Research: Long-Term Outcomes of Congenital Diaphragmatic Hernia: Report of a Multicenter Study in Japan

Children (Basel)

. 2022 Jun 8;9(6):856.

 doi: 10.3390/children9060856.

Long-Term Outcomes of Congenital Diaphragmatic Hernia: Report of a Multicenter Study in Japan

Masaya Yamoto 1Kouji Nagata 2Keita Terui 3Masahiro Hayakawa 4Hiroomi Okuyama 5Shoichiro Amari 6Akiko Yokoi 7Kouji Masumoto 8Tadaharu Okazaki 9Noboru Inamura 10Katsuaki Toyoshima 11Yuhki Koike 12Yuta Yazaki 13Taizo Furukawa 14Noriaki Usui 15

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Free PMC article


Background: Treatment modalities for neonates with congenital diaphragmatic hernia (CDH) have greatly improved in recent years, with a concomitant increase in survival. However, long-term outcomes restrict the identification of optimal care pathways for CDH survivors in adolescence and adulthood. Therefore, we evaluated the long-term outcomes within the Japanese CDH Study Group (JCDHSG).

Methods: Participants were born with CDH between 2006 and 2018 according to the JCDHSG. Participants were enrolled in the database at 1.5, 3, 6, and 12 years old. Follow-up items included long-term complications, operations for long-term complication, and home medical care.

Results: A total of 747 patients were included in this study, with 626 survivors (83.8%) and 121 non-survivors (16.2%). At 1.5, 3, 6, and 12 years old, 45.4%, 36.5%, 34.8%, and 43.6% developed complications, and 20.1%, 14.7%, 11.5%, and 5.1% of participants required home care, respectively. Recurrence, pneumonia, pneumothorax, gastroesophageal reflux disease, and intestinal obstruction decreased with age, and thoracic deformity increased with age.

Conclusions: As CDH survival rates improve, there is a need for continued research and fine-tuning of long-term care to optimize appropriate surveillance and long-term follow-up.

Keywords: congenital diaphragmatic hernia; gastroesophageal reflux disease; intestinal obstruction; long-term outcomes; pneumonia; pneumothorax; recurrence.

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