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Pediatric Inflammatory Bowel Disease: Unclassified

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Pediatric Inflammatory Bowel Disease

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Abstract

The term indeterminate colitis was first introduced in the 1970s to further classify a subset of patients with inflammatory bowel disease, whose surgical colectomy specimens showed features consistent with both ulcerative colitis and Crohn disease. With time, advances in diagnostics, and recognition of diverse phenotypes of disease, this definition has evolved. In this chapter, we will review the criteria used to establish a diagnosis of inflammatory bowel disease-unclassified (IBD-U), the epidemiology of IBD-U, diagnostic evaluation, as well as considerations for medical and surgical management.

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Abbreviations

CD:

Crohn Disease

UC:

Ulcerative Colitis

IBD:

Inflammatory Bowel Disease

IBD-U:

Inflammatory Bowel Disease-Unclassified

References

  1. Price AB. Overlap in the spectrum of non-specific inflammatory bowel disease—‘colitis indeterminate’. J Clin Pathol. 1978;31:567–77.

    CAS  PubMed  PubMed Central  Google Scholar 

  2. Satsangi J, Silverberg MS, Vermeire S, et al. The Montreal classification of inflammatory bowel disease: controversies, consensus, and implications. Gut. 2006;55:749–53.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Levine A, Griffiths A, Markowitz J, et al. Pediatric modification of the Montreal classification for inflammatory bowel disease: the Paris classification. Inflamm Bowel Dis. 2011;17:1314–21.

    PubMed  Google Scholar 

  4. North American Society for Pediatric Gastroenterology H, Nutrition, Colitis Foundation of A, et al. Differentiating ulcerative colitis from Crohn disease in children and young adults: report of a working group of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the Crohn’s and Colitis Foundation of America. J Pediatr Gastroenterol Nutr. 2007;44:653–74.

    Google Scholar 

  5. Levine A, Koletzko S, Turner D, et al. ESPGHAN revised Porto criteria for the diagnosis of inflammatory bowel disease in children and adolescents. J Pediatr Gastroenterol Nutr. 2014;58:795–806.

    CAS  PubMed  Google Scholar 

  6. Birimberg-Schwartz L, Zucker DM, Akriv A, et al. Development and validation of diagnostic criteria for IBD subtypes including IBD-unclassified in children: a multicentre study from the pediatric IBD Porto Group of ESPGHAN. J Crohns Colitis. 2017;11:1078–84.

    PubMed  Google Scholar 

  7. Benchimol EI, Guttmann A, Griffiths AM, et al. Increasing incidence of paediatric inflammatory bowel disease in Ontario, Canada: evidence from health administrative data. Gut. 2009;58:1490–7.

    CAS  PubMed  Google Scholar 

  8. Chan JM, Carroll MW, Smyth M, et al. Comparing health administrative and clinical registry data: trends in incidence and prevalence of pediatric inflammatory bowel disease in British Columbia. Clin Epidemiol. 2021;13:81–90.

    PubMed  PubMed Central  Google Scholar 

  9. Jabandziev P, Pinkasova T, Kunovsky L, et al. Regional incidence of inflammatory bowel disease in a Czech pediatric population: 16 years of experience (2002–2017). J Pediatr Gastroenterol Nutr. 2020;70:586–92.

    CAS  PubMed  PubMed Central  Google Scholar 

  10. Sykora J, Pomahacova R, Kreslova M, et al. Current global trends in the incidence of pediatric-onset inflammatory bowel disease. World J Gastroenterol. 2018;24:2741–63.

    PubMed  PubMed Central  Google Scholar 

  11. Adamiak T, Walkiewicz-Jedrzejczak D, Fish D, et al. Incidence, clinical characteristics, and natural history of pediatric IBD in Wisconsin: a population-based epidemiological study. Inflamm Bowel Dis. 2013;19:1218–23.

    PubMed  Google Scholar 

  12. Benchimol EI, Manuel DG, Guttmann A, et al. Changing age demographics of inflammatory bowel disease in Ontario, Canada: a population-based cohort study of epidemiology trends. Inflamm Bowel Dis. 2014;20:1761–9.

    PubMed  Google Scholar 

  13. Muller KE, Lakatos PL, Arato A, et al. Incidence, Paris classification, and follow-up in a nationwide incident cohort of pediatric patients with inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2013;57:576–82.

    PubMed  Google Scholar 

  14. Abramson O, Durant M, Mow W, et al. Incidence, prevalence, and time trends of pediatric inflammatory bowel disease in Northern California, 1996 to 2006. J Pediatr. 2010;157:233–9. e1

    PubMed  Google Scholar 

  15. Pinsk V, Lemberg DA, Grewal K, et al. Inflammatory bowel disease in the South Asian pediatric population of British Columbia. Am J Gastroenterol. 2007;102:1077–83.

    PubMed  Google Scholar 

  16. van der Zaag-Loonen HJ, Casparie M, Taminiau JA, et al. The incidence of pediatric inflammatory bowel disease in the Netherlands: 1999–2001. J Pediatr Gastroenterol Nutr. 2004;38:302–7.

    PubMed  Google Scholar 

  17. Chandradevan R, Hofmekler T, Mondal K, et al. Evolution of pediatric inflammatory bowel disease unclassified (IBD-U): incorporated with serological and gene expression profiles. Inflamm Bowel Dis. 2018;24:2285–90.

    PubMed  Google Scholar 

  18. Dhaliwal J, Walters TD, Mack DR, et al. Phenotypic variation in paediatric inflammatory bowel disease by age: a multicentre prospective inception cohort study of the canadian children IBD network. J Crohns Colitis. 2020;14:445–54.

    CAS  PubMed  Google Scholar 

  19. Malaty HM, Mehta S, Abraham B, et al. The natural course of inflammatory bowel disease-indeterminate from childhood to adulthood: within a 25 year period. Clin Exp Gastroenterol. 2013;6:115–21.

    PubMed  PubMed Central  Google Scholar 

  20. Prenzel F, Uhlig HH. Frequency of indeterminate colitis in children and adults with IBD – a metaanalysis. J Crohns Colitis. 2009;3:277–81.

    PubMed  Google Scholar 

  21. Everhov AH, Sachs MC, Malmborg P, et al. Changes in inflammatory bowel disease subtype during follow-up and over time in 44,302 patients. Scand J Gastroenterol. 2019;54:55–63.

    PubMed  Google Scholar 

  22. Heyman MB, Kirschner BS, Gold BD, et al. Children with early-onset inflammatory bowel disease (IBD): analysis of a pediatric IBD consortium registry. J Pediatr. 2005;146:35–40.

    PubMed  Google Scholar 

  23. Winter DA, Karolewska-Bochenek K, Lazowska-Przeorek I, et al. Pediatric IBD-unclassified is less common than previously reported; results of an 8-year audit of the EUROKIDS registry. Inflamm Bowel Dis. 2015;21:2145–53.

    PubMed  Google Scholar 

  24. Aziz DA, Moin M, Majeed A, et al. Paediatric inflammatory bowel disease: clinical presentation and disease location. Pak J Med Sci. 2017;33:793–7.

    PubMed  PubMed Central  Google Scholar 

  25. Buderus S, Scholz D, Behrens R, et al. Inflammatory bowel disease in pediatric patients: characteristics of newly diagnosed patients from the CEDATA-GPGE Registry. Dtsch Arztebl Int. 2015;112:121–7.

    PubMed  PubMed Central  Google Scholar 

  26. Rinawi F, Assa A, Eliakim R, et al. The natural history of pediatric-onset IBD-unclassified and prediction of Crohn’s disease reclassification: a 27-year study. Scand J Gastroenterol. 2017;52:558–63.

    PubMed  Google Scholar 

  27. Ashton JJ, Bonduelle Q, Mossotto E, et al. Endoscopic and histological assessment of paediatric inflammatory bowel disease over a 3-year follow-up period. J Pediatr Gastroenterol Nutr. 2018;66:402–9.

    PubMed  Google Scholar 

  28. Maltz R, Podberesky DJ, Saeed SA. Imaging modalities in pediatric inflammatory bowel disease. Curr Opin Pediatr. 2014;26:590–6.

    PubMed  Google Scholar 

  29. Quencer KB, Nimkin K, Mino-Kenudson M, et al. Detecting active inflammation and fibrosis in pediatric Crohn’s disease: prospective evaluation of MR-E and CT-E. Abdom Imaging. 2013;38:705–13.

    PubMed  PubMed Central  Google Scholar 

  30. Anupindi SA, Grossman AB, Nimkin K, et al. Imaging in the evaluation of the young patient with inflammatory bowel disease: what the gastroenterologist needs to know. J Pediatr Gastroenterol Nutr. 2014;59:429–39.

    PubMed  Google Scholar 

  31. Schooler GR, Hull NC, Mavis A, et al. MR imaging evaluation of inflammatory bowel disease in children: where are we now in 2019. Magn Reson Imaging Clin N Am. 2019;27:291–300.

    PubMed  Google Scholar 

  32. Gokli A, Dillman JR, Humphries PD, et al. Contrast-enhanced ultrasound of the pediatric bowel. Pediatr Radiol. 2021;51:2214–28.

    PubMed  PubMed Central  Google Scholar 

  33. Ripolles T, Martinez-Perez MJ, Paredes JM, et al. Contrast-enhanced ultrasound in the differentiation between phlegmon and abscess in Crohn’s disease and other abdominal conditions. Eur J Radiol. 2013;82:e525–31.

    PubMed  Google Scholar 

  34. de Bie CI, Buderus S, Sandhu BK, et al. Diagnostic workup of paediatric patients with inflammatory bowel disease in Europe: results of a 5-year audit of the EUROKIDS registry. J Pediatr Gastroenterol Nutr. 2012;54:374–80.

    PubMed  Google Scholar 

  35. Netz U, Galbraith NJ, O'Brien S, et al. Long-term outcomes following ileal pouch-anal anastomosis in patients with indeterminate colitis. Surgery. 2018;163:535–41.

    PubMed  Google Scholar 

  36. Min SB, Le-Carlson M, Singh N, et al. Video capsule endoscopy impacts decision making in pediatric IBD: a single tertiary care center experience. Inflamm Bowel Dis. 2013;19:2139–45.

    PubMed  Google Scholar 

  37. Monteiro S, Dias de Castro F, Boal Carvalho P, et al. Essential role of small bowel capsule endoscopy in reclassification of colonic inflammatory bowel disease type unclassified. World J Gastrointest Endosc. 2017;9:34–40.

    PubMed  PubMed Central  Google Scholar 

  38. Aloi M, Birimberg-Schwartz L, Buderus S, et al. Treatment options and outcomes of pediatric IBDU compared with other IBD subtypes: a retrospective multicenter study from the IBD Porto Group of ESPGHAN. Inflamm Bowel Dis. 2016;22:1378–83.

    PubMed  Google Scholar 

  39. Maxwell EC, Dawany N, Baldassano RN, et al. Diverting ileostomy for the treatment of severe, refractory, pediatric inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2017;65:299–305.

    PubMed  Google Scholar 

  40. Yu CS, Pemberton JH, Larson D. Ileal pouch-anal anastomosis in patients with indeterminate colitis: long-term results. Dis Colon Rectum. 2000;43:1487–96.

    CAS  PubMed  Google Scholar 

  41. Jackson KL, Stocchi L, Duraes L, et al. Long-term outcomes in indeterminate colitis patients undergoing ileal pouch-anal anastomosis: function, quality of life, and complications. J Gastrointest Surg. 2017;21:56–61.

    PubMed  Google Scholar 

  42. Turina M, Remzi FH. The J-pouch for patients with Crohn’s disease and indeterminate colitis: (when) is it an option? J Gastrointest Surg. 2014;18:1343–4.

    PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Brooke Boyer & Elana B. Mitchel

Authors
  1. Brooke Boyer
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  2. Elana B. Mitchel
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Corresponding author

Correspondence to Elana B. Mitchel .

Editor information

Editors and Affiliations

  1. Division of GI, Hepatology & Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Petar Mamula

  2. Division of GI, Hepatology & Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Judith R. Kelsen

  3. Division of GI, Hepatology & Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Andrew B. Grossman

  4. Division of GI, Hepatology & Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Robert N. Baldassano

  5. Division of Pediatric Gastroenterology, Prisma Health Children’s Hospital—Upstate, Greenville, SC, USA

    Jonathan E. Markowitz

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Boyer, B., Mitchel, E.B. (2023). Pediatric Inflammatory Bowel Disease: Unclassified. In: Mamula, P., Kelsen, J.R., Grossman, A.B., Baldassano, R.N., Markowitz, J.E. (eds) Pediatric Inflammatory Bowel Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-14744-9_9

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  • DOI: https://doi.org/10.1007/978-3-031-14744-9_9

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-031-14744-9

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