, and colostomy-related complications occurred in 24 of them (68.6%). The definitive surgical procedure was a Swenson pullthrough in 34 cases (82.9%) and posterior myectomy in 7 others (17.1%). Twenty patients (48.8%) experienced at least one postoperative complication. After follow-up of 7-64 months (mean: 31 months), 31 (75.6%) patients had a good outcome, 6 (14.6%) had persistent constipation, 3 had (7.3%) incontinence, and one child (2.4%) died from overwhelming infection. Hirschsprung's disease presenting after 1 year of age may be associated with high colostomy rates and increased morbidity. Continued dissemination of updated information on HD to medical practitioners and a public awareness campaign may improve time to diagnosis.