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

• Published in: Journal of pediatric surgery 2014-04, Vol.49 (4), p.631-638
• Main Authors: Dingemann, Carmen, Meyer, Annica, Kircher, Gabriele, Boemers, Thomas M, Vaske, Bernhard, Till, Holger, Ure, Benno M
• Format: Article
• Language: English
• Subjects: Adult; Age Factors; Anastomosis, Surgical; Case-Control Studies; Child; Child, Preschool; Children; Complicated esophageal atresia; Esophageal Atresia - complications; Esophageal Atresia - surgery; Esophagoplasty - methods; Female; Follow-Up Studies; Germany; Humans; Male; Patient support group; Pediatrics; Quality of Life; Questionnaires; Registries; Reoperation; Self-Help Groups; Surgery; Surveys and Questionnaires; Symptoms; Treatment Outcome
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2013.11.068

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.