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Long-term follow-up of patients with high-risk congenital diaphragmatic hernia
Shinkichi Kamata1, Noriaki Usui, Masahumi Kamiyama
1Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Japan. kamata@pedsurg.med.osaka-u.ac.jp
Insights
Long-term outcomes for high-risk congenital diaphragmatic hernia patients are influenced by respiratory infections. Monitoring for respiratory tract infections and nutritional status is crucial for these children.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Pulmonology
Background:
- Advances in prenatal diagnosis and neonatal care have improved survival rates for high-risk congenital diaphragmatic hernia (CDH).
- Long-term outcomes and prognostic factors for survivors of CDH remain incompletely understood.
Purpose of the Study:
- To investigate factors influencing the long-term outcomes of high-risk congenital diaphragmatic hernia patients.
- To identify key complications and their impact on growth and pulmonary function in CDH survivors.
Main Methods:
- A cohort of 33 children (mean age 4.1 years) with high-risk CDH underwent clinical evaluation.
- Assessments included growth measurements, echocardiography, and lung ventilation/perfusion scintigraphy.
Main Results:
- No late mortality was observed. Frequent respiratory tract infections and bowel obstruction were the most common complications.
- Frequent respiratory infections correlated with reduced lung ventilation/perfusion, poorer growth, and were potentially exacerbated by lung hypoplasia.
- Patch repair was associated with decreased lung perfusion uptake.
Conclusions:
- Frequent respiratory tract infections negatively impact long-term outcomes in CDH survivors.
- Routine monitoring for respiratory infections and nutritional status is essential in the follow-up care of high-risk CDH patients.
Background/Purpose:
Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established.
Methods:
Thirty-three children with ages 4.1 +/- 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy.
Results:
No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung.
Conclusion:
These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.