Original Article
Long-term health-related quality of life after complex and/or complicated esophageal atresia in adults and children registered in a German patient support group

https://doi.org/10.1016/j.jpedsurg.2013.11.068Get rights and content

Abstract

Background

Health-related quality of life (HRQoL) after esophageal atresia (EA) repair is postulated to be good. However, little is known about the long-term results after repair of complex and/or complicated EA regarding HRQoL. We investigated long-term HRQoL after delayed anastomosis, esophageal replacement, major revisions, or multiple dilatations in patients registered in a support group.

Methods

Patients registered in the German patient support group database (KEKS) were enrolled and allocated to subgroups according to surgical treatment and age. HRQoL was evaluated using validated questionnaires (GIQLI, WHO-5, KIDSCREEN27).

Results

Complete follow-up (mean 14.5 ± 9.8 years) was available for 90/92 patients. Patients were allocated to subgroups delayed anastomosis (n = 28), esophageal replacement (n = 27), major revisions (n = 15), and multiple dilatations (n = 20). Adult patients presented with impaired well-being according to WHO-score and gastrointestinal function (GIQLI). In contrast, HRQoL of children was comparable to controls in most KIDSCREEN27-dimensions. Delayed anastomosis was associated with most-favourable HRQoL. Regarding physical well-being, these children scored significantly better than controls [64.01 ± 10.40 vs. 52.36 ± 8.73;p = 0.0011], children after replacement [51.40 ± 5.70;p = 0.008], revisions [52.04 ± 6.97;p = 0.026], and multiple dilatations [50.22 ± 9.67,p = 0.04].

Conclusions

HRQoL after complex and/or complicated EA is excellent in children registered in a patient support group. In adults, disease-specific symptoms negatively affect HRQoL. Our data indicate that saving the esophagus may achieve the best HRQoL.

Section snippets

Patients and methods

This study was approved by the Institutional Ethics Committee (approval number 1245-2011).

All patients with cEA identified in the data base of the support group KEKS and treated between 10/1964 and 07/2011 were enrolled.

A signed informed consent was obtained from each patient or their guardians for their participation and to request their hospital records from the involved departments of pediatric surgery or general practitioners. Our study collective included patients with (1) delayed

Results

Ninety-two patients were accessible for follow-up. Ninety of these (98%; 63 children, 27 adults) agreed to participate, mean follow-up was 14.5 ± 9.8 years. Primary operations had been performed in 40 institutions. According to surgical treatment, patients were allocated to the subgroups delayed esophageal anastomosis (n = 28), esophageal replacement (n = 27), major surgical revisions (n = 15), and esophageal stenosis after anastomosis requiring > 10 dilatations (n = 20).

Discussion

Esophageal atresia is a heterogeneous entity ranging from uncomplicated short-gap to complex long-gap EA. Irrespective of the gap length, any type of EA may result in a complicated course requiring special interventions. We investigated patients who had undergone reconstruction of complex EA or who had a complicated course after anastomosis. Our definition of a complicated course of EA includes not only long-gap EA mostly resulting in delayed esophageal anastomosis, but also patients undergoing

Acknowledgments

The authors wish to thank Prof. Dr. Karin Lange, Department of Medical Psychology, Hannover Medical School, Hannover, Germany, for expert advice on selecting adequate questionnaires. Furthermore, we wish to thank all participating units of Pediatric Surgery for supporting this study by providing the requested patient data.

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    Disclosure: The authors have no potential conflicts of interest to disclose (financial, professional, or personal).

    1

    Both authors contributed equally to this work and are listed in alphabetical order.

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