MedlinePlus Medical Encyclopedia: Diaphragmatic hernia

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Diaphragmatic hernia

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider Prevention

Illustrations

Digestive system

Diaphragm

Diaphragm and lungs

Diaphragmatic hernia repair - series

Infant diaphragmatic hernia

Alternative names Return to top

Hernia - diaphragmatic; Congenital hernia of the diaphragm

Definition Return to top

A diaphragmatic hernia is an abnormal opening in the diaphragm that allows part of the abdominal organs to migrate into the chest cavity, occurring before birth.

Causes, incidence, and risk factors Return to top

A diaphragmatic hernia is caused by the improper fusion of structures during fetal development. The abdominal organs such as the stomach, small intestine, spleen, part of the liver, and the kidney appear in the chest cavity. The lung tissue on the affected side is thus not allowed to completely develop. Respiratory distress usually develops shortly after the baby is born because of ineffective movement of the diaphragm and crowding of the lung tissue which causes collapse. The reason why this occurs is not known.

Congenital diaphragmatic hernia is seen in 1/2200 to 1/5000 live births with the vast majority (80 to 90%) occurring on the left side. There is a 2% recurrence rate in first degree relatives of a patient with the disease.

Symptoms Return to top

severe breathing difficulty
bluish coloration of the skin due to lack of oxygen
fast breathing (tachypnea)
asymmetry of the chest wall
fast heart rate (tachycardia)

Signs and tests Return to top

The pregnant mother may have shown signs of polyhydramnios (excessive amounts of amniotic fluid). Fetal ultrasound may show abdominal contents in the chest cavity.

Examination of the infant shows:

chest movements asymmetric with breathing
breath sounds absent on the affected side
bowel sounds heard in the chest
concave abdomen that feels less full on examination by touch (palpation)

A chest X-ray may show abdominal organs in chest cavity.

Treatment Return to top

A diaphragmatic hernia is a surgical emergency. The abdominal organs must be replaced into the abdominal cavity, and the opening in the diaphragm repaired. Support of the respiratory system is necessary immediately after birth until the infant recovers from surgery. Some infants are placed on ECMO (extracorporeal membrane oxygenation) which is a heart/lung bypass machine which gives the lungs a chance to recover and expand after surgery.

If a diaphragmatic hernia is diagnosed early in gestation (~24 to 28 weeks), fetal surgery may be an option to be considered.

Expectations (prognosis) Return to top

Congenital diaphragmatic hernia is a very serious disorder. The lung tissue may be underdeveloped on the affected side, and the outcome depends upon the development of the lung tissue. With advances in neonatal and surgical care, survival is now greater than 80%. A poor prognosis, however, is associated with polyhydramnios, presence of the fetal stomach in the chest, and an early presentation (i.e., distress in the first few hours of life). Infants who survive may have long-term complications including persistent pulmonary hypertension (PPHN), recurrent lung infections, and gastrointestinal problems.

Complications Return to top

pulmonary (lung) infections
other congenital anomalies may be present

Calling your health care provider Return to top

Go to the emergency room or call the local emergency number (such as 911). A diaphragmatic hernia is a surgical emergency.

Prevention Return to top

There in no known prevention for this condition.

Update Date: 10/30/2003

Updated by: Philip L. Graham III, M.D., M.S., F.A.A.P., Department of Pediatrics, Children's Hospital of New York, Columbia University, New York, NY. Review provided by VeriMed Healthcare Network.

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