ERNICA is a network of expert multi-disciplinary healthcare professionals from specialist centers across Europe, partnered with patient advocacy and support groups who provide invaluable input from a patient perspective. Its main aim is to pool disease-specific expertise, knowledge, and resources on congenital anomalies to reduce health inequalities by standardizing practices, facilitating research and disseminating high-quality information. To that end, ERNICA consensus statements for the ‘Follow-Up and Framework’ of EA were formulated in 2018 in conjunction with EA patient organizations, based on the highest grade of evidence available [6]. Pre-existing ESPGHAN–NASPGHAN guidance for evaluation and treatment of gastrointestinal and nutritional complications in EA children were taken into consideration during consensus development, with ERNICA guidance covering more surgical aspects of follow-up (e.g., consideration of indication for fundoplication, treatment of recalcitrant strictures, etc.) compared with the mainly gastroenterological perspective of the ESPGHAN–NASPGHAN guidance [7].

Although ERNICA centers self-reported a higher volume of new EA cases per annum, provision of highly specialized surgical services (e.g., re-do/replacement surgery) as well as excellent availability of EA-associated medical and allied specialties were offered by both ERNICA and affiliate centers. Despite this, only two-thirds used these resources within an MDT clinic setting for EA patients, which was often only utilized for complex patients alone, rather than available to all. Whilst complex cases (e.g., recurrent fistula, re-do surgery, recalcitrant strictures, or long-gap EA) clearly require multi-disciplinary input [9], the high presence of associated pathologies, such as dysphasia, GERD, nutritional difficulties, and respiratory problems, which are often asymptomatic and may go unrecognized, indicate why speech and language therapy, gastroenterology, dietary, and pulmonology input and assessment as a minimum should be offered to all patients. In addition to the clinical need, it is also what EA patients and their families have asked for; focus groups have highlighted that patients and their parents want standardized MDT follow-up programmed during both child and adulthood under the guidance of one coordinating physician, to better understand all the aspects and consequences of EA. This expertise was felt to be lacking in general practitioners/primary care physicians [10, 11]. This study highlighted relative homogeneity in the self-reported responsible clinician across European centers, with 90% being pediatric surgeons.

The provision of routine investigations in asymptomatic EA patients was surprisingly heterogeneous and varied widely from that of both the ERNICA and ESPGHAN–NASPGHAN guidance; both recommend a pH/impedance study, LFTs, and a total of three endoscopies over the course of childhood follow-up. Of note, neither endorse barium swallow or bronchoscopy as a matter of routine. On the whole, ERNICA centers appeared to follow guidance more closely than affiliate centers, although the delivery of three endoscopies during childhood follow-up was universally poor, followed by only 24% of centers. With histological evidence of Barrett’s oesophagus found to be as high as 43% in the adolescent EA population [12], this approach should be strongly encouraged. Interestingly, on a survey of individual expert agreement with ESPGHAN guidance sent to ESPGHAN, NASPGHAN, and EUPSA members, 91% of those surveyed agreed in principle with the provision of three endoscopies, although from our survey, this does not appear to be currently offered in centers coordinating EA follow-up, highlighting a discordance between what expert clinicians think and what is actually being done [13]. Both ERNICA and ESPGHAN–NASPGHAN guidances also recommend the routine prescription of PPIs for all patients post-operatively, with the provision of a pH/impedance study at discontinuation of therapy. Whilst uptake of PPI prescription was widely followed post-EA-repair (86%), this was discontinued without a pH/impedance study, the gold standard for GERD diagnosis, in nearly half the centers. These results were consistent with that found in the INoEA survey, where there was high agreement that PPIs should be prescribed post-operatively but low agreement on the use of pH manometry on discontinuation [13].

Overall, just over half the centres provided formal transition pathways, with relative uniformity of implementation, with the majority reporting handover to adult gastroenterologists at 18 years of age. Transition was more established within ERNICA centers, which were more likely to offer dedicated transition clinics with adult specialists and adolescent education pathways. Our results correlate with the published experience of the self-reported provision of transitional care within congenital colorectal diseases, which was found to be present in only 44% of centers interviewed [14]. It also correlates with the documented current adult EA experience; several studies have highlighted a paucity of adult EA care, with one international study of 1100 adult patients documenting that 50% had no follow-up [3, 4]. With high rates of gastrointestinal and respiratory morbidity and increasing reports of esophageal carcinoma within the adult EA cohort [15, 16], the clinical need for this is unquestionable. However, the holistic benefit of transition for the patient and family should also not be underestimated. A large qualitative meta-synthesis of adolescent transition experiences on transfer from pediatric to adolescent care describes feelings of not belonging and being redundant to the process [17]. This was echoed in an EA-specific cross-sectional qualitative survey of patients and parents undergoing transition; five themes of feelings of abandonment, a cultural shift, a shift in responsibility, living with uncertainty, and a lack of any transition process were described [18]. The need for young adults to be valued as collaborators in their transfer to adult care is crucial, especially as the general transition between adolescence and adulthood is frequently associated with the desire to become independent from parents, caregivers, and those in perceived positions of authority as rapidly as possible. There are multiple examples of how structured transition pathways and education programs to guide adolescents through the transition process improve patient knowledge and improve outcomes, both in EA specifically, and other chronic pediatric conditions, such as inflammatory bowel disease, cystic fibrosis, asthma, and diabetes [19, 20].

As the ERNICA consensus statements and ESPGHAN–NASPGHAN guidelines are not standards, they are at the discretion of the clinician or unit to follow; however, most clinicians would not argue with the need for MDT follow-up and formalized transition care. What, then, are the barriers to achieving these more widely for all EA patients? The surprisingly low awareness of the consensus statements, although slightly higher in ERNICA centers themselves, may partially explain the variability in their uptake. A lack of resources, as alluded to by centers where MDT clinics did not exist, may also contribute to difficulty in provision of the recommendations from consensus statements and guidelines, although it was encouraging to note that the majority had plans to establish MDT clinics within the next five years. It may be that these recommendations are unattainable for centers with less accessibility to specialist investigations, and affiliate centers should be included in the future guideline revision to reflect this and offer alternative solutions, including clear criteria of when to refer patients to expert centres for specialist investigations and/or treatment. Finally, the existence guidelines with differing recommendations for the routine follow-up of the asymptomatic EA child (e.g., regarding PPIs for prevention of reflux) may not help clinicians determine which is the best framework to follow. Recent publication of the comprehensive INoEA guidance on transition and adult follow-up of EA patients now provides a specific and unified approach to follow for transition of EA patients into adulthood [21]. ERNICA and ESPGHAN are now working more closely together and hopefully future revisions of the ERNICA Consensus Statements and ESPGHAN–NASPGHAN Guidelines can be more closely aligned, or even amalgamated and endorsed by other relevant bodies, including INoEA, patient organizations, and national pediatric surgical and gastroenterological associations. Clear recommendations endorsed by multiple bodies will hopefully empower clinical teams to obtain the resources required for effective follow-up and transition clinics.

This initial survey has limitations—all surveys are limited by a tension between obtaining information and overburdening respondents, and often interpretation is limited by the desire to know additional information. For example, it would have been extremely useful to know what criteria are used in each center to accept patients into MDT clinics. Nevertheless, the survey provides useful information that can be taken forward to improve the knowledge of the consensus statements in expert and affiliate centers, and also to aid future development of unified guidelines across the different organizations to improve the transition of EA patients across Europe and beyond.