Congenital Diaphragmatic Hernia (CDH)

Congenital diaphragmatic hernia (CDH) is defined as defect/ a hole in the diaphragm muscle (the muscle that separates the chest from the abdomen) . Diaphragm fails to close during prenatal development, and the contents from the abdomen (stomach, intestines and/or liver) migrate into the chest through this hole/ defect.

Its incidence is 1 in every 2,500 live births.

The herniation of the abdominal organs in the chest prevents the lungs development, resulting in pulmonary hypoplasia (or underdeveloped lungs). This can cause reduced blood flow to the lungs and pulmonary hypertension (high blood pressure in the pulmonary circulation), as well as asthma, gastrointestinal reflux, feeding disorders and developmental delays.

CDH can occur mostly on the left side (80%), right side(20%) or, very rarely, on both sides. It can be life-threatening.

Diagnosis:

Prenatal: Prenatal ultrasound is accurate in 40-90% cases. If diagnosed before 24 weeks of gestation has poor prognosis. Polyhydramnios(excessive amniotic fluid) is reported in 80% of cases and associated with poor survival.

Prenal magnetic resonance imaging (MRI): It can readily determine position of liver in relation to the diaphragm and herniation of liver into either hemithorax. It can be used to assess lung volume and perhaps correlation with outcome.

After birth:

Plain chest radiograph: demonstrating loops of intestine in the chest. The position of gastric bubble can be confirmed by placing orogastric tube

Clinically: Respiratory distress at birth is indicative of degree of pulmonary hypoplasia and reactive pulmonary hypertension.

Abdomen is typically scaphoid
Chest is asymmetrically

Treatment:

CDH is a physiologic emergency and not a surgical emergency.

Hence it is necessary to stabilize cardiorespiratory system first and than proceed to surgery.