Follow-up and transition practices in esophageal atresia: a review of European Reference Network on rare Inherited and Congenital Anomalies (ERNICA) centres and affiliates

Abstract

Purpose The purpose of this study was to understand the provision and distribution of esophageal atresia (EA) follow-up (FU) and transition services across European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) member and affiliate centers. Methods A REDCap questionnaire was sent to clinical leads of 18 ERNICA members and 14 affiliate centers. Results 29 of 32 centers responded (91%), the majority of which were highly specialized. Two-thirds had a dedicated EA clinic with a specialist multi-disciplinary team (MDT), offered to selected/complex patients only in 40% of centers. ERNICA centers were more likely to offer an MDT FU clinic than affiliates, with lack of resources most cited as a barrier to uptake (67%). Delivery of routine investigations was heterogeneous, particularly provision of three endoscopies over the course of FU (24%). Only 55% had a dedicated transition pathway, more prevalent in ERNICA centers (81% vs. 30%; p < 0.01). Self-reported awareness of ERNICA and European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) guidance for FU and transition was poor (28%). Conclusion Despite the existence of European follow-up and transition guidelines, their delivery is not uniform and may be limited by lack of awareness of the guidelines and a lack of resources.