Functional Outcomes of Hirschsprung's Disease in Children - A Single Center Study Done 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 post-operative functional outcome after definitive surgery for Hirschsprung’s disease. Method: A retrospective study was conducted in the department of Pediatric Surgery, The Children's Hospital Lahore. The record of 64 patients was reviewed from the year 2009 - 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%, while contrast enema and biopsy in 29.7 %. Aganglionosis was limited to short segment in 81.3% & long segment in 14.5%. Decompressing enterostomy was made in 63% at mean age of 191 days. Definitive surgery included Duhamel pull through in 89.1%. The mean age at follow up was 10.6±4.19 years and alive patients are 95.4%. Post-operative enterocolitis was reported by 27%, while 35.5% reported fecal incontinence and 16.1% had complained of constipation. For voluntary bowel movements, 80% could hold bowel movement, among which 5% with feeling of urge and 15% had the capacity to verbalize. In 25 % of 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 requiring help of 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.