Eur J Pediatr Surg 2022; 32(04): 334-345
DOI: 10.1055/s-0041-1731391
Original Article

Long-Term Outcomes and Health-Related Quality of Life in a Swiss Patient Group with Esophageal Atresia

1   Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland
,
Jessica Brestel
1   Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland
,
2   Division of Immunology, University Children's Hospital Zurich, Zurich, Switzerland
,
1   Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland
,
1   Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland
,
1   Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland
,
1   Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland
› Author Affiliations
Funding None.

Abstract

Introduction Surgical treatment of esophageal atresia (EA) has markedly improved, allowing the focus to shift from short-term complications and mortality to long-term complications and quality of life. Health-related quality of life (HRQoL) is variable and reported to range from reduced to unimpaired in patients with repaired EA. We assessed the HRQoL, determined the prevalence of long-term complications and their possible impact on the HRQoL in patients who had correction of EA in Switzerland. Further, we also investigated in the general well-being of their parents.

Materials and Methods Patients with EA repair in Switzerland between 1985 and 2011 were enrolled. Long-term complications were assessed by enquiring disease-related symptoms, standardized clinical examinations, and analysis of radiographs. HRQoL was inquired using different validated questionnaires (KIDSCREEN-27, World Health Organization [WHO]-5, and Gastrointestinal Quality of Life Index [GIQLI]). Patients were grouped according to their age. In underage patients, general well-being of the parents was assessed using the WHO-5 questionnaire.

Results Thirty patients were included with a mean age of 11.3 ± 5.7 years. Long-term complications were present in 63% of all patients. HRQoL in underage patients was comparable to the provided reference values and rated as good, while adult patients reported a reduced HRQoL. The presence of gastroesophageal reflux disease symptoms was associated with reduced HRQoL in underage patients. Parents of underage patients stated a good general well-being.

Conclusion Long-term complications among patients with repair of EA in Switzerland are common. HRQoL in underage patients is good and general well-being of their parents is unimpaired. Adult patients reported a reduced HRQoL, consistent with other reports. As long-term complications may manifest only later in life, a structured follow-up of patients with an EA repair during childhood and adolescence is needed.

Informed Consent

The authors hereby confirm that informed consent was obtained from all individual patients included in this study.


Authors' Contribution

A. di Natale: Study design and conception, performed physical examinations and acquisition of data, wrote and edited the manuscript. J. Brestel: Acquisition of data. A.A. Mauracher: Substantial contribution in statistical analysis and interpretation of data, critical revision. S.J. Tharakan: Substantial contribution in interpretation of data, critical revision, and final approval of the version to be published. M. Meuli: Critical revision and final approval of the version to be published. U. Moehrlen: Substantial contribution in interpretation of data, critical revision, and final approval of the version to be published. U. Subotic: Designed study protocol, substantial contribution in interpretation of data, and final approval of the version to be published.


Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and cantonal research committee (The local Ethics Committee of Zurich, BASEC Nr. PB_2016-00301) and with the Declaration of Helsinki 1964 and its later amendments or comparable ethical standards.


Supplementary Material



Publication History

Received: 14 February 2021

Accepted: 17 May 2021

Article published online:
29 July 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Depaepe A, Dolk H, Lechat MF. EUROCAT Working Group. The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. Arch Dis Child 1993; 68 (06) 743-748
  • 2 Cassina M, Ruol M, Pertile R. et al. Prevalence, characteristics, and survival of children with esophageal atresia: a 32-year population-based study including 1,417,724 consecutive newborns. Birth Defects Res A Clin Mol Teratol 2016; 106 (07) 542-548
  • 3 Pedersen RN, Calzolari E, Husby S, Garne E. EUROCAT Working group. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child 2012; 97 (03) 227-232
  • 4 Spitz L. Oesophageal atresia. Orphanet J Rare Dis 2007; 2: 24
  • 5 Shaw-Smith C. Oesophageal atresia, tracheo-oesophageal fistula, and the VACTERL association: review of genetics and epidemiology. J Med Genet 2006; 43 (07) 545-554
  • 6 Stoll C, Alembik Y, Dott B, Roth MP. Associated anomalies in cases with esophageal atresia. Am J Med Genet A 2017; 173 (08) 2139-2157
  • 7 Spitz L. Esophageal atresia. Lessons I have learned in a 40-year experience. J Pediatr Surg 2006; 41 (10) 1635-1640
  • 8 Orford J, Cass DT, Glasson MJ. Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s. Pediatr Surg Int 2004; 20 (06) 402-407
  • 9 Sistonen SJ, Koivusalo A, Nieminen U. et al. Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up. Ann Surg 2010; 251 (06) 1167-1173
  • 10 Zimmer J, Eaton S, Murchison LE, De Coppi P, Ure BM, Dingemann C. State of play: eight decades of surgery for esophageal atresia. Eur J Pediatr Surg 2019; 29 (01) 39-48
  • 11 Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, Pakarinen MP. Cancer after repair of esophageal atresia: population-based long-term follow-up. J Pediatr Surg 2008; 43 (04) 602-605
  • 12 Deurloo JA, van Lanschot JJ, Drillenburg P, Aronson DC. Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. J Pediatr Surg 2001; 36 (04) 629-630
  • 13 Adzick NS, Fisher JH, Winter HS, Sandler RH, Hendren WH. Esophageal adenocarcinoma 20 years after esophageal atresia repair. J Pediatr Surg 1989; 24 (08) 741-744
  • 14 Deurloo JA, Ekkelkamp S, Hartman EE, Sprangers MA, Aronson DC. Quality of life in adult survivors of correction of esophageal atresia. Arch Surg 2005; 140 (10) 976-980
  • 15 Jayasekera CS, Desmond PV, Holmes JA, Kitson M, Taylor AC. Cluster of 4 cases of esophageal squamous cell cancer developing in adults with surgically corrected esophageal atresia--time for screening to start. J Pediatr Surg 2012; 47 (04) 646-651
  • 16 Deurloo JA, Ekkelkamp S, Bartelsman JF. et al. Gastroesophageal reflux: prevalence in adults older than 28 years after correction of esophageal atresia. Ann Surg 2003; 238 (05) 686-689
  • 17 Rintala RJ, Sistonen S, Pakarinen MP. Outcome of esophageal atresia beyond childhood. Semin Pediatr Surg 2009; 18 (01) 50-56
  • 18 Sistonen SJ, Pakarinen MP, Rintala RJ. Long-term results of esophageal atresia: Helsinki experience and review of literature. Pediatr Surg Int 2011; 27 (11) 1141-1149
  • 19 Ijsselstijn H, van Beelen NW, Wijnen RM. Esophageal atresia: long-term morbidities in adolescence and adulthood. Dis Esophagus 2013; 26 (04) 417-421
  • 20 Kovesi T, Rubin S. Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest 2004; 126 (03) 915-925
  • 21 Wei S, Saran N, Emil S. Musculoskeletal deformities following neonatal thoracotomy: long-term follow-up of an esophageal atresia cohort. J Pediatr Surg 2017; 52 (12) 1898-1903
  • 22 Bastard F, Bonnard A, Rousseau V. et al. Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort. J Pediatr Surg 2018; 53 (04) 605-609
  • 23 Miyake H, Chen Y, Hock A, Seo S, Koike Y, Pierro A. Are prophylactic anti-reflux medications effective after esophageal atresia repair? Systematic review and meta-analysis. Pediatr Surg Int 2018; 34 (05) 491-497
  • 24 Way C, Wayne C, Grandpierre V, Harrison BJ, Travis N, Nasr A. Thoracoscopy vs. thoracotomy for the repair of esophageal atresia and tracheoesophageal fistula: a systematic review and meta-analysis. Pediatr Surg Int 2019; 35 (11) 1167-1184
  • 25 Lawal TA, Gosemann JH, Kuebler JF, Glüer S, Ure BM. Thoracoscopy versus thoracotomy improves midterm musculoskeletal status and cosmesis in infants and children. Ann Thorac Surg 2009; 87 (01) 224-228
  • 26 Peetsold MG, Heij HA, Deurloo JA, Gemke RJ. Health-related quality of life and its determinants in children and adolescents born with oesophageal atresia. Acta Paediatr 2010; 99 (03) 411-417
  • 27 Dingemann C, Meyer A, Kircher G. et al. 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. J Pediatr Surg 2014; 49 (04) 631-638
  • 28 Hölscher AC, Laschat M, Choinitzki V. et al. Quality of life after surgical treatment for esophageal atresia: long-term outcome of 154 patients. Eur J Pediatr Surg 2017; 27 (05) 443-448
  • 29 Koivusalo A, Pakarinen MP, Turunen P, Saarikoski H, Lindahl H, Rintala RJ. Health-related quality of life in adult patients with esophageal atresia--a questionnaire study. J Pediatr Surg 2005; 40 (02) 307-312
  • 30 Gibreel W, Zendejas B, Antiel RM, Fasen G, Moir CR, Zarroug AE. Swallowing dysfunction and quality of life in adults with surgically corrected esophageal atresia/tracheoesophageal fistula as infants: forty years of follow-up. Ann Surg 2017; 266 (02) 305-310
  • 31 Lepeytre C, De Lagausie P, Merrot T, Baumstarck K, Oudyi M, Dubus JC. [Medium-term outcome, follow-up, and quality of life in children treated for type III esophageal atresia]. Arch Pediatr 2013; 20 (10) 1096-1104
  • 32 Legrand C, Michaud L, Salleron J. et al. Long-term outcome of children with oesophageal atresia type III. Arch Dis Child 2012; 97 (09) 808-811
  • 33 Bal HS, Sen S, Karl S, Mathai J, Thomas RJ. An assessment of quality of life of operated cases of esophageal atresia in the community. J Indian Assoc Pediatr Surg 2016; 21 (03) 131-138
  • 34 Svoboda E, Fruithof J, Widenmann-Grolig A. et al. A patient led, international study of long term outcomes of esophageal atresia: EAT 1. J Pediatr Surg 2018; 53 (04) 610-615
  • 35 Witt S, Dellenmark-Blom M, Dingemann J. et al. Quality of life in parents of children born with esophageal atresia. Eur J Pediatr Surg 2019; 29 (04) 371-377
  • 36 Witt S, Dellenmark-Blom M, Flieder S. et al. Health-related quality of life experiences in children and adolescents born with esophageal atresia: a Swedish-German focus group study. Child Care Health Dev 2019; 45 (01) 79-88
  • 37 Flieder S, Dellenmark-Blom M, Witt S. et al. Generic health-related quality of life after repair of esophageal atresia and its determinants within a German-Swedish cohort. Eur J Pediatr Surg 2019; 29 (01) 75-84
  • 38 Dellenmark-Blom M, Quitmann J, Dingemann C. Health-related quality of life in patients after repair of esophageal atresia: a review of current literature. Eur J Pediatr Surg 2020; 30 (03) 239-250
  • 39 Hannon E, Eaton S, Curry JI, Kiely EM, Spitz L, De Coppi P. Outcomes in adulthood of gastric transposition for complex and long gap esophageal atresia. J Pediatr Surg 2020; 55 (04) 639-645
  • 40 Mikkelsen A, Boye B, Diseth TH. et al. Traumatic stress, mental health and quality of life in adolescents with esophageal atresia. J Pediatr Surg 2020; DOI: 10.1016/j.jpedsurg.2020.10.029.
  • 41 Dellenmark-Blom M, Quitmann J, Dingemann J. et al. Clinical factors affecting condition-specific quality-of-life domains in pediatric patients after repair of esophageal atresia: the Swedish-German EA-QOL study. Eur J Pediatr Surg 2020; 30 (01) 96-103
  • 42 Amin R, Knezevich M, Lingongo M. et al. Long-term quality of life in neonatal surgical disease. Ann Surg 2018; 268 (03) 497-505
  • 43 Gross RE. The Surgery of Infancy and Childhood. Philadelphia: WB Saunders Company; 1953
  • 44 Guyatt GH, Feeny DH, Patrick DL. Measuring health-related quality of life. Ann Intern Med 1993; 118 (08) 622-629
  • 45 Leidy NK, Revicki DA, Genesté B. Recommendations for evaluating the validity of quality of life claims for labeling and promotion. Value Health 1999; 2 (02) 113-127
  • 46 Matza LS, Swensen AR, Flood EM, Secnik K, Leidy NK. Assessment of health-related quality of life in children: a review of conceptual, methodological, and regulatory issues. Value Health 2004; 7 (01) 79-92
  • 47 Dellenmark-Blom M, Abrahamsson K, Quitmann JH. et al. Development and pilot-testing of a condition-specific instrument to assess the quality-of-life in children and adolescents born with esophageal atresia. Dis Esophagus 2017; 30 (07) 1-9
  • 48 Dellenmark-Blom M, Dingemann J, Witt S. et al. The esophageal-atresia-quality-of-life questionnaires: feasibility, validity and reliability in Sweden and Germany. J Pediatr Gastroenterol Nutr 2018; 67 (04) 469-477
  • 49 Dellenmark-Blom M, Chaplin JE, Gatzinsky V. et al. Health-related quality of life experiences among children and adolescents born with esophageal atresia: Development of a condition-specific questionnaire for pediatric patients. J Pediatr Surg 2016; 51 (04) 563-569
  • 50 Ravens-Sieberer U, Gosch A, Rajmil L. et al; KIDSCREEN Group. The KIDSCREEN-52 quality of life measure for children and adolescents: psychometric results from a cross-cultural survey in 13 European countries. Value Health 2008; 11 (04) 645-658
  • 51 Ravens-Sieberer U, Gosch A, Abel T. et al; European KIDSCREEN Group. Quality of life in children and adolescents: a European public health perspective. Soz Praventivmed 2001; 46 (05) 294-302
  • 52 Eypasch E, Williams JI, Wood-Dauphinee S. et al. Gastrointestinal quality of life index: development, validation and application of a new instrument. Br J Surg 1995; 82 (02) 216-222
  • 53 Ure BM, Slany E, Eypasch EP, Weiler K, Troidl H, Holschneider AM. Quality of life more than 20 years after repair of esophageal atresia. J Pediatr Surg 1998; 33 (03) 511-515
  • 54 Bech P. Clinical Psychometrics. Oxford: Wiley Blackwell; 2012
  • 55 Browne D. Patent ductus arteriosus. Proc R Soc Med 1952; 45 (10) 719-722
  • 56 Hampel P, Rudolph H, Stachow R, Laß-Lentzsch A, Petermann F. Coping among children and adolescents with chronic illness. Anxiety Stress Coping 2005; 18 (02) 145-155
  • 57 Dellenmark-Blom M, Chaplin JE, Quitmann JH. et al. The prevalence and role of coping strategies in the nutritional intake of children born with esophageal atresia: a condition-specific approach. Dis Esophagus 2019; 32 (07) doz004
  • 58 Dellenmark-Blom M, Chaplin JE, Jönsson L, Gatzinsky V, Quitmann JH, Abrahamsson K. Coping strategies used by children and adolescents born with esophageal atresia - a focus group study obtaining the child and parent perspective. Child Care Health Dev 2016; 42 (05) 759-767
  • 59 Dingemann C, Eaton S, Aksnes G. et al. ERNICA consensus conference on the management of patients with esophageal atresia and tracheoesophageal fistula: follow-up and framework. Eur J Pediatr Surg 2020; 30 (06) 475-482
  • 60 Dellenmark-Blom M, Chaplin JE, Gatzinsky V, Jönsson L, Abrahamson K. Health-related quality of life among children, young people and adults with esophageal atresia: a review of the literature and recommendations for future research. Qual Life Res 2015; 24 (10) 2433-2445
  • 61 Coppens CH, van den Engel-Hoek L, Scharbatke H, de Groot SAF, Draaisma JMT. Dysphagia in children with repaired oesophageal atresia. Eur J Pediatr 2016; 175 (09) 1209-1217
  • 62 Mahoney L, Rosen R. Feeding problems and their underlying mechanisms in the esophageal atresia-tracheoesophageal fistula patient. Front Pediatr 2017; 5: 127
  • 63 Connor MJ, Springford LR, Kapetanakis VV, Giuliani S. Esophageal atresia and transitional care--step 1: a systematic review and meta-analysis of the literature to define the prevalence of chronic long-term problems. Am J Surg 2015; 209 (04) 747-759
  • 64 Little DC, Rescorla FJ, Grosfeld JL, West KW, Scherer LR, Engum SA. Long-term analysis of children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2003; 38 (06) 852-856
  • 65 Shah R, Varjavandi V, Krishnan U. Predictive factors for complications in children with esophageal atresia and tracheoesophageal fistula. Dis Esophagus 2015; 28 (03) 216-223
  • 66 Krishnan U, Mousa H, Dall'Oglio L. et al. ESPGHAN-NASPGHAN guidelines for the evaluation and treatment of gastrointestinal and nutritional complications in children with esophageal atresia-tracheoesophageal fistula. J Pediatr Gastroenterol Nutr 2016; 63 (05) 550-570
  • 67 Tovar JA, Fragoso AC. Anti-reflux surgery for patients with esophageal atresia. Dis Esophagus 2013; 26 (04) 401-404
  • 68 Sistonen SJ, Helenius I, Peltonen J, Sarna S, Rintala RJ, Pakarinen MP. Natural history of spinal anomalies and scoliosis associated with esophageal atresia. Pediatrics 2009; 124 (06) e1198-e1204
  • 69 Chetcuti P, Myers NA, Phelan PD, Beasley SW, Dickens DR. Chest wall deformity in patients with repaired esophageal atresia. J Pediatr Surg 1989; 24 (03) 244-247
  • 70 Chetcuti P, Dickens DR, Phelan PD. Spinal deformity in patients born with oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child 1989; 64 (10) 1427-1430
  • 71 Jaureguizar E, Vazquez J, Murcia J, Diez Pardo JA. Morbid musculoskeletal sequelae of thoracotomy for tracheoesophageal fistula. J Pediatr Surg 1985; 20 (05) 511-514
  • 72 Harmon CM, Coran AG. Congenital anomalies of the esophagus. In: Coran AG. ed. Pediatric Surgery. Seventh Edition. Chap. 69. Philadelphia: Mosby; 2012: 893-918
  • 73 Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Oesophageal atresia and associated anomalies. Arch Dis Child 1989; 64 (03) 364-368
  • 74 Soule MC, Beale EE, Suarez L. et al. Understanding motivations to participate in an observational research study: why do patients enroll?. Soc Work Health Care 2016; 55 (03) 231-246
  • 75 Pinheiro PFM, Simões e Silva AC, Pereira RM. Current knowledge on esophageal atresia. World J Gastroenterol 2012; 18 (28) 3662-3672
  • 76 Koivusalo AI, Pakarinen MP, Rintala RJ. Modern outcomes of oesophageal atresia: single centre experience over the last twenty years. J Pediatr Surg 2013; 48 (02) 297-303
  • 77 Friedmacher F, Puri P. Delayed primary anastomosis for management of long-gap esophageal atresia: a meta-analysis of complications and long-term outcome. Pediatr Surg Int 2012; 28 (09) 899-906
  • 78 Tan Tanny SP, Comella A, Hutson JM, Omari TI, Teague WJ, King SK. Quality of life assessment in esophageal atresia patients: a systematic review focusing on long-gap esophageal atresia. J Pediatr Surg 2019; 54 (12) 2473-2478
  • 79 Bundesamt für Statistik Lebendgeburten nach Monat und Fruchtbarkeit seit 1803. Published 2020. Accessed December 30, 2020 at: https://www.bfs.admin.ch/bfs/de/home/statistiken/bevoelkerung/geburten-todesfaelle.assetdetail.14387170.html