Registry

With the improved survival of newborns with rare inherited and congenital anomalies, clinical outcomes has become increasingly important. Therefore in 2020 ERNICA applied for and successfully obtained financial support of the European Union to develop a patient registry

ERNICA registries

ERNICA has developed its own registry in collaboration with DICA, covering eight congenital conditions: EA, Hirschsprung, Omphalocele, Gastroschisis, CDH, NEC, Malrotation, and Anorectal Malformations. Please read more about the
EPSA|ERNICA registry below.

For intestinal failure, we collaborate with the existing International Intestinal Failure Registry (IIFR), a registry for paediatric intestinal failure patients hosted by the Transplantation Society (TTS) in Montreal, Canada. Please read more below.

EPSA|ERNICA Registry

BACKGROUND INFORMATION

The European Pediatric Surgical Audit (EPSA) was developed by the Dutch Institute for Clinical Auditing in 2014 in collaboration with the Dutch Pediatric Surgical Association. Initially, it registered four diseases seen in Dutch newborn children: Hirschsprung’s disease, Congenital Diaphragmatic Hernia, Esophageal Atresia, and Anorectal Malformation (registered exclusively by Dutch centers). In 2017, the audit was extended to include patients with Omphalocele and Gastroschisis. At the beginning of 2024 Malrotation was added, and currently datasets are being developed for Necrotizing Enterocolitis and Intestinal Atresia. This registry measures structural, process, and outcome indicators for these seven diseases. In the EPSA, data is entered anonymously and then processed by MRDM in accordance with GDPR regulations.

Since then, ERNICA has obtained financial support from the European Commission to create a comprehensive registry for all European children with rare inherited diseases and congenital anomalies. This registry will build upon the EPSA, starting by expanding the registry to include more European hospitals.

REGISTRY PURPOSE

The primary aim of the EPSA | ERNICA Registry is to improve the quality of patient care. It does this by enabling healthcare providers to gain insights into their own outcomes as well as the anonymized overall outcomes of participating centers, using quality indicators selected by international experts in the field. Additionally, cumulative data in the EPSA can be used to conduct scientific research, for example, to compare treatments or identify certain risk factors for complications.

Inclusion criteria

All patients who have undergone intervention since the 1st of january 2014, because of Hirschsprung disease, Congenital Diaphragmatic Hernia, Esophageal Atrasia, Anorectal Malformations (registered exclusively by Dutch centers), Omphalocele and Gastroschisis.

Apply to register: how to sign up your Medical Center:

Do you have any questions, or do you with to participate in the EPSA | ERNICA Registy?

Please contact the audit managers:

E-mail: EPSA@dica.nl

Already registering?

For more practical information, links to the registration system and the data dictoray, visit:

http://dica.nl/epsa/home

Because the diseases registered in EPSA are rare, there is a paucity of (level 1-) evidence on these topics. ERNICA considers scientific research culminating in evidence-based medicine, of vital importance to enhance quality of care and therefore supports initiatives to use data collected in the EPSA to that end. Scientific proposals to use the data will be reviewed thoroughly and on several levels, ensuring the data is utilised carefully, optimally and resulting in sound scientific evidence.

As the EPSA | ERNICA Registry is currently being built, not all entered data is yet considered to be sufficient, accurate and complete. Following a data verification project involving all six Dutch pediatric surgical centers, the Dutch data was deemed as ready to be used for scientific purposes. At this time the rest of the data is not yet available. The application procedure is described in the attached file “Core values and Governance EPSA”

Below you can find the list of EPSA publications:

The European Pediatric Surgical Audit: improving quality of care in rare congenital malformations
Nadine M. Teunissen, Daniel Rossi, Michel W. Wouters, Simon Eaton, Ernest L.W. van Heurn, Rene M.H. Wijnen, on behalf of the EPSA|ERNICA Registry Group

Variability in the Reporting of Baseline Characteristics, Treatment, and Outcomes in Esophageal Atresia Publications: A Systematic Review.
Nadine M. Teunissen, Julia Brendel, Ernest L.W. van Heurn, Benno M. Ure, Rene M.H. Wijnen, Simon Eaton on behalf of the EPSA|ERNICA Registry Group and the EA Quality of Care Initiative

Selection of Quality Indicators to Evaluate Quality of Care for Patients with Esophageal Atresia Using a Delphi Method.
Nadine M. Teunissen, Julia Brendel, Ernest L.W. van Heurn, Benno M. Ure, Rene M.H. Wijnen, Simon Eaton on behalf of the EPSA|ERNICA Registry Group and the EA Quality of Care Initiative

Bridging the Gap: Reporting Baseline Characteristics, Process and Outcome Parameters in Hirschsprung's Disease. A Systematic Review.
Daniel Rossi, Anna Löf Granström, Nadine M. Teunissen, René M.H. Wijnen, Tomas Wester, Cornelius E.J. Sloots

Prevalence and early surgical outcome of congenital diaphragmatic hernia in the Netherlands: a population based cohort study from the European Pediatric Surgical Audit
Nadine M. Teunissen, Horst Daniels, J. Marco Schnater, Ivo de Blaauw, Rene M.H. Wijnen

Clinical Audit Board

Prof. dr. R.M.H. Wijnen, Erasmus MC, Rotterdam, chair

Prof. dr. L.W.E. van Heurn, Amsterdam MC, Amsterdam, chair

Dr. S. Eaton, UCL Great Ormond Children's Hospital, Londen, member

Prof. dr. M. Pakarinen, Helsinki University Hospital, Helsinki, member

Prof. dr. J.B.F. Hulscher, University Medical Center Groningen, Groningen, member

Prof. dr. T. Wester, Karolinska Institutet, Stockholm, member

Dr. K. Toczewski, Wroclaw University Hospital, member

Clinical Audit Managers EPSA | ERNICA Registry

Marlinde van der Kamp, MSc, Erasmus MC | DICA

Romee van Steekelenburg, MSc, Erasmus MC | DICA

Contact

For questions about organisation, research or content of the registry, contact EPSA@dica.nl.

Mission

To gather comparable, comprehensive and complete pre-, peri- and postnatal data to improve the quality of care.

The IFFR Registry

BACKGROUND INFORMATION

Intestinal failure is a rare disease, and research has historically been limited by small patient numbers at individual centers. To address this, the International Intestinal Rehabilitation and Transplant Association (IIRTA) established the International Intestinal Failure Registry (IIFR), a dedicated global platform to track outcomes and trends in intestinal failure.

A successful pilot phase involving twelve centers ran from 2018–2020. Since 2021, the registry has been actively collecting data from contributing centers worldwide.

For more information, visit the IIFR website

REGISTRY MISSION

The mission of the International Intestinal Failure Registry (IIFR) is to provide the international intestine rehabilitation and transplant community with accurate data on the outcomes and course of intestinal failure to support research, quality improvement, and policy development.

GENTLEMEN's AGREEMENT

ERNICA HCPs contributing to the IIFR registry participate under a gentlemen’s agreement, which fosters mutual understanding, cooperation, and progress. This document outlines the terms for:

Participation in the IIFR registry
Contributions to publications
Involvement in the IIFR scientific committee

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