Hirschsprung’s disease in children: a five year experience at a University teaching hospital in northwestern Tanzania

Abstract

BackgroundHirschsprung’s disease (HD) is the commonest cause of functional intestinal obstruction in children and poses challenges to pediatricians and pediatric surgeons practicing in resource-limited 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.MethodsThis 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.ResultsA 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 pull-through 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.ConclusionHD 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.

Complications of Swenson’s Procedure in Hirschsprung’s Disease- A Hospital Based 5 Year Study

“…Mabula et al have reported their majority of patients after one year of life. [13] This study also showed that males were more afflicted by the disease as reported in the literature. [14] In our study we found males to be affected more than females.…”

Profile of Hirschsprung’s disease cases diagnosed in tertiary care teaching hospital: a retrospective observational study

“…Early diagnosis will help in decreasing morbidity and mortality associated with the disease. 1 This retrospective observational study on HD cases diagnosed in a tertiary care teaching hospital was conducted to develop a profile for Hirschsprung's disease in Indian population based on insights about its incidence in different age groups and gender distribution and its diagnosis by biopsy. This profile will help in the diagnosis of HD in adults which is often missed.…”

“…Hirschsprung's disease is a developmental defect of the enteric nervous system. 1 It is one of the frequent causes of intestinal obstruction in newborns, secondary to gut motility disorder. It has features of complex genetic heterogeneous disorder with variable inheritance.…”

Early and long-term complications following transanal pull through Soave technique in infants with Hirschsprung's diseaseric infants with Hirschsprung's disease

“…In the pathogenesis of HD, the migration arrest of enteric neural crest cells (ENCCs) is prominent, leading to the failure of reaching to the hindgut during embryogenesis. A total of 98% of neonates pass meconium within 24-48 hours after birth, while this fails in patients with HD [21]. There are various techniques for managing the disease.…”