Omphalocele

Definition

An omphalocele is a birth defect in which the infant's intestine or other abdominal organs stick out of the belly button (navel). In babies with an omphalocele, the intestines are covered only by a thin layer of tissue and can be easily seen.

An omphalocele is a type of hernia. Hernia means "rupture."

See also: Umbilical hernia

Causes

An omphalocele develops as a baby grows inside the mother's womb. The muscles in the abdominal wall (umbilical ring) do not close properly. As a result, the intestine remains outside the umbilical cord.

Approximately 25 - 40% of infants with an omphalocele have other birth defects. They may include genetic problems (chromosomal abnormalities), congenital diaphragmatic hernia, and heart defects.

Symptoms

An omphalocele can be clearly seen, because the abdominal contents stick out (protrude) through the belly button area.

There are different sizes of omphaloceles. In small ones, only the intestines stick out. In larger ones, the liver or spleen may stick out of the body as well.

Exams and Tests

Prenatal ultrasounds often identify infants with an omphalocele before birth. Otherwise, physical examination of the infant is sufficient for your health care provider to diagnose this condition. Testing is usually not necessary.

Treatment

Omphaloceles are repaired with surgery, although not always immediately. A sac protects the abdominal contents and allows time for other more serious problems (such as heart defects) to be dealt with first, if necessary.

To fix an omphalocele, the sac is covered with a special synthetic material, which is then stitched in place. Slowly, over time, the abdominal contents are pushed into the abdomen.

When the omphalocele can comfortably fit within the abdominal cavity, the synthetic material is removed and the abdomen is closed.

Outlook (Prognosis)

Complete recovery is expected after surgery for an omphalocele. However, omphaloceles are frequently associated with other birth defects. How well a child does depends on any other conditions.

If the omphalocele is identified before birth, the mother should be closely monitored to make sure the unborn baby remains healthy. Plans should be made for careful delivery and immediate management of the problem after birth. The baby should be delivered in a medical center that is skilled with repairing the omphalocele. The baby's outcome is improved if he or she does not need to be taken to another center for further treatment.

Parents should consider screening the unborn baby for other genetic problems that are associated with this condition.

Possible Complications

• Intestinal infection
• Death of the intestinal tissue related to dryness or trauma to the unprotected intestine

When to Contact a Medical Professional

This problem is diagnosed and repaired in the hospital at birth. After returning home, call your health care provider if the infant develops any of these symptoms:

• Feeding problems
• Vomiting (not normal baby spitting-up)
• Green or yellowish green vomitus
• Distended abdomen
• Decreased bowel movements
• Fever
• Behavioral changes that are worrisome

Townsend CM, Beauchamp RD, Evers BM, Mattox KL. Sabiston Textbook of Surgery, 17th ed. St. Louis, M0: WB Saunders; 2004:2116-2117.

Ledbetter DJ . Gastroschisis and omphalocele. Surg Clin North Am. April 2006; 86(2): 249-60, vii.