Advertisement

An approach to minimize postoperative enterocolitis in Hirschsprung's disease

      Abstract

      Background/Purpose

      Enterocolitis (EC) is a common and severe complication after pull-through for Hirschsprung's disease; its pathogenesis remains unclear, but the role of coexistent intestinal neuronal dysplasia (IND) in the proximal colon may be relevant. This study evaluated the relationship between postoperative EC and IND and assessed whether a surgical protocol including resection of coexistent IND could prevent postoperative EC.

      Methods

      Between June 1993 and June 2002, 36 patients with aganglionosis were submitted to definitive surgical treatment. There were 2 sequential sets of patients: group I (n = 17), in whom the resection was confined to the aganglionic colon, and group II (n = 19), who were additionally submitted to resection of the coexistent IND segment; excision was restricted to the hepatic flexure in long segmental IND. The prevalence of postoperative EC and anorectal function were evaluated and compared between the 2 groups.

      Results

      There was no mortality. Fifteen patients had isolated aganglionosis, and 21 presented with aganglionosis plus proximal IND. All 6 children who developed postoperative EC had coexistent IND. In group I, 9 patients had coexistent IND and 5 developed postoperative EC (5/17, 29%). In group II, 12 patients had coexistent IND but only 1 patient, with long segmental IND, developed postoperative EC (1/19, 5%). Among the patients with proximal IND, the prevalence of postoperative EC was 29%; but it was significantly lower in group II than in group I (1/12 or 8% vs 5/9 or 56%; P = .02). Anorectal function was excellent or good in more than 80% of the patients in both groups.

      Conclusions

      Postoperative EC was associated with retained proximal IND, suggesting that coexisting IND may be, at least, a predictive marker for this complication. Histochemical characterization of the proximal colon with no radical resection of the IND segment seems to be an effective and safe approach to minimize the prevalence of postoperative EC.

      Index words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Kobayashi H.
        • Hirakawa H.
        • Puri P.
        • et al.
        Intestinal neuronal dysplasia is a possible cause of persistent bowel symptoms after pull-through operation for Hirschsprung's disease.
        J Pediatr Surg. 1995; 30: 253-259
        • Schärli A.F.
        Neuronal intestinal dysplasia.
        Pediatr Surg Int. 1992; 7: 2-7
        • Moore S.W.
        • Laing D.
        • Kaschula R.O.C.
        • et al.
        A histological grading system for the evaluation of co-existing NID with Hirschsprung's disease.
        Eur J Pediatr Surg. 1993; 4: 293-297
        • Banani S.A.
        • Forootan H.R.
        • Kumar P.V.
        Intestinal neuronal dysplasia as a cause of surgical failure in Hirschsprung's disease: a new modality for surgical management.
        J Pediatr Surg. 1996; 31: 572-574
        • Ure B.M.
        • Holschneider A.M.
        • Schulten D.
        • et al.
        Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients.
        Pediatr Surg Int. 1997; 12: 377-382
        • Ure B.M.
        • Holschneider A.M.
        • Meier-Ruge W.
        Neuronal intestinal malformations: a retro- and prospective study in 203 patients.
        Eur J Pediatr Surg. 1998; 4: 279-286
        • Estevão-Costa J.
        • Carvalho J.L.
        • Soares-Oliveira M.
        Risk factors for the development of enterocolitis after pull-through for Hirschsprung's disease.
        J Pediatr Surg. 1999; 34: 1581-1582
        • Schmittenbecher P.P.
        • Sacher P.
        • Cholewa D.
        • et al.
        Hirschsprung's disease and intestinal neuronal dysplasia—a frequent association with implications for postoperative course.
        Pediatr Surg Int. 1999; 15: 553-558
        • Meier-Ruge W.
        • Schärli A.F.
        • Stoss F.
        How to improve histopathological results in the biopsy diagnosis of gut dysganglionosis—a methodological review.
        Pediatr Surg Int. 1995; 10: 454-458
        • Nogueira A.
        • Campos M.
        • Soares-Oliveira M.
        • et al.
        Histochemical and immunohistochemical study of the intrinsic innervation of the colon dysganglionosis.
        Pediatr Surg Int. 2001; 17: 144-151
        • Borchard F.
        • Meier-Ruge W.
        • Wiebecke B.
        • et al.
        Innervationsstörungen des Dickdarmes—Klassifikation und Diagnostik.
        Pathologe. 1991; 12: 171-174
        • Carvalho J.L.
        • Campos M.
        • Soares-Oliveira M.
        • et al.
        Laparoscopic colonic mapping of dysganglionosis.
        Pediatr Surg Int. 2001; 17: 493-495
        • Stephens F.D.
        • Smith E.D.
        Classification, identification and assessment of surgical treatment of anorectal anomalies.
        in: Report of a workshop meeting. Racine, Wisconsin, May 25-27, 19841984
        • Moore S.W.
        • Albertyn R.
        • Cywes S.
        Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease.
        J Pediatr Surg. 1996; 31: 1496-1502
        • Elhalaby E.A.
        • Coran A.G.
        • Blane C.E.
        • et al.
        Enterocolitis associated with Hirschsprung's disease: a clinical-radiological characterization based on 168 patients.
        J Pediatr Surg. 1995; 30: 76-83
        • Reding R.
        • de Goyet J.V.
        • Gosseye S.
        • et al.
        Hirschsprung's disease: a 20-year experience.
        J Pediatr Surg. 1997; 32: 1221-1225
        • Hackam D.J.
        • Filler R.M.
        • Pearl R.H.
        Enterocolitis after the surgical treatment of Hirschsprung's disease: risk factors and financial impact.
        J Pediatr Surg. 1998; 33: 830-833
        • Coran A.G.
        • Teitelbaum D.H.
        Recent advances in the management of Hirschsprung's disease.
        Am J Surg. 2000; 180: 382-387
        • Mattioli G.
        • Castagnetti M.
        • Martucciello G.
        • et al.
        Results of a mechanical Duhamel pull-through for the treatment of Hirschsprung's disease and intestinal neuronal dysplasia.
        J Pediatr Surg. 2004; 39: 1349-1355
        • Minford J.L.
        • Ram A.
        • Turnock R.R.
        • et al.
        Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung's disease.
        J Pediatr Surg. 2004; 39: 161-165
        • Nixon
        Hirschsprung's disease: progress in management and diagnostics.
        World J Surg. 1985; 9: 189-202
        • Weber T.
        • Fortuna R.S.
        • Sillen M.L.
        • et al.
        Reoperation for Hirschsprung's disease.
        J Pediatr Surg. 1999; 34: 153-157
        • Wang J.S.
        • Lee H.C.
        • Huang F.Y.
        • et al.
        Unexpected mortality in pediatric patients with postoperative Hirschsprung's disease.
        Pediatr Surg Int. 2004; 20: 525-528
        • Teitelbaum D.H.
        • Caniano D.A.
        • Qualman S.J.
        The pathophysiology of Hirschsprung's-associated enterocolitis: importance of histologic correlates.
        J Pediatr Surg. 1989; 24: 1271-1277
        • Wilson-Storey D.
        • Scobie W.G.
        Impaired gastrointestinal mucosal defense in Hirschsprung's disease: a clue to the pathogenesis of enterocolitis?.
        J Pediatr Surg. 1989; 24: 462-464
        • Imamura A.
        • Puri P.
        • O'Brian D.S.
        • et al.
        Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung's disease.
        Gut. 1992; 33: 801-806
        • Turnock R.R.
        • Spitz L.
        • Strobel S.
        A study of mucosal gut immunity in infants who develop Hirschsprung's-associated enterocolitis.
        J Pediatr Surg. 1992; 27: 828-829
        • Soeda J.
        • O'Brian D.S.
        • Puri P.
        Regional reduction in intestinal neuroendocrine cell population in enterocolitis complicating Hirschsprung's disease.
        J Pediatr Surg. 1993; 28: 1063-1068
        • Lui V.C.
        • Sham M.H.
        • Tam P.K.
        CDX-1 and CDX-2 are expressed in human colonic mucosa and are down-regulated in patients with Hirschsprung's disease associated enterocolitis.
        Biochim Biophys Acta. 2001; 1537: 89-100
        • Farrugia M.-K.
        • Alexander N.
        • Clarke S.
        • et al.
        Does transitional zone pull-through in Hirschsprung's disease imply a poor prognosis?.
        J Pediatr Surg. 2003; 38: 1766-1769
        • Mattar A.F.
        • Coran A.G.
        • Teitelbaum D.H.
        MUC-2 mucin production in Hirschsprung's disease: possible association with enterocolitis development.
        J Pediatr Surg. 2003; 38: 124-131
        • Schulten D.
        • Holschneider A.M.
        • Meier-Ruge W.
        Proximal segment histology of resected bowel in Hirschsprung's disease predicts postoperative bowel function.
        Eur J Pediatr Surg. 2000; 10: 378-381
        • Hanimann B.
        • Inderbitzin D.
        • Briner J.
        • et al.
        Clinical relevance of Hirschsprung-associated neuronal intestinal dysplasia (HANID).
        Eur J Pediatr Surg. 1992; 2: 147-149
        • Martucciello G.
        • Favre A.
        • Torre M.
        • et al.
        A new rapid acetylcholinesterase histochemical method for intraoperative diagnosis of Hirschsprung's disease and intestinal neuronal dysplasia.
        Eur J Pediatr Surg. 2001; 11: 300-304
        • Yamataka A.
        • Yoshida R.
        • Kobayashi H.
        • et al.
        Laparoscopy-assisted suction colonic biopsy and intraoperative rapid acetylcholinesterase staining during transanal pull-through for Hirschsprung's disease.
        J Pediatr Surg. 2002; 37: 1661-1663