Original Article
A patient led, international study of long term outcomes of esophageal atresia: EAT 1

https://doi.org/10.1016/j.jpedsurg.2017.05.033Get rights and content

Abstract

Introduction

Long term outcomes of esophageal atresia (OA) are poorly understood. The Federation of Esophageal Atresia and Tracheo-Esophageal Fistula support groups (EAT), a collaboration of patient support groups aimed to define patient reported long term outcomes and quality of life (QoL) in a large international cohort of OA patients.

Methods

Questionnaires were designed focusing on patient/parent reported outcomes including surgical history, current symptomatology and quality of life. Members of support groups within EAT were invited to complete questionnaires electronically via SurveyMonkey®.

Results

1100 patients from 25 countries responded to the questionnaire and 928 were analyzed. 80% had type C anatomy, 19% type A and 1% type E. Patient ages were < 5 years (42%), 5–10 years (26%), 11–17 years (16%) and 18 years and older (16%). 49% of all patients reported previous dilatations which was similar across age groups. Reflux symptoms affected 58% of patients and persisted into adulthood. Dysphagia also persisted in the adult population with 50% reporting sometimes or often getting food stuck. Reflux was significantly more frequent in ‘long gap’ versus ‘standard gap’ patients (p < 0.005). Respiratory symptoms and chest infections decreased in frequency with age. In children median SDS for height was − 0.41 (IQR − 1.4 to 0.67) and that for weight was − 0.63 (− 1.6 to 0.67). BMI in adults was 21.5. Quality of life was described as significantly affected by OA in 18% of patients while 25% reported no effect on QoL.

Conclusions

These results highlight the significant long term morbidity suffered by OA patients as children and into adulthood and suggest the need for quality transitional care. The patient designed and reported nature of the study gives a unique perspective to the results and emphasizes the benefits of collaboration.

Section snippets

Background

Outcomes in esophageal atresia (OA) have improved in the last 50 years with survival now of more than 90% [1], [2], [3] leading to an increasing population of long term survivors. While our understanding of short term outcomes in infants with OA is good [4], [5], [6], [7], longer term functional outcomes are more poorly understood with a limited research base [8], [9], [10]. There is increasing interest therefore in longer term outcome studies, and results of such studies potentially impact

Methods

This study was conceptualized as a questionnaire which was then designed by the board of EAT (see author list) with support from a Professor of Quality of Care (MvD). It was designed to be a patient/carer focused study looking at outcomes of specific interest to those groups. The questionnaire was therefore designed to assess the following keys areas of interest:

  • 1.

    Current symptomatology

  • 2.

    Patient/carer satisfaction

  • 3.

    Quality of life (QoL)

Demographics and operative details were also obtained as part of

Demographics

1100 responses were received from an estimated 2500 members of EAT organizations invited to participate. Following exclusions, 928 responses (56% male and 44% female) were analyzed with a patient age range of 1 month to 60 years. For analysis, patients were divided into the following age groups, < 5 years (42% of responses), 5–10 years (26%), 11–17 years (16%) and 18 years and older (16%).

Responses were received from 25 different countries (Fig. 1). Smaller numbers of responses were also received from

Discussion

The EAT 1 survey offers unique, international patient directed short, medium and long term follow-up data on one the largest cohorts of OA patients in the literature to date. It highlights the clinical long term morbidity that OA patients suffer from and how these may change with age. It also importantly examines the often ignored quality of life issues faced by this patient group. The large size of this study group makes it more representative of the wider international population of OA

Acknowledgments

This collaborative work has involved many different people from the design of the study to data collection and subsequent analysis.

The advisory work and support of Prof Rene Wijnen, Head of Paediatric Surgery and Professor Monique van Dijk and their colleagues at Sofia Children's Hospital, Erasmus MC is greatly appreciated. Prof van Dijk advised on questionnaire design.

All member groups and the patients of EAT are acknowledged for their assistance in making this study possible.

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