The Extent of the Transition Zone in Hirschsprung Disease

      Abstract

      Background

      Retained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease.

      Objective

      We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease.

      Design

      We 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.

      Results

      The 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.

      Conclusion

      Our 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 Evidence

      2.

      Key words

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      References

        • Holschneider A.M.
        • Puri P.
        Hirschsprung's disease and allied disorders.
        3rd ed. Springer Verlag, Berlin Heidelberg2008
        • Kapur R.P.
        • Kennedy A.J.
        Transitional zone pull through: surgical pathology considerations.
        Semin Pediatr Surg. 2012; 21: 291-301
        • Dasgupta R.
        • Langer J.C.
        Hirschsprung disease.
        Curr Probl Surg. 2004; 41: 942-988
        • Das K.
        • Kini U.
        • Babu M.K.
        • et al.
        The distal level of normally innervated bowel in long segment colonic Hirschsprung's disease.
        Pediatr Surg Int. 2010; 26: 593-599
        • Lawal T.A.
        • Chatoorgoon K.
        • Collins M.H.
        • et al.
        Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel.
        J Pediatr Surg. 2011; 46: 342-347
        • Swaminathan M.
        • Kapur R.P.
        Counting myenteric ganglion cells in histologic sections: an empirical approach.
        Hum Pathol. 2010; 41: 1097-1108
        • Kapur R.P.
        Histology of the transition zone in Hirschsprung disease.
        Am J Surg Pathol. 2016; 40: 1637-1646
        • Kapur R.P.
        • Kennedy A.J.
        Histopathologic delineation of the transition zone in short-segment Hirschsprung disease.
        Pediatr Dev Pathol. 2013; 16: 252-266
        • Coe A.
        • Collins M.H.
        • Lawal T.
        • et al.
        Reoperation for Hirschsprung disease: pathology of the resected problematic distal pull-through.
        Pediatr Dev Pathol. 2012; 15: 30-38
        • Krammer H.J.
        • Karahan S.T.
        • Sigge W.
        • et al.
        Immunohistochemistry of markers of the enteric nervous system in whole-mount preparations of the human colon.
        Eur J Pediatr Surg. 1994; 4: 274-278
        • Wedel T.
        • Roblick U.
        • Gleiss J.
        • et al.
        Organization of the enteric nervous system in the human colon demonstrated by wholemount immunohistochemistry with special reference to the submucous plexus.
        Ann Anat. 1999; 181: 327-337
        • Monforte-Munoz H.
        • Gonzalez-Gomez I.
        • Rowland J.M.
        • et al.
        Increased submucosal nerve trunk caliber in aganglionosis: a "positive" and objective finding in suction biopsies and segmental resections in Hirschsprung's disease.
        Arch Pathol Lab Med. 1998; 122: 721-725
        • Mirjalili S.A.
        • Tarr G.
        • Stringer M.D.
        The length of the large intestine in children determined by computed tomography scan.
        Clin Anat. 2017; 30: 887-893
        • Puri P.
        • Hollwarth M.E.
        Paediatric surgery.
        Springer-Verlag, Berlin-Heidelberg2006
        • Schappi M.G.
        • Staiano A.
        • Milla P.J.
        • et al.
        A practical guide for the diagnosis of primary enteric nervous system disorders.
        J Pediatr Gastroenterol Nutr. 2013; 57: 677-686
        • Georgeson K.E.
        Laparoscopic-assisted pull-through for Hirschsprung's disease.
        Semin Pediatr Surg. 2002; 11: 205-210
        • Frongia G.
        • Gunther P.
        • Schenk J.P.
        • et al.
        Contrast enema for Hirschsprung disease investigation: diagnostic accuracy and validity for subsequent diagnostic and surgical planning.
        Eur J Pediatr Surg. 2015; 26: 207-214
        • Jamieson D.H.
        • Dundas S.E.
        • Belushi S.A.
        • et al.
        Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease?.
        Pediatr Radiol. 2004; 34: 811-815
        • Ghose S.I.
        • Squire B.R.
        • Stringer M.D.
        • et al.
        Hirschsprung's disease: problems with transition-zone pull-through.
        J Pediatr Surg. 2000; 35: 1805-1809
        • White F.V.
        • Langer J.C.
        Circumferential distribution of ganglion cells in the transition zone of children with Hirschsprung disease.
        Pediatr Dev Pathol. 2000; 3: 216-222
        • Doi T.
        • Kobayashi H.
        • Yamataka A.
        • et al.
        Complete innervation profile of whole bowel resected at pull-through for Hirschsprung's disease. Unexpected findings.
        Pediatr Surg Int. 2005; 21: 889-898
        • Bandyopadhyay R.
        • Chatterjee U.
        • Basu A.K.
        • et al.
        Morphometry of nerve trunks in Hirschsprung's disease.
        J Indian Assoc Pediatr Surg. 2003; 8: 195-201
        • Kapur R.P.
        Submucosal nerve diameter of greater than 40 mum is not a valid diagnostic index of transition zone pull-through.
        J Pediatr Surg. 2016; 51: 1585-1591