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  Omphalocele
  The Facts

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Essentially, omphalocele is a type of hernia.  Omphalocele is when the infant’s intestines and other organs stick out of the belly button.  An Omphalocele develops while the baby is growing in the womb.  The muscles in the abdomen do not close properly and as a result the intestines and other organs may stick outside the belly button (umbilical ring).  A small omphalocele is generally when only a portion of the intestines protrudes.  A giant omphalocele is generally happens when more organs protrude – usually those organs are the intestines, liver and spleen.  The cause of Omphalocele is not readily known, and is not due to anything the mother did or did not due.  It is believed there may be some underling genetic issue that may be the cause.   

Frequency

1 in 5000 – small omphalocele

1 in 10,000 – large omphalocele

An omphalocele happens more frequently in mothers over 35 years of age.

Affects more boys than girls.

Diagnosis and Prognostic Criteria/Diagnosis and Staging

Omphalocele is diagnosed by an ultrasound during the pregnancy.  Specifically the doctors will look for the type of defect, the severity of the defect, and associated defects.

Sometimes omphalocele may result in abnormal results on a blood or serum screening test during pregnancy.

About 25 to 40% of infants with omphalocele have other birth defects such as genetic defects, congenital diaphragmatic hernia and/or heart defects.

Management Options and Outcomes

After diagnosis the mother will have an ultrasound every two to four weeks to ensure the fetus remains surrounded with amniotic fluid and experiences adequate growth and development. Biophysical profiles (BPP) may also be scheduled weekly. The BPP is an ultrasound that monitors amniotic fluid volume, the baby's breathing movements, and movements of the extremities along with the non-stress test.

A c-section may be preferred depending on the size of the omphalocele.

When born, the abdominal wall is covered with a membranous sac.  But sometimes the sac may be punctured. So the organs will be at a risk for infection.  Either way until surgery – the bowels will be covered and baby’s lower body placed in a bag to hold in moisture and heat.

Candidacy for Fetal Treatment

Currently fetal surgery to repair the omphalocele is not available as there is no need to repair the defect in the womb.  The surgery to fix omphalocele will be done after birth.  If it is a large omphalocele a silo will be placed around the protruding organs.

Details of Procedures

The purpose of the surgery is to place the organs back into the baby’s abdominal cavity.  The goal is to do that during the initial surgery – which would be called a primary closure.  However, if the entire bowel is does not fit at this time, the organ will be placed in a silo/sac and pushed in over a period of time.  After all is pushed into the abdominal wall, the surgeons will close the abdominal wall.  This could take 6 to 12 months.

Postoperative Care

After the surgery – the baby may not be able to breath of its own due to the pressure on the diaphragm.  The doctors will also place a tube through the nose or mouth into the stomach to keep the stomach empty.  After surgery the baby will receive nutrition through a central line – TPN.  When the baby shows signs of a functioning healed bowel, the doctors will slowly introduce normal feeds. 

Long term – if there was damage to the intestines or other organs the child may have long term problems with elimination, digestion, and infection.

Additional Resources

·         UCSF Fetal Treatment Center - Informative Videos

·         GEEPS – Gastroschisis Exomphalos Extrophies Support




 
 
 
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