Functional outcome of Hirschsprung's disease in children: A single center study at The Children's Hospital Lahore

Aim Hirschsprung's disease (HD) is a congenital bowel disorder resulting in functional dysmotility. Advancement in surgical techniques has improved outcomes, but recent studies have identified problems having significant impact on patient's quality of life. The aim of this study is to analyze postoperative functional outcome after definitive surgery for Hirschsprung's disease. Method A retrospective study was conducted at the Department of Pediatric Surgery, The Children's Hospital Lahore. The record of 64 patients was reviewed from the year 2009 to 2019. Parents were interviewed on phone calls and a predesigned questionnaire was filled. Degree of constipation, incontinence and voluntary bowel movements was analyzed according to Krickenbeck classification. Results Median age at initial presentation was 11 days, with male to female ratio of 3.9:1. Most common presentation was delayed passage of meconium (38%). Diagnosis was made on biopsy in 70.3%, but contrast enema and biopsy in 29.7%. Aganglionosis was limited to short segment in 81.3% and long segment in 14.5%. Decompressing enterostomy was made in 63% at a mean age of 191 days. Definitive surgery included Duhamel pull-through in 89.1%. Mean age at follow-up was 10.6 +/- 4.19 years and alive patients were 95.4%. Postoperative enterocolitis was reported by 27%, while 35.5% reported fecal incontinence and 16.1% complained of constipation. 80% could voluntarily hold bowel movement, among which 5% had a feeling of urge and 15% had the capacity to verbalize. In 25% of the patients, fecal incontinence was a constant social problem and in 25% of these patients constipation was resistant to diet and laxatives. Conclusion On long-term follow-up, fecal incontinence was more worrisome, affecting quality of life and requiring help of the bowel management program. Such patients need close follow-up after definitive procedure, so that they have normal stooling habits in their adulthood and lead a normal life.