Presentation and Management of Hirschsprung's Disease in Infants at A Tertiary Care Centre and Outcome of Primary Pull-Through in These Patients.

Abstract

Hirschsprung's disease is a common cause of neonatal intestinal obstruction. The treatment of Hirschsprung's disease has evolved to a single stage pull-through. . The aim of study was to evaluate the feasibility of performing the primary pull-through surgery and to develop an optimum treatment protocol for patients of Hirschsprung's disease (HD) presenting to our institution. It was a prospective cohort study of newborn and young infants with features of Hirschsprung's disease conducted in our department from Oct. 2014, till Oct. 2018. The diagnosis was based on history, clinical examination, water soluble contrast enema and a definitive rectal biopsy. Transition zone was confirmed by intra-operative frozen section. Patients with weight of more than 5kgs and absence of features of concurrent enterocolitis, were taken for surgery. Patients included in the study underwent primary pull-through by different methods were put on strict follow-up protocol for 24 months and results were evaluated. Out of 40 patients of Hirschsprung's disease presented to SKIMS from Oct. 2014 till Oct. 2017,only 25 patients underwent primary pull-through. Mean age of patients at the time of pull-through was6.9±7.12 months and mean weight was 8.76 ±2.67kg. Delayed passage of meconium was seen in23 patients (92%). Preoperative rectal biopsy was done for confirmation of diagnosis and intra-operative frozen section for delineation level of aganglionosis. Laparoscopic transanal pull-through was most common operation done in 13 (52%) patients. Anastomotic leak was seen in 2(8%) patients, wound infection occurred 2(8%) patients, enterocolitis in1(4%) and constipation in 2 (8%) patients. One patient who had undergone trans-anal pull-through underwent a redo for a retained cuff. Primary pull-through is feasible and safe option with excellent results in neonates and infants with Hirschsprungs disease.