Outcomes of children with long-segment and total colon Hirschsprung disease following pull-through

Hirschsprung disease (HSCR) is a congenital disorder caused by the absence of ganglion cells, which leads to a functional obstruction in infants. HSCR is divided into short, long and total colon aganglionosis (TCA). However, post-operative outcome assessment of patients with long-segment and TCA is scarce. We determined the functional outcomes, Hirschsprung-associated enterocolitis (HAEC) and complications of long-segment and TCA HSCR's children following pull-through surgery.

Descriptive analysis research was done for children with HSCR long-segment and TCA who underwent an operation at our institutionfrom 2013 to 2020. We assessed the functional outcome and HAEC by the Krickenbeck and the HAEC scoring, respectively.

We ascertained 13 HSCR long-segment and six TCA. We performed the following surgical procedures: Duhamel (n=7), Martin (n=4), Kimura (n=1), transabdominal Yancey-Soave (n=3) and transanal endorectal pull-through (n=4). All long-segment patients revealed good functional outcomes, whereas two TCA children suffered soiling and failed to achieve voluntary bowel movement. HAEC was noted in three long-segment and four TCA patients. Furthermore, surgical site infection and diaper rash were noticed in 10 and two patients, respectively.

Long-segment patients might have better functional outcomes TCA group, whereas the frequency of HAEC is compatible among arms. Long-term follow-up is important and necessary to identify complications early and define the proper treatment. Our study comprehensively analyzes functional outcomes, HAEC and complications of children with HSCR long-segment and TCA after definitive surgery in a developing country.