In congenital diaphragmatic hernias, loops of bowel or other intra-abdominal contents protrude into the thoracic cavity.
The protruded intra-abdominal contents occupy space in the thoracic cavity and can prevent the lungs from expanding and properly developing. This can lead to the development of pulmonary hypoplasia.
As a result of the intra-abdominal contents compressing on the lungs as well as the associated pulmonary hypoplasia, neonates can develop severe respiratory distress immediately after birth. Since the fetal pulmonary respiratory function is not vital for survival during the intrauterine period, patients are typically asymptomatic up until birth.
Bochdalek hernia is a subtype of congenital diaphragmatic hernia which accounts for more than 90% of all cases. This type of hernia occurs on the posterior left-side of the chest. This type of hernia is not typically seen on the right side of the chest because the liver prevents herniation of intra-abdominal contents. Bochdalek hernias occur because of the failure of closure of the pleuroperitoneal membrane in utero. This eventually leads to a defect in the posterior attachment of the diaphragm.
Morgagni hernia is one subtype of congenital diaphragmatic hernia. It is a rare type of hernia and accounts for approximately 3% of all cases. In contrast to Bochdalek hernias, Morgagni hernias typically occur on the anterior right-side of the chest wall. Morgagni hernias occur as a result of intra-abdominal contents protruding through the foramen of Morgagni. This anatomic area serves as a passing point of the internal thoracic vessels into the abdomen and constitutes a weak point through which abdominal contents can herniate.
Newborn chest X-ray can be used to diagnose congenital diaphragmatic hernias. Chest X-rays will typically show bowel loops on one side of the chest.
Prenatal ultrasound can be used to diagnose congenital diaphragmatic hernias.
Steroids may be used to treat CDH is diagnosed during the prenatal period. Steroids help expedite pulmonary development and reduce the risk of preterm labor and delivery. If the patient has significant respiratory distress i.e. NRDS, then surfactant can be administered to keep alveoli open to allow for adequate gas exchange.
Patients may require intubation immediately after birth, mechanical ventilation, and stabilization. In some cases, patients may require extracorporeal membrane oxygenation (ECMO).
Patients typically require surgery to correct the anatomic defects. In some cases, surgery is delayed for 3-4 days after birth to allow for lung maturation. Surgery involves reducing the bowel contents back into the abdomen and closing the diaphragmatic defect.
In congenital diaphragmatic hernias, the protruded loops of bowel may become incarcerated or perforate. This is a serious complication of CDH and requires immediate treatment.
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