What is Omphalocele?

Here are some short articles we found helpful in a basic way for explaining what is going on with "Bayta"


Definition of Omphalocele
Omphalocele: A birth defect in which part of the intestine, covered only by a thin transparent membrane, protrudes outside the abdomen at the umbilicus. The underlying error is a failure during embryonic development for a section of the intestines (the midgut) to return from outside the abdomen and reenter the abdomen, as it should. The opening in the abdominal wall cannot close because, to do so, would pinch off part of the intestines. An omphalolocele must be repaired by surgery.

With an omphalocele, the part of the intestine that sticks out is covered by two thin transparent membranes called the amnion (the amniotic membrane) and peritoneum (the abdominal membrane). The liver and spleen may also be within the omphalocele.

An omphalocele is caused by an error in the normal embryonic development of the intestinal tract. During embryogenesis (the time during which the embryo forms), there are initially three distinct portions of the intestinal tract (the foregut, midgut and hindgut) that extend the length of the embryo. Much of the midgut is then herniated (protruded) outside the abdomen at the umbilicus (belly button). The midgut later reenters the abdomen (belly) and the opening in the abdominal wall closes. The error responsible for an omphalocele is a failure for the midgut to return and reenter the abdomen. The opening in the abdominal wall cannot close because to do so would pinch off part of the intestines.

About 1 in 5,000 babies is born with an omphalocele. The omphalocele can be an isolated birth defect (all by itself). Or it may occur in children who have additional malformations as for example a congenital heart defect. Omphalocele is a characteristic malformation in certain chromosome abnormalities as the trisomy 13 (Patau) syndrome and the trisomy 18 (Edwards) syndrome. It also is seen in some genetic disorders such the Beckwith-Wiedemann syndrome.

"Omphalo-" indicates a relationship to the umbilicus (the navel) and the suffix "-cele" refers to a hernia or rupture, so omphalocele literally = a hernia or rupture at the umbilicus.

Illustrations

Infant omphalocele

Omphalocele repair - series


Definition Return to top

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 Return to top

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 Return to top

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 Return to top

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 Return to top

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.

Sometimes the omphalocele is so large that it cannot be place back inside the infants abdomen. The skin around the omphalocele grows and eventually covers the omphalocele. The abdominal muscles and skin can be repaired when the child is older in order to achieve a better cosmetic outcome.

Outlook (Prognosis) Return to top

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 Return to top

Death of the intestinal tissue
Intestinal infection
When to Contact a Medical Professional Return to top

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:

Decreased bowel movements
Feeding problems
Fever
Green or yellowish green vomit
Swollen belly area
Vomiting (different than normal baby spit-up)
Worrisome behavioral changes
References Return to top

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.


Update Date: 10/8/2007