Abstract
Aim and objectives
Hirschsprung-associated enterocolitis (HAEC) continues to be an important cause of morbidity in patients with Hirschsprung’s disease (HSCR). HAEC can occur at any time during the course of the disease. The reported incidence of HAEC before surgery ranges from 6 to 50%, and after surgery, it ranges from 2 to 35%. HAEC and inflammatory bowel disease (IBD) have similar clinical presentation including diarrhea, hematochezia, and abdominal pain. In recent years, isolated cases of IBD have been reported in patients who had surgical treatment for HSCR. The exact pathogenesis of HAEC or IBD is not known. However, both conditions are characterized by an abnormal intestinal mucosal barrier function, which may be a common pathway. The purpose of this meta-analysis was to determine the clinical presentation and outcome in patients with HSCR who developed IBD after pull-through operation.
Materials and methods
A systematic literature search for relevant articles was performed in four databases using the combinations of the following terms “inflammatory bowel disease”, “Crohn/Crohn’s disease”, “ulcerative colitis”, and “Hirschsprung disease/Hirschsprung’s disease” for studies published between 1990 and 2017. The relevant cohorts of HSCR associated with IBD were systematically searched for clinical presentation and outcomes.
Results
14 studies met defined inclusion criteria, reporting a total of 66 patients who had HSCR associated with IBD. Mean age at first operation for HSCR was 5.8 months, mean age at diagnosis of IBD was 7.7 years, and the majority of patients were male (73%). The extent of aganglionosis was total colonic aganglionosis in 41% of patients, long segment in 45%, and rectosigmoid in 14%. The majority of patients underwent a Duhamel procedure (84%) for HSCR. The distribution of IBD was Crohn’s disease in 72.3% of patients, ulcerative colitis in 16.9%, and others in 10.8%. Eight articles (47 patients) reported about HAEC, and 22 patients (47%) had experienced HAEC after surgery for HSCR.
Conclusion
Male patients with extensive colonic aganglionosis who continue to suffer from postoperative HAEC after a Duhamel procedure are more susceptible to develop IBD. Recognition of IBD may be important in the long-term follow-up of HSCR patients who have had postoperative HAEC.
Similar content being viewed by others
References
Puri P (2011) Hirschsprung’s disease. In: Puri P (ed) Newborn surgery. Hodder Arnold, London, pp 554–565
Engum SA, Grosfeld JL (2004) Long-term results of treatment of Hirschsprung’s disease. Semin Pediatr Surg 13:273–285
Hackam DJ, Filler RM, Pearl RH (1998) Enterocolitis after the surgical treatment of Hirschsprung’s disease: risk factors and financial impact. J Pediatr Surg 33:830–833
Szigethy E, McLafferty L, Goyal A (2010) Inflammatory bowel disease. Child Adolesc Psychiatr Clin N Am 19:301–318, ix
de Souza HS, Fiocchi C (2016) Immunopathogenesis of IBD: current state of the art. Nat Rev Gastroenterol Hepatol 13:13–27
Guan Q, Zhang J (2017) Recent advances: the imbalance of cytokines in the pathogenesis of inflammatory bowel disease. Mediat Inflamm 2017:4810258
Freeman JJ, Rabah R, Hirschl RB, Maspons A, Meier D, Teitelbaum DH (2015) Anti-TNF-alpha treatment for post-anastomotic ulcers and inflammatory bowel disease with Crohn’s-like pathologic changes following intestinal surgery in pediatric patients. Pediatr Surg Int 31:77–82
Kessler BH, So HB, Becker JM (1999) Crohn’s disease mimicking enterocolitis in a patient with an endorectal pull-through for Hirschsprung’s disease. J Pediatr Gastroenterol Nutr 29:601–603
Arumugam R, Brandt ML, Jaksic T, Gilger M (2000) Crohn’s disease presenting as chronic constipation: a case report. Clin Pediatr (Phila) 39:369–371
Coran AG (1990) A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull-throughs for benign disease of the colon and rectum in children and adults. Ann Surg 212:242–247 (discussion 247–248)
Deising A, Veerappan G, Betteridge J (2011) Crohn’s disease presenting in an adult surgically treated for Hirschsprung’s disease. Inflamm Bowel Dis 17:51–52
Kim HY, Kim TW (2017) Crohn’s disease with ankylosing spondylitis in an adolescent patient who had undergone long ileo-colonic anastomosis for Hirschsprung’s disease as an infant. Intest Res 15:133–137
Fremond ML, Viala J, Treton X, Roy M, Berrebi D, Gottrand F et al (2014) Digestive perianastomotic ulcerations and Crohn’s disease. J Crohns Colitis 8:1624–1631
Muller CO, Rossignol G, Montalva L, Viala J, Martinez-Vinson C, Mosca A et al (2016) Long-term outcome of laparoscopic Duhamel procedure for extended Hirschsprung’s disease. J Laparoendosc Adv Surg Tech A 26:1032–1035
Cucino C, Sonnenberg A (2001) The comorbid occurrence of other diagnoses in patients with ulcerative colitis and Crohn’s disease. Am J Gastroenterol 96:2107–2112
Dray X, Sokol H, Vahedi K, Lavergne-Slove A, Marteau P (2007) Pure ileal Crohn’s disease without colonic involvement after a long ileo-colonic anastomosis (Lester Martin procedure) for Hirschsprung’s disease: an argument favoring a specific sensitivity of the ileum in a subset of patients with Crohn’s disease. Inflamm Bowel Dis 13:243–244
Levin DN, Marcon MA, Rintala RJ, Jacobson D, Langer JC (2012) Inflammatory bowel disease manifesting after surgical treatment for Hirschsprung disease. J Pediatr Gastroenterol Nutr 55:272–277
Pena A, Elicevik M, Levitt MA (2007) Reoperations in Hirschsprung disease. J Pediatr Surg 42:1008–1013 (discussion 1013–1004)
Ikeuchi H, Kusunoki M, Yamamura T, Utsunomiya J (1997) A case of Crohn’s disease following Hirschsprung disease. J Jpn Pract Surg Soc 58:622–625
Imamura A, Puri P, O’Briain DS, Reen DJ (1992) Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut 33:801–806
Pontarelli EM, Ford HR, Gayer CP (2013) Recent developments in Hirschsprung’s-associated enterocolitis. Curr Gastroenterol Rep 15:340
Korzenik JR, Podolsky DK (2006) Evolving knowledge and therapy of inflammatory bowel disease. Nat Rev Drug Discov 5:197–209
Loftus EV, Jr (2004) Clinical epidemiology of inflammatory bowel disease: Incidence, prevalence, and environmental influences. Gastroenterology 126:1504–1517
Binder V (2004) Epidemiology of IBD during the twentieth century: an integrated view. Best Pract Res Clin Gastroenterol 18:463–479
Hanauer SB (2006) Inflammatory bowel disease: epidemiology, pathogenesis, and therapeutic opportunities. Inflamm Bowel Dis 12(Suppl 1):S3–S9
Ahmad T, Satsangi J, McGovern D, Bunce M, Jewell DP (2001) Review article: the genetics of inflammatory bowel disease. Aliment Pharmacol Ther 15:731–748
Kusunoki M, Ikeuchi H, Yanagi H, Shoji Y, Yamamura T (1997) Stapled fistulectomy to treat enteroenteric fistulas in Crohn’s disease. Surg Today 27:574–575
Elhalaby EA, Coran AG, Blane CE, Hirschl RB, Teitelbaum DH (1995) Enterocolitis associated with Hirschsprung’s disease: a clinical-radiological characterization based on 168 patients. J Pediatr Surg 30:76–83
Surana R, Quinn FM, Puri P (1994) Short-gut syndrome: intestinal adaptation in a patient with 12 cm of jejunum. J Pediatr Gastroenterol Nutr 19:246–249
Quinn FM, Surana R, Puri P (1994) The influence of trisomy 21 on outcome in children with Hirschsprung’s disease. J Pediatr Surg 29:781–783
Laughlin DM, Friedmacher F, Puri P (2012) Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 28:773–779
Shikhare G, Kugathasan S (2010) Inflammatory bowel disease in children: current trends. J Gastroenterol 45:673–682
Mundt E, Bates MD (2010) Genetics of Hirschsprung disease and anorectal malformations. Semin Pediatr Surg 19:107–117
Bonen DK, Cho JH (2003) The genetics of inflammatory bowel disease. Gastroenterology 124:521–536
Lacher M, Fitze G, Helmbrecht J, Schroepf S, Berger M, Lohse P et al (2010) Hirschsprung-associated enterocolitis develops independently of NOD2 variants. J Pediatr Surg 45:1826–1831
Lidar M, Langevitz P, Shoenfeld Y (2009) The role of infection in inflammatory bowel disease: initiation, exacerbation and protection. Isr Med Assoc J 11:558–563
Mattar AF, Coran AG, Teitelbaum DH (2003) MUC-2 mucin production in Hirschsprung’s disease: possible association with enterocolitis development. J Pediatr Surg 38:417–421 (discussion 417–421)
Johansson ME, Hansson GC (2013) Mucus and the goblet cell. Dig Dis 31:305–309
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Rights and permissions
About this article
Cite this article
Nakamura, H., Lim, T. & Puri, P. Inflammatory bowel disease in patients with Hirschsprung’s disease: a systematic review and meta-analysis. Pediatr Surg Int 34, 149–154 (2018). https://doi.org/10.1007/s00383-017-4182-4
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-017-4182-4