Congenital Diaphragmatic Hernias Flashcards
Congenital Diaphragmatic Hernias types
1- Posterolateral (Bochdalek) Left-sided 85% Right-sided 13% Bilateral, rare, often fatal
2-Anterior (Morgagni)
3- Hiatus
Congenital Diaphragmatic Hernias incidence
1 in 2000 to 5000 live births Presents within hours of life although some cases of delayed presentation M=F >10% are associated with other congenital anomalies Prenatal diagnosis common
Congenital Diaphragmatic Hernias
Associated
- Left-sided: small bowel, large bowel, stomach, and solid viscera (spleen, left lobe of liver) herniate into thorax
- Right-sided: liver, large bowel herniate into thorax Pulmonary hypoplasia Pulmonary HTN
Congenital Diaphragmatic Hernias clinical manifestations
Early respiratory distress Cyanosis Scaphoid abdomen Prenatal diagnosis
-Decreased air entry ± bowel sounds in the chest Displaced heart sounds
😱😱 It is noteworthy that in some infants, the first 24 to 48 hours after birth are often characterized by a period of relative stability with high levels of PaO 2 and relatively good perfusion. This has been termed the “honeymoon period” and is often followed by progressive cardiorespiratory deterioration.
Congenital Diaphragmatic Hernias investigation
Prenatal US/MRI ABG CXR (bowel loops in hemithorax, shifted heart) Echocardiography Genetic consultation if warranted
Treatment
🌚🌝Stabilisation:
Intubate Orogastric suction Period of respiratory stabilization due to associated pulmonary hypoplasia (may require extracorporeal membrane oxygenation)
🌚🌝 Surgical repair after stable by hernia reduction and closure of diaphragmatic defect open vs. thoracoscopic vs. laparoscopic with or without prosthetic or muscular patch depending on size of defect