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  1. Wardhani AK, Dwiantama K, Iskandar K, Yunus J, Gunadi
    Med J Malaysia, 2024 Aug;79(Suppl 4):1-5.
    PMID: 39215407
    INTRODUCTION: 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.

    MATERIALS AND METHODS: 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.

    RESULTS: 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.

    CONCLUSION: 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.

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