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

摘要

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.