Hirschsprung's disease in children with Down syndrome: a comparative study

Abstract

Background and aim: There is controversy in the literature regarding the outcome after surgical treatment of Hirschsprung's disease in children with Down syndrome (DS). The aim of this study was to compare the outcome of our series of DS children with Hirschsprung's disease to our series of children without Down syndrome (NDS) with Hirschsprung's disease. The impact of laparoscopy within the DS group was analyzed.

Material and methods: Between March 1987 and August 2008, 149 children were operated on for Hirschsprung's disease. 20 children of this group were additionally diagnosed with Down syndrome. All children underwent either an open or a laparoscopic Duhamel procedure. We evaluated postoperative hospital stay, short-term complications and the incidence of enterocolitis, constipation and incontinence.

Results: 20 patients (13.4%) in this series had Down syndrome. There were no significant differences in the extent of aganglionosis between children with or without Down syndrome. There were no intra-operative complications and no conversions. Postoperative leak occurred significantly more often in children with DS (n=5, 25%) compared to NDS children (n=1, 0.7%; p<0.0001). Postoperative leakage-related abscess formation was higher in the DS group (n=3, 15%) compared to the NDS group (0%). Within the DS group there was no significant difference between open or laparoscopic Duhamel procedure with regard to these postoperative complications. Postoperative hospital stay was significantly longer in the DS group compared to the NDS group (p<0.05). In the DS group there was a slightly shorter postoperative stay after laparoscopic Duhamel procedure. Mean long-term follow-up was 5.1 years. One death occurred in the DS group 9 months postoperatively due to sepsis and cardiomyopathy. Severe constipation was present significantly more often in DS children (n=11, 55%) compared to NDS children (n=29, 22.3%; p<0.01). There was no difference in incontinence between DS and NDS children. Enterocolitis occurred more frequently in DS patients after operation (40 [31% NDS] vs. 9 [45% DS]; p=0.038).

Conclusion: Compared to NDS children, children with DS have a higher rate of postoperative complications and a longer hospital stay. During long-term follow-up most patients with DS are severely constipated and have a higher incidence of enterocolitis.