Clinical manifestations of Crohn disease in children and adolescents
- Mala Setty, MD
Mala Setty, MD
- Assistant Professor, UCSF Department of Pediatrics;
- Associate Gastroenterologist, UCSF Benioff Children's Hospital Oakland
- George H Russell, MD, MS
George H Russell, MD, MS
- Research staff (Assistant professor)
- Harvard Medical School
- Athos Bousvaros, MD
Athos Bousvaros, MD
- Associate Professor of Pediatrics
- Harvard Medical School
- Section Editors
- Kathleen J Motil, MD, PhD
Kathleen J Motil, MD, PhD
- Section Editor — Pediatric Nutrition
- Professor of Pediatric Nutrition
- Baylor College of Medicine
- Melvin B Heyman, MD, MPH
Melvin B Heyman, MD, MPH
- Section Editor — Gastroenterology
- Professor of Pediatrics
- University of California, San Francisco
Crohn disease (CD, also known as regional enteritis) is an immune-mediated inflammatory disease that can affect any portion of the intestinal tract from the mouth to the anus. The disease typically involves the ileum, ileum and cecum, or ileum and entire colon. In some cases, the disease is found only in the colon, making it difficult to distinguish from ulcerative colitis (UC). Gastritis and upper intestinal tract inflammation are present in 30 percent of patients and may or may not be related to the underlying inflammatory bowel disease (IBD). (See "Definition, epidemiology, and risk factors in inflammatory bowel disease".)
The incidence of CD is approximately 5 to 10 new cases per 100,000 individuals/year and appears to be rising . Approximately 20 to 25 percent of these cases are diagnosed in children younger than age 18 years [2-4]. Although the exact pathophysiology has not been fully defined, there is both a genetic and environmental component. It is likely that inherited gene mutations may predispose individuals with CD to an inflammatory response directed against intraluminal antigens (including intestinal bacteria, dietary antigens, and environmental antigens such as tobacco smoke). Environmental risk factors under investigation include geographic trends, early exposures (breastfeeding, antibiotics), infections, and diet. (See "Epidemiology and environmental factors in inflammatory bowel disease in children and adolescents" and "Genetic factors in inflammatory bowel disease".)
The clinical manifestations of CD in children and adolescents are reviewed here. The diagnosis of CD, including the differentiation from UC and the management of CD are discussed separately. (See "Clinical presentation and diagnosis of inflammatory bowel disease in children" and "Overview of the management of Crohn disease in children and adolescents".)
Children with CD may present with intestinal and/or extraintestinal manifestations (table 1). Intestinal manifestations are more common. In one of the largest series, which included 891 children ages 6 to 17 years, the most common presenting symptoms were :
●Abdominal pain – 44 percent
- Kappelman MD, Moore KR, Allen JK, Cook SF. Recent trends in the prevalence of Crohn's disease and ulcerative colitis in a commercially insured US population. Dig Dis Sci 2013; 58:519.
- 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.
- 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.
- Kappelman MD, Rifas-Shiman SL, Kleinman K, et al. The prevalence and geographic distribution of Crohn's disease and ulcerative colitis in the United States. Clin Gastroenterol Hepatol 2007; 5:1424.
- Gupta N, Bostrom AG, Kirschner BS, et al. Presentation and disease course in early- compared to later-onset pediatric Crohn's disease. Am J Gastroenterol 2008; 103:2092.
- Silverberg MS, Satsangi J, Ahmad T, et al. Toward an integrated clinical, molecular and serological classification of inflammatory bowel disease: report of a Working Party of the 2005 Montreal World Congress of Gastroenterology. Can J Gastroenterol 2005; 19 Suppl A:5A.
- 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.
- Gupta N, Bostrom AG, Kirschner BS, et al. Incidence of stricturing and penetrating complications of Crohn's disease diagnosed in pediatric patients. Inflamm Bowel Dis 2010; 16:638.
- Dubinsky MC, Lin YC, Dutridge D, et al. Serum immune responses predict rapid disease progression among children with Crohn's disease: immune responses predict disease progression. Am J Gastroenterol 2006; 101:360.
- Pfefferkorn MD, Marshalleck FE, Saeed SA, et al. NASPGHAN clinical report on the evaluation and treatment of pediatric patients with internal penetrating Crohn disease: intraabdominal abscess with and without fistula. J Pediatr Gastroenterol Nutr 2013; 57:394.
- Schaefer ME, Machan JT, Kawatu D, et al. Factors that determine risk for surgery in pediatric patients with Crohn's disease. Clin Gastroenterol Hepatol 2010; 8:789.
- Markowitz J, Grancher K, Rosa J, et al. Highly destructive perianal disease in children with Crohn's disease. J Pediatr Gastroenterol Nutr 1995; 21:149.
- Keljo DJ, Markowitz J, Langton C, et al. Course and treatment of perianal disease in children newly diagnosed with Crohn's disease. Inflamm Bowel Dis 2009; 15:383.
- Wong S, Lemberg DA, Day AS. Exclusive enteral nutrition in the management of perianal Crohn's disease in children. J Dig Dis 2010; 11:185.
- Tolia V. Perianal Crohn's disease in children and adolescents. Am J Gastroenterol 1996; 91:922.
- Harty S, Fleming P, Rowland M, et al. A prospective study of the oral manifestations of Crohn's disease. Clin Gastroenterol Hepatol 2005; 3:886.
- Pittock S, Drumm B, Fleming P, et al. The oral cavity in Crohn's disease. J Pediatr 2001; 138:767.
- Galbraith SS, Drolet BA, Kugathasan S, et al. Asymptomatic inflammatory bowel disease presenting with mucocutaneous findings. Pediatrics 2005; 116:e439.
- Ramaswamy K, Jacobson K, Jevon G, Israel D. Esophageal Crohn disease in children: a clinical spectrum. J Pediatr Gastroenterol Nutr 2003; 36:454.
- Ammoury RF, Pfefferkorn MD. Significance of esophageal Crohn disease in children. J Pediatr Gastroenterol Nutr 2011; 52:291.
- Ruuska T, Vaajalahti P, Arajärvi P, Mäki M. Prospective evaluation of upper gastrointestinal mucosal lesions in children with ulcerative colitis and Crohn's disease. J Pediatr Gastroenterol Nutr 1994; 19:181.
- Motil KJ, Grand RJ, Davis-Kraft L, et al. Growth failure in children with inflammatory bowel disease: a prospective study. Gastroenterology 1993; 105:681.
- Kanof ME, Lake AM, Bayless TM. Decreased height velocity in children and adolescents before the diagnosis of Crohn's disease. Gastroenterology 1988; 95:1523.
- Paerregaard A, Uldall Urne F. Anthropometry at the time of diagnosis in Danish children with inflammatory bowel disease. Acta Paediatr 2005; 94:1682.
- Sawczenko A, Sandhu BK. Presenting features of inflammatory bowel disease in Great Britain and Ireland. Arch Dis Child 2003; 88:995.
- Vasseur F, Gower-Rousseau C, Vernier-Massouille G, et al. Nutritional status and growth in pediatric Crohn's disease: a population-based study. Am J Gastroenterol 2010; 105:1893.
- Gupta N, Bostrom AG, Kirschner BS, et al. Gender differences in presentation and course of disease in pediatric patients with Crohn disease. Pediatrics 2007; 120:e1418.
- Mittermaier C, Dejaco C, Waldhoer T, et al. Impact of depressive mood on relapse in patients with inflammatory bowel disease: a prospective 18-month follow-up study. Psychosom Med 2004; 66:79.
- Persoons P, Vermeire S, Demyttenaere K, et al. The impact of major depressive disorder on the short- and long-term outcome of Crohn's disease treatment with infliximab. Aliment Pharmacol Ther 2005; 22:101.
- Limsrivilai J, Stidham RW, Govani SM, et al. Factors That Predict High Health Care Utilization and Costs for Patients With Inflammatory Bowel Diseases. Clin Gastroenterol Hepatol 2016.
- PRESENTING SYMPTOMS
- INTESTINAL DISEASE
- Disease location and classification
- ORAL AND PERIANAL LESIONS
- Perianal lesions
- Oral lesions
- Isolated orofacial and perianal findings
- ESOPHAGEAL AND GASTRIC DISEASE
- Growth failure
- Micronutrient deficiencies
- EXTRAINTESTINAL MANIFESTATIONS