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Treatment courses and outcomes of oesophageal atresia in patients with trisomy 18: a case series of 271 patients from a nationwide database in Japan
  1. Mai Kutsukake1,2,
  2. Takaaki Konishi2,
  3. Michimasa Fujiogi2,3,
  4. Naohiro Takamoto1,2,
  5. Kaori Morita1,2,
  6. Ikuta Yasuhisa2,
  7. Yohei Hashimoto2,
  8. Hiroki Matsui2,
  9. Kiyohide Fushimi4,
  10. Jun Fujishiro1,
  11. Hideo Yasunaga2
  1. 1 Department of Pediatric Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
  2. 2 Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan
  3. 3 Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan
  4. 4 Department of Health Policy and Informatics, Tokyo Medical and Dental University, Tokyo, Japan
  1. Correspondence to Jun Fujishiro, Department of Pediatric Surgery, The University of Tokyo, Bunkyo-ku, 113-8655, Japan; fujishiroj-psu{at}


Objective To describe characteristics, treatments and clinical outcomes of patients with trisomy 18 and oesophageal atresia, using a nationwide database in Japan.

Design Descriptive study using a retrospective cohort.

Setting A nationwide inpatient database including 90% of hospitals with neonatal intensive care units in Japan.

Patients Patients hospitalised within a day after birth for both oesophageal atresia and trisomy 18 between July 2010 and March 2020.

Interventions Radical surgery for oesophageal atresia.

Main outcome measures Characteristics, treatment course and outcomes.

Results Among 271 patients with both oesophageal atresia and trisomy 18, 70 patients underwent radical surgery for oesophageal atresia. Patients who underwent radical surgery were less likely to have severe cardiac anomalies (17% vs 32%; p=0.020), but more likely to undergo cardiac surgery (21% vs 9.5%; p=0.012) than those who did not. The overall in-hospital mortality was lower (54% vs 79%; p<0.001) and the median age at death was higher (210 days vs 39 days; p<0.001) in patients who underwent radical surgery than the others. Postoperative mortality within 30 days after radical surgery was 5.7%. Patients who underwent radical surgery were likely to be discharged to home (50% vs 18%; p<0.001), whereas the age at home discharge (median 314 days vs 216 days; p=0.19) and the requirement for each home treatment did not differ significantly by radical surgery.

Conclusion This study provides information that will aid the clinical decision-making process for patients with oesophageal atresia and trisomy 18. Radical surgery may be a safe and feasible treatment option.

  • Mortality
  • Intensive Care Units, Neonatal

Data availability statement

No data are available.

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Data availability statement

No data are available.

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  • Contributors MK and TK contributed to the study conception and design, data curation and analysis. MF, KM, IY, JF and HY conceptualised and designed the study. YH, HM, KF and HY curated data. KF and HY acquired fundings and supervised data collection. MK wrote the first draft of the manuscript, and all the other authors critically reviewed the manuscript. All authors approved the final version of the manuscript. MK is the guarantor of this study.

  • Funding This work was supported by grants from the Ministry of Health, Labor and Welfare, Japan (23AA2003 (to HY) and 22AA2003 (to KF)) and the Ministry of Education, Culture, Sports, Science and Technology, Japan (20H03907). These grants are used for data management and English editing. The study sponsors had no role in the study design; collection, analysis and interpretation of the data; the writing of the report; or the decision to publish.

  • Competing interests None declared.

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

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.