Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study

Inflamm Bowel Dis. 2020 May 9;izaa080. doi: 10.1093/ibd/izaa080. Online ahead of print.

Basavaraj Kerur 1, Eric I Benchimol 2, Karoline Fiedler 3, Marisa Stahl 4, Jeffrey Hyams 5, Michael Stephens 6, Ying Lu 7, Marian Pfefferkorn 8, Raza Alkhouri 9, Jennifer Strople 10, Judith Kelsen 11, Leah Siebold 12, Alka Goyal 13, Joel R Rosh 14, Neal LeLeiko 15, Johan Van Limbergen 16, Anthony L Guerrerio 17, Ross Maltz 18, Lina Karam 19, Eileen Crowley 20, Anne Griffiths 3, Melvin B Heyman 21, Mark Deneau 22, Keith Benkov 23, Joshua Noe 24, Dedrick Mouton 25, Helen Pappa 26, Joseph A Galanko 27, Scott Snapper 28, Aleixo M Muise 3, Michael D Kappelman 27


Author information

1University of Massachusetts Medical School (UMMS), Worcester, MA, United States.

2Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

3The Hospital for Sick Children, Toronto, ON, Canada.

4Children's Hospital Colorado, Denver, CO, United States.

5Connecticut Children's Medical Center, Hartford, CT, United States.

6Center for Individualized Medicine, Mayo Clinic, Rochester, MN, United States.

7Cohen Children's Medical Center of New York, New York, NY, United States.

8Riley Children's Hospital, Indianapolis, IN, United States.

9University at Buffalo, Buffalo, NY, United States.

10Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States.

11Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.

12UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, United States.

13Children's Mercy, Kansas City, MO, United States.

14Goryeb Children's Hospital/Atlantic Health System, Morristown, NJ, United States.

15Hasbro Children's Hospital, Providence, RI, United States.

16Amsterdam University Medical Centres, Emma Children's Hospital, Amsterdam, Netherlands.

17Johns Hopkins University School of Medicine Baltimore, MD, United States.

18Nationwide Children's Medical Center, Columbus, OH, United States.

19Texas Children's Hospital, Houston, TX, United States.

20Children's Hospital, London Health Science Centre, University of Western Ontario, London, Ontario, Canada.

21UCSF Benioff Children's Hospital, San Francisco, CA, United States.

22University of Utah/Intermountain Primary Children's Hospital, Salt Lake City, UT, United States.

23Icahn School of Medicine at Mount Sinai, New York, NY, United States.

24Medical College of Wisconsin, Milwaukee, WI, United States.

25Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, United States.

26Cardinal Glennon Children's Hospital, St. Louis, MO, United States.

27University of North Carolina in Chapel Hill, Chapel Hill, NC, United States.

28Children's Hospital Boston, Boston, MA, United States.


Background: The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described.

Methods: We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years.

Results: The study population included 269 children (105 [39%] Crohn's disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9-5.2). Most (94%) Crohn's disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn's disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis.

Conclusions: Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%-15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.

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