Eur J Pediatr Surg
DOI: 10.1055/s-0037-1605349
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Pediatric Intestinal Rehabilitation and Transplantation Registry: Initial Report from a European Collaborative Registry

Giorgia Totonelli1, *, Roberto Tambucci1, 2, *, Alessandro Boscarelli1, Dominique Hermans3, Luigi Dall'Oglio4, Antonella Diamanti5, Aloys du Bois d'Aische6, Mikko Pakarinen7, Raymond Reding2, Francesco Morini1, Pietro Bagolan1, Fabio Fusaro1
  • 1Department of Medical and Surgical Neonatology, Bambino Gesù Children's Research Hospital, Rome, Italy
  • 2Pediatric Surgery and Transplantation Unit, Department of Surgery, Saint-Luc University Clinics, Université catholique de Louvain, Brussels, Belgium
  • 3General Pediatric and Nutrition Unit, Department of Pediatrics, Saint-Luc University Clinics, Université catholique de Louvain, Brussels, Belgium
  • 4Digestive Endoscopy and Surgery Unit, Bambino Gesù Children's Research Hospital, Rome, Italy
  • 5Artificial Nutrition Unit, Bambino Gesù Children's Research Hospital, Rome, Italy
  • 6Eonix, Mons, Belgium
  • 7Department of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
Further Information

Publication History

20 April 2017

04 July 2017

Publication Date:
24 August 2017 (eFirst)

Abstract

Introduction Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database.

Materials and Methods Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994–2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off).

Results In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0–38.0); median birth weight was 2,440 g (IQR: 1,700–2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0–92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients.

Conclusion We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF.

* Both the authors contributed equally to the article.