• J. Pediatr. Surg. · Nov 2019

    Observational Study

    The Extent of the Transition Zone in Hirschsprung Disease.

    • David Coyle, Anne Marie O'Donnell, Christian Tomuschat, John Gillick, and Prem Puri.
    • Dept. of Paediatric Surgery, Temple Street Children's University Hospital, Dublin, Ireland; National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland. Electronic address: davidcoyle@rcsi.ie.
    • J. Pediatr. Surg. 2019 Nov 1; 54 (11): 2318-2324.

    BackgroundRetained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease.ObjectiveWe aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease.DesignWe performed an observational study. DAB+ immunohistochemistry for Protein Gene Product 9.5 was used to evaluate the neuronal networks in serial sections of pull-through specimens obtained from children with Hirschsprung's disease (n = 12). Reference ranges for ganglion size/density and nerve trunk diameter were statistically determined using healthy controls obtained from colostomy specimens from children with anorectal malformations (n = 8). The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis.ResultsThe mean submucosal nerve trunk diameter in controls was 19.56 μm +/- 3.87 μm. The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). The median length of the transition zone across the population was 8 cm (4-22 cm). Median transition zone extent was significantly longer in patients with long-segment aganglionosis (n = 6) compared to rectosigmoid aganglionosis (n = 6, 13 cm vs 6 cm, p = 0.041). Due to the age of the patients enrolled, long-term follow-up of bowel function is not yet available.ConclusionOur data suggest that, in children with rectosigmoid Hirschsprung's disease, the transition zone can extend for up to 13 cm. In children with long-segment disease, a longer transition zone is possible. Extended resection at a minimum 5 cm beyond the most distal ganglionic intra-operative biopsy and intra-operative histological examination of the proximal resection margin are required to minimize transition zone pull-through.Level Of Evidence2.Copyright © 2019 Elsevier Inc. All rights reserved.

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