Standards of Care

Clinical guidelines / consensus statements

For some rare or complex conditions, there are no clinical guidelines or consensus statements available at present. For some diseases, these are available but some may require review and revision.

ERNICA seeks to endorse, develop or revise existing clinical guidelines and consensus documents to ensure clinical recommendations are made available to clinicians, patients and their families based on research of the highest quality. 

Esophageal Diseases:

In 2018, the first ERNICA consensus conference took place on the management of patients with esophageal atresia and tracheoesophageal fistula. As a result of this conference, two papers were published in the European Journal of Pediatric Surgery. 

A second ERNICA consensus conference on the perioperative, surgical and long-term management of patients with long-gap esophageal atresia took place in November 2019. 

See the resources page for more information. 

Intestinal Diseases:

The ERNICA guideline for the management of rectosigmoid Hirschsprung's disease was published online in the Orphanet Journal for Rare Diseases in June 2020. See the resources page for more information. 

Discussions have also started in regards to the development of a guideline on total colonic aganglionosis. 

Abdominal wall defects:

Discussions have started in regards to the development of a guideline on gastroschisis.

Patient Journeys

ERNICA clinicians and patient representatives within the esophageal diseases working group have been developing a 'patient journey' for esophageal atresia. This project is led by patient representatives from ‘EAT’, the international federation of esophageal atresia support groups. 

The project sets out to describe the patient's journey at a number of key stages in the patient’s life from possible pre-natal diagnosis to adulthood, identifying key issues and relevant good practice at each stage. 

The development of a Hirschsprung disease 'Patient Journey' has also now just begun within the Intestinal Diseases working group.