부산대학교 도서관

Background Hirschsprung's disease (HD) is the commonest cause of functional intestinal obstruction in children and poses challenges to pediatricians and pediatric surgeons practicing in resourcelimited countries. This study describes the clinical characteristics and outcome of management of this disease in our setting and highlights challenges associated with the care of these patients and proffer solutions for improved outcome. Methods This was a descriptive prospective study of children aged ⩽ 10 years who were histologically diagnosed and treated for HD at our centre between July 2008 and June 2013. Results A total of 110 patients (M: F ratio= 3.6:1) with a median age of 24 months were studied. Six (5.5%) patients were in the neonatal period. Sixty-four (58.2%) patients had complete intestinal obstruction whereas 42 (38.2%) and 4 (3.6%) patients had chronic intestinal obstruction and intestinal perforation respectively. No patient had enterocolitis. Constipation (94.5%) was the most common complaints. 109 (99.1%) patients had colostomy prior to the definitive pull-through. The median duration of colostomy before definitive pull-through was 4 months. The majority of patients (67.3%) had short segment of aganglionosis localized to the recto-sigmoid region. The definitive pull-through was performed in 94 (85.5%) patients (Swenson's pull-through 76 (80.9%), Duhamel's pull-through (12.8%) and Soave's pullthrough 4 (4.3%) patients). Postoperative complication rate was 47.3%. The median length of hospital stay was 26 days. Patients who developed complications stayed longer in the hospital and this was statistically significant (p <0.001). Mortality rate was 21.8%. The age < 4 weeks, delayed presentation and surgical site infection were the main predictors of mortality (p < 0.001). During the follow-up period, the results of Swenson's and Duhamel's pull through procedures were generally good in 87.8% and 42.9% of patients respectively. The result of Soave's procedures was generally poor in this study. Conclusion HD remains the commonest cause of functional intestinal obstruction in children and contributes significantly to high morbidity and mortality in our setting. The majority of patients present late when the disease becomes complicated. Early diagnosis and timely definitive pull through procedure are essential in order to decrease the morbidity and mortality associated with this disease. [ABSTRACT FROM AUTHOR]