About CDH

Congenital Diaphragmatic Hernia (CDH)

Overview

 Congenital Diaphragmatic Hernia (CDH) is a condition in which a hole in the diaphragm allows abdominal organs to move into the chest and restrict lung development. Congenital diaphragmatic hernia (CDH) is a birth defect that occurs in about one in every 2,500 live births. It is characterized by the development, very early in gestation, of a hole in the diaphragm, the breathing muscle that separates the chest from the abdomen. The hole occurs most commonly on the left side. As a result, the intestines and other organs in the abdomen can move into the chest and compress the developing lungs. This prevents the lungs from growing and developing normally, which can cause reduced blood flow to the lungs and pulmonary hypertension (high blood pressure in the pulmonary circulation). It can be life-threatening unless treated.

Diagnosis

As with other birth defects, most congenital diaphragramatic hernias (CDHs) are typically discovered by routine ultrasound at 16 to 18 weeks gestation.

The severity of congenital diaphragmatic hernia (CDH) is largely determined by the position of the liver. Outcomes are generally better in cases where the liver remains down in the abdomen. The overall survival rate is 70 percent.

Once stabilized, the baby is transported to the Newborn/Infant Intensive Care Unit (N/IICU), where the team continues to evaluate and carefully monitor the newborn’s condition. Babies with severely compromised or fragile lungs may require ECMO (Extracorporeal Membrane Oxygenation), a temporary bypass technique used to oxygenate the blood and allow the lungs to rest, for several days or even weeks.

Surgical repair of CDH depends on the baby’s progress in the days following birth, and can occur as early as three days of life. Surgery is performed in the N/IICU, avoiding the risks of transport to the operating room. An incision is made just below the baby’s rib cage, the organs in the chest are guided back down into the abdomen and the hole in the diaphragm is sewn closed. The space created in the chest allows the lungs to continue to grow; children can experience compensatory lung growth until age 8 or 9.

For babies with larger defects or completely lacking a diaphragm on one side, the hole is closed with a soft tissue patch. As the child grows, the condition of the patch will be regularly monitored by doctors to ensure that it remains intact.  Length of stay in the N/IICU can vary widely. Some infants need mechanical ventilation only briefly, have the surgical repair and can go home in a relatively short period of time. Others may need ventilation or ECMO for prolonged periods and require longer hospital stays.

Reference

Congenital Diaphragmatic Hernia