title: 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.
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.
authors.affiliation: Center of Pediatric Surgery, Hannover Medical School and Bult Children's Hospital, Hannover, Germany. Electronic address: dingemann.carmen@mh-hannover.de.
authors.forename: Carmen
authors.initials: C
authors.lastname: Dingemann
authors.affiliation: Center of Pediatric Surgery, Hannover Medical School and Bult Children's Hospital, Hannover, Germany.
authors.forename: Annica
authors.initials: A
authors.lastname: Meyer
authors.affiliation: German support group for patients with diseased esophagus "KEKS", Stuttgart, Germany.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Kircher
authors.affiliation: Department of Pediatric Surgery and Pediatric Urology, Children's Hospital of Cologne, Cologne, Germany.
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Boemers
authors.affiliation: Institute of Biostatistics, Hannover Medical School, Hannover, Germany.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Vaske
authors.affiliation: Department of Pediatric and Adolescent Surgery, Medical University of Graz, Austria.
authors.forename: Holger
authors.initials: H
authors.lastname: Till
authors.affiliation: Center of Pediatric Surgery, Hannover Medical School and Bult Children's Hospital, Hannover, Germany.
authors.forename: Benno M
authors.initials: BM
authors.lastname: Ure
mesh_terms: D000328:Adult; D000367:Age Factors; D000714:Anastomosis, Surgical; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004933:Esophageal Atresia / Q000150:complications / Q000601:surgery*; D004944:Esophagoplasty / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D008297:Male; D011788:Quality of Life; D012042:Registries; D012086:Reoperation; D012657:Self-Help Groups; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
keywords: Children; Complicated esophageal atresia; Patient support group; Quality of life; Questionnaires; Symptoms