Defecation disorders after surgery for Hirschsprung's disease in children; an Iranian experience

Abstract

Hirschsprung's disease (HD) is a congenital disease of the intestinal nervous system characterized by absence of ganglionic cells in distal colon followed by functional obstruction. After corrective surgery, the majority of children with HD develop defecation disorders such as soiling, constipation, fecal incontinence, and/or enterocolitis. The aim of this investigation was to determine the prevalence, diagnoses, therapies, and 6-month clinical outcomes in children with HD after corrective surgery. In this cross-sectional study performed at pediatric surgery ward in Tabriz Children's Hospital, prevalence, diagnosis and treatment of defecation disorders after surgery for HD were studied. First, defecation pattern was determined in 230 HD patients who had undergone surgery within previous ten years. Later, thirty patients with severe defecation disorders were recruited. Diagnostic and therapeutic procedures were performed. Clinical outcome was evaluated after six months. Defecation pattern was normal in 65% of the patients. In 21% of the cases, defecation disorders were mild and negligible, with no need of treatment. In 13% (30 patients), the pattern was impaired. Soiling, constipation, fecal incontinence and enterocolitis were the postoperative disorders. Of 30 patients with defecation disorders, 18 children (60%) and 12 patients (40%) had undergone multi-stage and TOSEPT surgical procedures, respectively. Defecation disorder was developed in 25.3% and 7.7% of the patients underwent multi-stage and transanal one-stage endorectal pull-through (TOSEPT) surgical procedures, respectively. Twelve patients and 10 children were treated with reoperation and medical therapy, respectively. Clinical outcome was excellent in five patients (16.7%), good in 15 patients (50%), fair in 8 patients (26.7%), and poor in 2 patients (6.6%). In conclusion, majority of the children with HD and postoperative defecation disorders have a favorable long-term clinical outcome when treated with minimally invasive surgical methods such as TOSEPT. Moreover, postoperative defecation disorders can be successfully treated using surgical procedures, medical therapy, as well as teaching both parents and their children.