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Omphalocele


Definition:

An omphalocele is a birth defect in which an infant's intestine or other abdominal organs are outside of the body because of a hole in the belly button (navel) area. The intestines are covered only by a thin layer of tissue and can be easily seen.

Causes:

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

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 abdominal wall.

Infants with an omphalocele often have other birth defects. Defects include genetic problems (chromosomal abnormalities), congenital diaphragmatic hernia, and heart defects.

Symptoms:

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

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

Exams and Tests:

Prenatal ultrasounds often identify infants with an omphalocele before birth. Otherwise, a physical examination of the infant is enough 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 man-made material, which is then stitched in place to form what is referred to as a “silo”. Slowly, as the baby grows over time, the abdominal contents are pushed into the abdomen.

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

Sometimes the omphalocele is so large that it cannot be placed back inside the infant's 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 for a better cosmetic outcome.

Outlook (Prognosis):

Complete recovery is expected after surgery for an omphalocele. However, omphaloceles often occur with other birth defects. How well a child does depends on which other conditions the child has.

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 at repairing omphaloceles. 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 their unborn baby for other genetic problems that are associated with this condition.

Possible Complications:
  • Death of the intestinal tissue
  • Intestinal infection
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:

  • 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:

Chung DH. Pediatric surgery. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 19th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 67.

Ledbetter DJ. Gastroschisis and omphalocele. Surg Clin North Am. 2006;86(2):249-260.


Review Date: 10/29/2013
Reviewed By: Kimberly G Lee, MD, MSc, IBCLC, Associate Professor of Pediatrics, Division of Neonatology, Medical University of South Carolina, Charleston, SC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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