J Pediatr Gastroenterol Nutr. 2022 Feb 1;74(2):258-266. doi: 10.1097/MPG.0000000000003335.

Tal David Berger ^1 2, Huey Miin Lee ³, Lavenya Ramasamy Padmanaban ³, Eytan Wine ⁴, Anat Yerushalmy-Feler ^2 5, Iva Hojsak ⁶, Denis Kazeka ⁷, Daniela Elena Serban ⁸, Dotan Yogev ⁹, Oren Ledder ⁹, Paolo Lionetti ¹⁰, Luca Scarallo ¹⁰, Marco Gasparetto ¹¹, Nicholas M Croft ^11 12, Erasmo Miele ¹³, Annamaria Staiano ¹³, Joseph Meredith ¹⁴, Marina Aloi ¹⁵, Patrizia Alvisi ¹⁶, Darja Urlep ¹⁷, Batia Weiss ^1 2, Mikkel Malham ¹⁸, Manar Matar ^2 19, Víctor Manuel Navas-López ²⁰, Claudio Romano ²¹, Valeria Dipasquale ²¹, Lorenzo Norsa ²², Kaija-Leena Kolho ²³, Raanan Shamir ^2 19, Dror S Shouval ^2 19, Paediatric IBD Porto Group of ESPGHAN

Author information

¹Pediatric Gastroenterology Unit, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Ramat Gan.

²Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.

³Paediatric Liver, GI & Nutrition Centre and Mowat Labs, King's College Hospital NHS Foundation Trust, London, UK.

⁴Edmonton Pediatric IBD Clinic, University of Alberta, Edmonton, Canada.

⁵Pediatric Gastroenterology Unit, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel-Aviv, Israel.

⁶Children's Hospital Zagreb, University of Zagreb School of Medicine, University of J. J. Strossmayer School of Medicine Osijek, Croatia.

⁷Department of Paediatrics, University Hospital Motol, Prague, Czech Republic.

⁸2nd Clinic of Pediatrics, "Iuliu Hatieganu" University of Medicine and Pharmacy, Emergency Clinical Hospital for Children, Cluj-Napoca, Romania.

⁹Juliet Keidan Institute of Paediatric Gastroenterology and Nutrition, Shaare Zedek Medical Center, affiliated to the Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.

¹⁰Department Neurofarba, University of Florence, Meyer Children Hospital, Florence, Italy.

¹¹The Royal London Children's Hospital, Barts' Health NHS Trust, London.

¹²Barts and the London School of Medicine, Queen Mary University of London, London, UK.

¹³Department of Translational Medical Science, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.

¹⁴Department of Paediatric Gastroenterology and Nutrition, Royal Hospital for Sick Children, Edinburgh, UK.

¹⁵Pediatric Gastroenterology and Liver Unit, Sapienza University of Rome, Rome.

¹⁶Pediatric Unit, Maggiore Hospital, Bologna, Italy.

¹⁷Department of Gastroenterology Hepatology and Nutrition, Children's Hospital University Medical Centre Ljubljana, Ljubljana, Slovenia.

¹⁸The pediatric Department, Copenhagen University Hospital, Hvidovre, and The Pediatric Department, Holbaek Hospital, Denmark.

¹⁹Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

²⁰Pediatric Gastroenterology and Nutrition Unit. Hospital Regional Universitario de Málaga; Spain.

²¹Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messina, Messina, Italy.

²²Paediatric Hepatology Gastroenterology and Transplantation, Papa Giovanni XXIII Hospital, Bergamo, Italy.

²³Paediatric Gastroenterology of the Children's Hospital, University of Helsinki, Helsinki, Finland.

Abstract

Objectives: Adult studies suggest that patients with isolated colonic Crohn disease (L2 CD) exhibit unique characteristics differentiating them from patients with ileo-caecal (L1) CD and ulcerative colitis (UC). We aimed to characterize clinical features and outcomes of paediatric patients with L2.

Methods: Retrospective data was collected through the Porto Inflammatory Bowel Disease group of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) on Paediatric patients with L2, L1 or UC at different time-points. Outcome measures included time to first flare, hospital admissions, initiation of anti-tumor necrosis factor-alpha (TNFα) drug, stricture and surgery.

Results: Three hundred patients were included: 102 L1, 94 L2 and 104 UC. Rates of hematochezia at presentation were 14.7%, 44.7% and 95.2%, while rates of fever were 12.7%, 26.6% and 2.9%, for patients with L1, L2 and UC, respectively (P < 0.001 for all comparisons). Skip lesions were identified in 65% of patients with L2, and granulomas in 36%, similar to L1 patients. Rates of anti-Saccharomyces cerevisiae antibodies (ASCA) and perinuclear antineutrophil cytoplasmic (pANCA) positivity significantly differed between the three groups: 25.4% and 16.7% for patients with L2, compared with 55.2% and 2.3%, and 1.8% and 52.9% for patients with L1 and UC, respectively. Response rates to exclusive enteral nutrition were comparable between L1 and L2 (78.3-82.4%), as was the response to oral steroids (70.4-76.5%) in the three groups. While times to first flare and admission were similar between groups, patients with L1 were commenced on anti-TNFα earlier. Moreover, stricturing phenotype and need for colectomy were very rare in patients with L2.

Conclusions: Significant differences are observed in the clinical presentation and outcomes of Paediatric patients with L2, compared to patients with L1 and UC.