Problems and Outcome of Hirschsprung’s Disease Presenting after 1 Year of Age in a Developing Country

• Published in: World journal of surgery Vol. 35; no. 1; pp. 22 - 26
• Main Authors: Ekenze, S. O., Ngaikedi, C., Obasi, A. A.
• Format: Journal Article
• Language: English
• Published: New York Springer-Verlag 2011; Springer‐Verlag; Springer; Springer Nature B.V
• Subjects: Abdominal Surgery; Adolescent; Biological and medical sciences; Cardiac Surgery; Child; Child, Preschool; Colostomy; Colostomy Closure; Decompression, Surgical; Female; Gastroenterology. Liver. Pancreas. Abdomen; General aspects; General Surgery; Hirschsprung Disease - complications; Hirschsprung Disease - epidemiology; Hirschsprung Disease - surgery; Humans; Infant; Male; Malformations; Medical sciences; Medicine; Medicine & Public Health; Nigeria - epidemiology; Nigeria Teaching Hospital; Postoperative Complications; Rectal Washout; Retrospective Studies; Risk Factors; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Surgery; Tertiary Health Facility; Thoracic Surgery; Toxic Megacolon; Treatment Outcome; Vascular Surgery; Nigeria; Colostomy Closure; Tertiary Health Facility; Nigeria Teaching Hospital; Rectal Washout; Toxic Megacolon; Nervous system diseases; Prognosis; Neuronal intestinal malformation; Developing countries; Congenital disease; Colonic disease; Medicine; Treatment; Surgery; Hirschsprung disease; Digestive diseases; Intestinal disease; Evolution; Age
• DOI: 10.1007/s00268-010-0828-2
• ISSN: 0364-2313

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 Hirschprung’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.