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  Gastroschisis
  The Facts

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First and foremost, gastroschisis is a hernia.  Gastroschisis is a birth defect that happens when the intestines stick of the body through one side of the umbilical cord.  Due to the condition and lack of membrane around the intestines, the intestines are exposed to the amniotic fluid and unprotected during the pregnancy.

Frequency

1 in 5000 and usually happens in babies carried by young mothers (usually under the age of 20).  It is usually an isolated defect, not associated with chromosome abnormalities or other structural malformations.

Diagnosis and Prognostic Criteria/Diagnosis and Staging

Usually discovered during an ultrasound or when the mother's alpha feto protein (AFP) blood levels become elevated during the second trimester 

Management Options and Outcomes

There is an increased risk for third trimester complications, such as bowel dilatation, decreased fetal growth and amniotic fluid volume, preterm delivery, as well as the slight risk of fetal death.  Approximately 20 to 40 percent will have some type of gastrointestinal abnormality such as: malrotation (bowel is not in correct position), atresia (passage is blocked), volvulus (twisting of the bowel, blood supply can be cut off), or infarction (blood supply has been cut off and that area will be damaged).

This is controversy regarding whether c-section or vaginal delivery is preferred – but it is agreed that timing of labor and birth of child is vital due to an increased risk of still birth.  Please be aware that these babies have a 75% chance of begin classified as growth restricted.

Candidacy for Fetal Treatment

No – not a surgery to correct the problem in utero.  However, there are some potential management techniques.

For example, if the bowel damage is caused by exposure to amniotic fluid itself or inflammatory components in amniotic fluid, the amniotic fluid itself can be cleansed by serial amino exchange. The idea of serial amniocentesis or replacing amniotic fluid with clean fluid is currently being tested.

Another surgery may enlarge the hole in the fetus’s abdominal wall if the bowel is damaged by being squeezed at its base by the smallness of the hole in the fetus’s abdominal wall. This is done by a fetoscopic procedure.

Details of Procedures

Two different types of procedures may be done after birth depending on the severity:

(1) Primary gastroschisis repair for the less severe cases: The baby undergoes surgical repair immediately after birth. The bowel is returned to the abdominal cavity, and the abdominal wall is closed during one procedure. This is the preferred method of repair because it is associated with a reduced risk of infection and fluid loss.

(2) Staged gastroschisis repair for the more severe cases: Prolonged exposure to irritating amniotic fluid or trauma during birth may inflame or stretch the bowel. If so, the bowel is covered by a sheet of protective material (silo) and gradually returns to the abdominal cavity by tightening the silo.  This process may take three to ten days. Then, the abdomen is closed surgically.  Normally the infant will need breathing assistance during this period of time.

Postoperative Care

After the gastroschisis repair, it may still take time for the intestines to heal and recover.  Your baby will probably be fed intravenously by total parenteral nutrition (TPN) until bowel function returns.  Then your baby will be fed by a naso-gastric tube until normal feeding can be tolerated.  More likely than not, your baby will remain in NICU for approximately 6 to 12 weeks.  Your baby may experience set backs including the need for bowel surgery or rest.

Additional Resources

·         UCSF Fetal Treatment Center - Informative Videos

·         Avery’s Angels Gastroschisis Foundation

·         GEEPS – Gastroschisis Exomphalos Extrophies Support

 




 
 
 
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