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Long-term medical and psychosocial outcomes in congenital diaphragmatic hernia survivors
  1. Jason K Tan1,
  2. Georgia Banton2,
  3. Corrado Minutillo1,
  4. Graham L Hall2,3,
  5. Andrew Wilson3,4,
  6. Conor Murray5,
  7. Elizabeth A Nathan6,
  8. Maureen Verheggen4,
  9. James Ramsay7,
  10. Naeem Samnakay8,
  11. Jan Dickinson9
  1. 1 Neonatal Intensive Care Unit, Perth Children’s Hospital, Perth, Western Australia, Australia
  2. 2 Children’s Lung Health, Telethon Kids Institute, Perth, Western Australia, Australia
  3. 3 School of Physiotherapy and Exercise Science, Curtin University – Perth City Campus, Perth, Western Australia, Australia
  4. 4 Department of Respiratory Medicine, Perth Children’s Hospital, Perth, Western Australia, Australia
  5. 5 Department of Radiology, Perth Children’s Hospital, Perth, Western Australia, Australia
  6. 6 Division of Obstetrics and Gynaecology, University of Western Australia Faculty of Medicine Dentistry and Health Sciences, Crawley, Australia
  7. 7 Department of Cardiology, Perth Children’s Hospital, Perth, Western Australia, Australia
  8. 8 Division of Surgery, University of Western Australia Faculty of Health and Medical Sciences, Perth, Western Australia, Australia
  9. 9 Division of Obstetrics and Gynaecology, University of Western Australia Faculty of Health and Medical Sciences, Perth, Western Australia, Australia
  1. Correspondence to Dr. Jason K Tan, Neonatal Intensive Care Unit, Perth Children’s Hospital, Perth, Western Australia 6009, Australia; Jason.Tan{at}health.wa.gov.au

Abstract

Objective Survival rates for congenital diaphragmatic hernia (CDH) are increasing. The long-term outcomes of CDH survivors were compared with a healthy control group to assess the morbidity for guidance of antenatal counselling and long-term follow-up programmes.

Participants and design Participants born with CDH in Western Australia 1993–2008 were eligible with matched controls from the general population. Participants had comprehensive lung function tests, echocardiogram, low-dose chest CT scan and completed a Strengths and Difficulties Questionnaire (SDQ) and quality of life (QOL) questionnaire.

Results 34 matched case–control pairs were recruited. Demographic data between groups were similar. Cases were smaller at follow-up (weight Z-score of −0.2vs0.3; p=0.03; height Z-score of −0.3vs0.6; p=0.01). Cases had lower mean Z-scores for forced expiratory volume in 1 s (FEV1) (−1.49 vs −0.01; p=0.004), FEV1/forced vital capacity (−1.92 vs −1.2; p=0.009) and forced expiratory flow at 25-75% (FEF25-75) (−1.18vs0.23; p=0.007). Cases had significantly worse respiratory mechanics using forced oscillation technique. Subpleural triangles architectural distortion, linear opacities and scoliosis on chest CT were significantly higher in cases. Prosthetic patch requirement was associated with worse lung mechanics and peak cough flow. Cases had significantly higher rates of gastro-oesophageal reflux disease (GORD) and GORD medication usage. Developmental delay was significantly higher in cases. More cases had a total difficulties score in the high to very high range (25% vs 0%, p=0.03) on the SDQ and reported lower objective QOL scores (70.2 vs 79.8, p=0.02).

Conclusion Survivors of CDH may have significant adverse long-term medical and psychosocial issues that would be better recognised and managed in a multidisciplinary clinic.

  • congenital diaphragmatic hernia
  • long-term outcomes
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Footnotes

  • Contributors JKGT conceptualised and designed the study, designed the data collection instruments, collected data, coordinated and supervised data collection, drafted the initial manuscript and reviewed and revised the manuscript. GB designed the data collection instruments, collected data, coordinated and supervised data collection and reviewed and revised the manuscript. CM conceptualised and designed the study, designed the data collection instruments, collected data, coordinated and supervised data collection and reviewed and revised the manuscript. GLH and MV designed the study, designed the data collection instruments, reviewed and reported all respiratory function tests and reviewed and revised the manuscript. AW and CM conceptualised and designed the study, designed the data collection instruments, reviewed and reported all chest CT scans and reviewed and revised the manuscript. EAN designed the study, reviewed and conducted all statistical analyses and reviewed and revised the manuscript. JR conceptualised and designed the study, designed the data collection instruments, reviewed and reported all echocardiograms and reviewed and revised the manuscript. NS designed the study, coordinated and supervised data collection and critically reviewed the manuscript for important intellectual content. JD conceptualised and designed the study, coordinated and supervised data collection and reviewed and revised the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

  • Funding All phases of this study were supported by a research grant from Channel 7 Telethon.

  • Competing interests None declared.

  • Ethics approval The Princess Margaret Hospital Human Research Ethics Committee approved this study (reference 2017EP).

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement Data that are included in the manuscript may be available upon contact with the corresponding author. There are some data that are unpublished. These data were reviewed by the statistician who felt that because of the small numbers no statistical analysis should be performed. These data may be available upon contact with the corresponding author.

  • Patient consent for publication Not required.

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