Skip to main content

Crohn’s Disease

  • Living reference work entry
  • First Online:
Book cover Pediatric Surgery

Abstract

Crohn’s disease (CD) in children has become increasingly common in recent years, especially in the developed world. Despite advances considering knowledge of the pathogenesis of these diseases, the precise etiology is not understood, and there remains no permanent cure. Pain, diarrhea, and weight loss are typical symptoms of pediatric CD. Atypical symptoms and extra-intestinal manifestations may occur. The patient’s growth and nutritional status are often compromised at diagnosis of CD. The location and severity of pediatric CD differ substantially from adult-onset disease. The initial presentation is more widespread and severe that in adults. Prompt diagnosis, thereby avoiding the consequences of diagnostic delay, is essential in the work-up of suspected CD. The aim of the management of CD is to achieve rapid remission and there are several options to reach this goal. The initial treatment is medical; surgery is considered in a complicated disease course or in the case of refractory disease. Acute indications for surgery include emergent conditions such as toxic megacolon, intra-abdominal abscesses, and sepsis or severe bleeding. Elective indications for surgery are severe strictures and perianal disease, significant prepubertal growth delay, and complications of or unresponsiveness to medical therapy. CD in children requires multidisciplinary care in the context of a growing child. The management of pediatric CD focuses on controlling gut inflammation and optimizing growth, development, and quality of life.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Institutional subscriptions

References

  • Alves A, Panis Y, Trancart D, Regimbeau JM, Pocard M, Valleur P. Factors associated with clinically significant anastomotic leakage after large bowel resection: multivariate analysis of 707 patients. World J Surg. 2002;26(4):499–502.

    Article  PubMed  Google Scholar 

  • Amil-Dias J, Kolacek S, Turner D, et al. Surgical management of Crohn disease in children: guidelines from the Paediatric IBD Porto Group of ESPGHAN. J Pediatr Gastroenterol Nutr. 2017;64(5):818–35.

    Article  Google Scholar 

  • Auvin S, Molinié F, Gower-Rousseau C, Brazier F, Merle V, Grandbastien B, Marti R, Lerebours E, Dupas JL, Colombel JF, Salomez JL, Cortot A, Turck D. Incidence, clinical presentation and location at diagnosis of pediatric inflammatory bowel disease: a prospective population-based study in northern France (1988–1999). J Pediatr Gastroenterol Nutr. 2005;41:49–55.

    Article  PubMed  Google Scholar 

  • Cameron DJ. Upper and lower gastrointestinal endoscopy in children and adolescents with Crohn’s disease: a prospective study. J Gastroenterol Hepatol. 1991;6:355–8.

    Article  CAS  PubMed  Google Scholar 

  • Castellaneta SP, Afzal NA, Greenberg M, Deere H, Davies S, Murch SH, Walker-Smith JA, Thomson M, Srivistrava A. Diagnostic role of upper gastrointestinal endoscopy in pediatric inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2004;39:257–61.

    Article  CAS  PubMed  Google Scholar 

  • Coscia M, Gentilini L, Laureti S, Gionchetti P, Rizzello F, Campieri M, Calabrese C, Poggioli G. Risk of permanent stoma in extensive Crohn’s colitis: the impact of biological drugs. Color Dis. 2013;15(9):1115–22.

    CAS  Google Scholar 

  • de Bie CI, Escher JC, de Ridder L. Antitumor necrosis factor treatment for pediatric inflammatory bowel disease. Inflamm Bowel Dis. 2012;18:981–98.

    Google Scholar 

  • de Zoeten EF, Pasternak BA, Mattei P, Kramer RE, Kader HA. Diagnosis and treatment of perianal Crohn disease: NASPGHAN clinical report and consensus statement. J Pediatr Gastroenterol Nutr. 2013;57:401–12.

    Article  PubMed  Google Scholar 

  • Debruyn JC, Soon IS, Hubbard J, Wrobel I, Panaccione R, Kaplan GG. Nationwide temporal trends in incidence of hospitalization and surgical intestinal resection in pediatric inflammatory bowel diseases in the United States from 1997 to 2009. Inflamm Bowel Dis. 2013;19(11):2423–32.

    Article  PubMed  Google Scholar 

  • Di Nardo G, Aloi M, Oliva S, Civitelli F, Casciani E, Cucchiara S. Investigation of small bowel in pediatric Crohn’s disease. Inflamm Bowel Dis. 2012;18:1760–76.

    Article  PubMed  Google Scholar 

  • Diamond IR, Gerstle JT, Kim PCW, Langer JC. Outcomes after laparoscopic surgery in children with inflammatory bowel disease. Surg Endosc. 2010;24(11):2796–802.

    Article  PubMed  Google Scholar 

  • Dignass A, Van Assche G, Lindsay JO, Lémann M, Söderholm J, Colombel JF, Danese S, D’Hoore A, Gassull M, Gomollón F, Hommes DW, Michetti P, O’Morain C, Oresland T, Windsor A, Stange EF, Travis SP, European Crohn’s and Colitis Organisation (ECCO). The second European evidence-based consensus on the diagnosis and management of Crohn’s disease: current management. J Crohns Colitis. 2010;4(1):28–62.

    Article  CAS  PubMed  Google Scholar 

  • Dolgin SE. Surgical management of upper gastrointestinal tract and small bowel Crohn’s disease. Semin Pediatr Surg. 2007;16(3):172–7.

    Article  PubMed  Google Scholar 

  • Dotson JL, Hyams JS, Markowitz J, LeLeiko NS, Mack DR, Evans JS, Pfefferkorn MD, Griffiths AM, Otley AR, Bousvaros A, Kugathasan S, Rosh JR, Keljo D, Carvalho RS, Tomer G, Mamula P, Kay MH, Kerzner B, Oliva-Hemker M, Langton CR, Crandall W. Extraintestinal manifestations of pediatric inflammatory bowel disease and their relation to disease type and severity. J Pediatr Gastroenterol Nutr. 2010;51:140–5.

    Article  PubMed  Google Scholar 

  • Gearry RB, Kamm MA, Hart AL, Bassett P, Gabe SM, Nightingale JM. Predictors for developing intestinal failure in patients with Crohn’s disease. J Gastroenterol Hepatol. 2013;28(5):801–7.

    Article  PubMed  Google Scholar 

  • Giles E, Barclay AR, Chippington S, Wilson DC. Systematic review: MRI enterography for assessment of small bowel involvement in paediatric Crohn’s disease. Aliment Pharmacol Ther. 2013;37(12):1121–31.

    Article  CAS  PubMed  Google Scholar 

  • Hildebrand H, Finkel Y, Grahnquist L, Lindholm J, Ekbom A, Askling J. Changing pattern of paediatric inflammatory bowel disease in northern Stockholm 1990–2001. Gut. 2003;52:1432–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Hojsak I, Pavić AM, Mišak Z, Kolaček S. Risk factors for relapse and surgery rate in children with Crohn’s disease. Eur J Pediatr. 2014;173:617–21.

    Article  PubMed  Google Scholar 

  • Hukkinen M, Pakarinen M, Piekkala M, Koivusalo A, Rintala R, Kolho KL. Treatment of complex perianal fistulas with seton and infliximab in adolescents with Crohn’s disease. J Crohns Colitis. 2014;8:756–62.

    Article  PubMed  Google Scholar 

  • Jostins L, Ripke S, Weersma RK, et al. Host-microbe interactions have shaped the genetic architecture of inflammatory bowel disease. Nature. 2012;491:119–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kiran RP, Nisar PJ, Goldblum JR, Fazio VW, Remzi FH, Shen B, Lavery IC. Dysplasia associated with Crohn’s colitis: segmental colectomy or more extended resection? Ann Surg. 2012;256:221–6.

    Google Scholar 

  • Kleer CG, Appelman HD. Surgical pathology of Crohn’s disease. Surg Clin North Am. 2001;81:13–30.

    Article  CAS  PubMed  Google Scholar 

  • Knights D, Lassen KG, Xavier RJ. Advances in inflammatory bowel disease pathogenesis: linking host genetics and the microbiome. Gut. 2013;62:1505–10.

    Article  CAS  PubMed  Google Scholar 

  • Kostakis ID, Cholidou KG, Vaiopoulos AG, Vlachos IS, Perrea D, Vaos G. Fecal calprotectin in pediatric inflammatory bowel disease: a systematic review. Dig Dis Sci. 2013;58:309–19.

    Article  CAS  PubMed  Google Scholar 

  • Kucharska M, Daniluk U, Kwiatek-Średzińska KA, et al. Hepatobiliary manifestations of inflammatory bowel disease in children. Clin Exp Hepatol. 2019;5(3):203–9.

    Article  PubMed  PubMed Central  Google Scholar 

  • Levine A, Griffiths A, Markowitz J, Wilson DC, Turner D, Russell RK, Fell J, Ruemmele FM, Walters T, Sherlock M, Dubinsky M, Hyams JS. Pediatric modification of the Montreal classification for inflammatory bowel disease: the Paris classification. Inflamm Bowel Dis. 2011;17:1314–21.

    Article  PubMed  Google Scholar 

  • Levine A, Koletzko S, Turner D, Escher JC, Cucchiara S, de Ridder L, Kolho KL, Veres G, Russell RK, Paerregaard A, Buderus S, Greer ML, Dias JA, Veereman-Wauters G, Lionetti P, Sladek M, Carpi JM, Staiano A, Ruemmele FM, Wilson DC. The ESPGHAN revised Porto criteria for the diagnosis of inflammatory bowel disease in children and adolescents. J Pediatr Gastroenterol Nutr. 2013;58:795–806.

    Google Scholar 

  • Mack DR, Langton C, Markowitz J, LeLeiko N, Griffiths A, Bousvaros A, Evans J, Kugathasan S, Otley A, Pfefferkorn M, Rosh J, Mezoff A, Moyer S, Oliva-Hemker M, Rothbaum R, Wyllie R, delRosario JF, Keljo D, Lerer T, Hyams J, Pediatric Inflammatory Bowel Disease Collaborative Research Group. Laboratory values for children with newly diagnosed inflammatory bowel disease. Pediatrics. 2007;119:1113–9.

    Article  PubMed  Google Scholar 

  • Mackner LM, Crandall WV. Long-term psychosocial outcomes reported by children and adolescents with inflammatory bowel disease. Am J Gastroenterol. 2005;100:1386–92.

    Article  PubMed  Google Scholar 

  • Moir CR. Surgical management of Crohn’s colitis. Semin Pediatr Surg. 2007;16(3):178–84.

    Article  PubMed  Google Scholar 

  • Morgan XC, Tickle TL, Sokol H, Gevers D, Devaney KL, Ward DV, Reyes JA, Shah SA, LeLeiko N, Snapper SB, Bousvaros A, Korzenik J, Sands BE, Xavier RJ, Huttenhower C. Dysfunction of the intestinal microbiome in inflammatory bowel disease and treatment. Genome Biol. 2012;13:R79.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ogura Y, Bonen DK, Inohara N, Nicolae DL, Chen FF, Ramos R, Britton H, Moran T, Karaliuskas R, Duerr RH, Achkar JP, Brant SR, Bayless TM, Kirschner BS, Hanauer SB, Nuñez G, Cho JH. A frameshift mutation in NOD2 associated with susceptibility to Crohn’s disease. Nature. 2001;411:603–6.

    Article  CAS  PubMed  Google Scholar 

  • Ono Y, Hirai F, Matsui T, Beppu T, Yano Y, Takatsu N, Takaki Y, Nagahama T, Hisabe T, Yao K, Higashi D, Futami K. Value of concomitant endoscopic balloon dilation for intestinal stricture during long-term infliximab therapy in patients with Crohn’s disease. Dig Endosc. 2012;24(6):432–8.

    Article  PubMed  Google Scholar 

  • Pacilli M, Eaton S, Fell JM, Rawat D, Clarke S, Haddad MJ. Surgery in children with Crohn disease refractory to medical therapy. J Pediatr Gastroenterol Nutr. 2011;52(3):286–90.

    Article  PubMed  Google Scholar 

  • Palder SB, Shandling B, Bilik R, Griffiths AM, Sherman P. Perianal complications of pediatric Crohn’s disease. J Pediatr Surg. 1991;26:513–5.

    Article  CAS  PubMed  Google Scholar 

  • Piekkala M, Pakarinen M, Ashorn M, Rintala R, Kolho KL. Long-term outcomes after surgery on pediatric patients with Crohn disease. J Pediatr Gastroenterol Nutr. 2013;56(3):271–6.

    Article  PubMed  Google Scholar 

  • Sandborn WJ, Phillips SF. Pathophysiology of symptoms and clinical features of inflammatory bowel disease. In: Kirsner JB, Shorter RG, editors. Inflammatory bowel disease. 4th ed. Baltimore: Williams & Wilkins; 1995. p. 407–28.

    Google Scholar 

  • Santos MPC, Gomes C, Torres J. Familial and ethnic risk in inflammatory bowel disease. Ann Gastroenterol. 2018;31(1):14–23.

    PubMed  Google Scholar 

  • Sawczenko A, Sandhu BK, Logan RF, Jenkins H, Taylor CJ, Mian S, Lynn R. Prospective survey of childhood inflammatory bowel disease in the British Isles. Lancet. 2001;357:1093–4.

    Article  CAS  PubMed  Google Scholar 

  • Shapiro M, Greenstein AJ, Byrn J, Corona J, Greenstein AJ, Salky B, Harris MT, Divino CM. Surgical management and outcomes of patients with duodenal Crohn’s disease. J Am Coll Surg. 2008;207(1):36–42.

    Article  PubMed  Google Scholar 

  • Sherlock ME, Griffiths AM. Medical therapy for pediatric inflammatory bowel disease. Curr Gastroenterol Rep. 2012;14:166–73.

    Article  PubMed  Google Scholar 

  • Shivananda S, Lennard-Jones J, Logan R, Fear N, Price A, Carpenter L, van Blankenstein M. Incidence of inflammatory bowel disease across Europe: is there a difference between north and south? Results of the European Collaborative Study on Inflammatory Bowel Disease (EC-IBD). Gut. 1996;39(5):690–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Turunen P, Kolho KL, Auvinen A, Iltanen S, Huhtala H, Ashorn M. Incidence of inflammatory bowel disease in Finnish children, 1987–2003. Inflamm Bowel Dis. 2006;12:677–83.

    Article  PubMed  Google Scholar 

  • Van Assche G, Dignass A, Reinisch W, van der Woude CJ, Sturm A, De Vos M, Guslandi M, Oldenburg B, Dotan I, Marteau P, Ardizzone A, Baumgart DC, D’Haens G, Gionchetti P, Portela F, Vucelic B, Söderholm J, Escher J, Koletzko S, Kolho KL, Lukas M, Mottet C, Tilg H, Vermeire S, Carbonnel F, Cole A, Novacek G, Reinshagen M, Tsianos E, Herrlinger K, Oldenburg B, Bouhnik Y, Kiesslich R, Stange E, Travis S, Lindsay J. The second European evidence-based consensus on the diagnosis and management of Crohn’s disease: special situations. J Crohns Colitis. 2010;4:63–101.

    Article  PubMed  Google Scholar 

  • van Balkom KA, Beld MP, Visschers RG, van Gemert WG, Breukink SO. Long-termresults after restorative proctocolectomy with ileal pouch-anal anastomosis at a young age. Dis Colon Rectum. 2012;55:939–47.

    Google Scholar 

  • Van Limbergen J, Russell RK, Drummond HE, Aldhous MC, Round NK, Nimmo ER, Smith L, Gillett PM, McGrogan P, Weaver LT, Bisset WM, Mahdi G, Arnott ID, Satsangi J, Wilson DC. Definition of phenotypic characteristics of childhood-onset inflammatory bowel disease. Gastroenterology. 2008;135:1114–22.

    Article  PubMed  Google Scholar 

  • Vernier-Massouille G, Balde M, Salleron J, Turck D, Dupas JL, Mouterde O, Merle V, Salomez JL, Branche J, Marti R, Lerebours E, Cortot A, Gower-Rousseau C, Colombel JF. Natural history of pediatric Crohn’s disease: a population-based cohort study. Gastroenterology. 2008;135:1106–13.

    Article  PubMed  Google Scholar 

  • Yu YR, Rodriguez JR. Clinical presentation of Crohn’s, ulcerative colitis, and indeterminate colitis: symptoms, extraintestinal manifestations, and disease phenotypes. Semin Pediatr Surg. 2017;26(6):349–55.

    Article  PubMed  Google Scholar 

  • Zimmerman L, Bousvaros A. The pharmacotherapeutic management of pediatric Crohn’s disease. Expert Opin Pharmacother. 2019;20:2161–8.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Risto J. Rintala .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer-Verlag GmbH Germany, part of Springer Nature

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Rintala, R.J., Pakarinen, M.P., Koivusalo, A. (2020). Crohn’s Disease. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_107-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-38482-0_107-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-38482-0

  • Online ISBN: 978-3-642-38482-0

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics