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Published on: March 10, 2020
Congenital diaphragmatic hernia
Elçin Ersöz Köse1, İrfan Yalçınkaya1
1Department of Thoracic Surgery, Health Sciences University Istanbul Hamidiye Medical Faculty, Süreyyapaşa Chest Diseases and Thoracic Surgery Hospital, Istanbul, Türkiye.
Insights
Congenital diaphragmatic hernia (CDH) is a rare birth defect. Advances in prenatal diagnosis and postnatal care improve outcomes for infants with CDH, with surgical repair often delayed until stabilization.
Area of Science:
- Pediatric Surgery
- Neonatology
- Developmental Biology
Background:
- Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterized by diaphragm defects, leading to abdominal organs entering the chest.
- This condition causes pulmonary hypoplasia and hypertension, significantly impacting infant survival and development.
- Recent progress in prenatal diagnosis and understanding prognostic factors guides fetal and postnatal management strategies.
Purpose of the Study:
- To provide a comprehensive review of congenital diaphragmatic hernia (CDH).
- To focus on the pathophysiology, diagnostic advancements, fetal therapies, and postnatal management of CDH.
- To highlight improvements in infant outcomes through refined treatment protocols.
Main Methods:
- Review of current literature on congenital diaphragmatic hernia (CDH).
- Analysis of advances in prenatal diagnosis and prognostic factor identification.
- Evaluation of established and emerging fetal and postnatal therapeutic approaches.
Main Results:
- Prenatal diagnosis and prognostic factor identification have refined fetal therapy approaches for CDH.
- Protocolized postnatal care, including lung-protective ventilation and pulmonary hypertension management, has improved infant outcomes.
- Surgical repair is often deferred until the infant's pulmonary status is stable, optimizing surgical timing.
Conclusions:
- Congenital diaphragmatic hernia (CDH) management has evolved significantly with improved prenatal and postnatal strategies.
- Early and aggressive management of pulmonary hypertension and lung-protective ventilation are critical for better outcomes.
- A multidisciplinary approach integrating fetal interventions and optimized postnatal care is essential for improving survival rates in CDH patients.
Abstract:
Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, characterized by the herniation of abdominal contents into the chest, resulting in varying degrees of pulmonary hypoplasia and pulmonary hypertension. Significant advances in the prenatal diagnosis and identification of prognostic factors have resulted in the continued refinement of the approach to fetal therapies for CDH. In the postnatal period, protocolized approaches to lung-protective ventilation, nutrition, prevention of infection, and early aggressive management of pulmonary hypertension have led to improved outcomes in infants with CDH. Surgical repair of CDH is not urgent in most circumstances and can be delayed until the pulmonary status of the patient has stabilized. This article provides a comprehensive review of CDH, focusing on the complex pathophysiology, advances in prenatal diagnosis, fetal interventions, and optimal postnatal management of CDH.
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