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The Japanese experience with prenatally diagnosed congenital diaphragmatic hernia based on a multi-institutional review

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Abstract

Purpose

To review the recent Japanese experience with prenatally diagnosed congenital diaphragmatic hernia (CDH) based on a multi-institutional survey.

Methods

A multicenter, retrospective cohort study was conducted on 117 patients born between 2002 and 2007 with isolated prenatally diagnosed CDH. All patients were managed by maternal transport, planned delivery, immediate resuscitation and gentle ventilation. The primary outcome measurements were the 90-day survival and intact discharge. The examined prenatal factors included gestational age (GA) at diagnosis, lung-to-head ratio (LHR), lung-to-thorax transverse area ratio (L/T) and liver position. Physical growth and motor/speech development were evaluated at 1.5 and 3 years of age. Data were expressed as the median (range).

Results

The mean GA at diagnosis was 29 (17–40) weeks. The LHR and L/T were 1.56 (0.37–4.23) and 0.11 (0.04–0.25), respectively. There were 48 patients with liver up. The mean GA at birth was 38 (28–42) weeks. The 90-day survival rate and intact discharge rate were 79 and 63%, respectively. Twelve patients had major morbidity at discharge, and 71% of these patients had physical growth or developmental retardation at 3 years of age.

Conclusion

This multicenter study demonstrated that the 90-day survival rate of isolated prenatally diagnosed CDH was 79%, and that subsequent morbidity remained high. A new treatment strategy is needed to reduce the mortality and morbidity of severe CDH.

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Acknowledgments

This work was supported by a grant from The Ministry of Health, Labor and Welfare of Japan (Health and Labor Sciences Research Grants of Clinical Research for New Medicine).

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Authors and Affiliations

  1. Department of Pediatric Surgery, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663-8501, Japan

    Hiroomi Okuyama

  2. Division of General Surgery, National Center for Child Health and Development, Tokyo, Japan

    Yoshihiro Kitano, Nobuyuki Morikawa & Hajime Takayasu

  3. Division of Clinical Research, National Center for Child Health and Development, Tokyo, Japan

    Mari Saito

  4. Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan

    Noriaki Usui

  5. Department of Pediatric Surgery, Kyusyu University Hospital, Fukuoka, Japan

    Kouji Masumoto

  6. Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan

    Tomoo Nakamura

  7. Department of Obstetrics and Gynecology, Kanagawa Children’s Medical Center, Yokohama, Japan

    Hiroshi Ishikawa

  8. Department of Neonatology, Kanagawa Children’s Medical Center, Yokohama, Japan

    Motoyoshi Kawataki

  9. Division of Maternal-Fetal and Neonatal Medicine, National Center for Child Health and Development, Tokyo, Japan

    Satoshi Hayashi & Haruhiko Sago

  10. Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

    Noboru Inamura

  11. Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

    Keisuke Nose

Authors
  1. Hiroomi Okuyama
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  2. Yoshihiro Kitano
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  3. Mari Saito
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  4. Noriaki Usui
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  5. Nobuyuki Morikawa
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  6. Kouji Masumoto
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  7. Hajime Takayasu
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  8. Tomoo Nakamura
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  9. Hiroshi Ishikawa
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  10. Motoyoshi Kawataki
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  11. Satoshi Hayashi
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  12. Noboru Inamura
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  13. Keisuke Nose
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  14. Haruhiko Sago
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Corresponding author

Correspondence to Hiroomi Okuyama.

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Okuyama, H., Kitano, Y., Saito, M. et al. The Japanese experience with prenatally diagnosed congenital diaphragmatic hernia based on a multi-institutional review. Pediatr Surg Int 27, 373–378 (2011). https://doi.org/10.1007/s00383-010-2805-0

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  • DOI: https://doi.org/10.1007/s00383-010-2805-0

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