Birth Defect: Omphalocele
Omphalocele (om – fa – lo – seal) is a congenital (present-at-birth) birth defect. It happens when the abdominal wall fails to close, allowing abdominal organs to protrude (herniate) into the base of the umbilical cord. A baby is born with his/her abdominal organs in a kind of sac at the base of the umbilical cord.
There are two kinds of omphalocele: giant and small. A giant omphalocele, in which the majority of the liver herniates into the umbilical cord, occurs in 1 in 10,000 births. A small omphalocele happens when only some of the intestines develop outside the belly, and occurs in 1 in every 5,000 births.
Because the baby’s organs have developed inside the umbilical cord instead of inside the abdominal cavity, the baby’s abdomen and lungs may be smaller than normal and underdeveloped.
The CDC estimates that 775 babies are born every year with omphalocele in the United States, or 1 out of every 5,386 babies. More than half of babies born with omphalocele have other birth defects such as atrial septal defect or neural tube defects and chromosomal defects. (CHB) “Thirty percent have a genetic abnormality, most commonly Trisomy 13, Trisomy 18, Trisomy 21, Turner syndrome or triploidy.” (CHB)
Causes and Risk Factors of Omphalocele
“Between the sixth and tenth weeks of pregnancy, the intestines actually project into the umbilical cord as they are growing. By the eleventh week of development, the intestines should return to the abdomen. An omphalocele occurs when the abdominal organs do not return to the abdominal cavity as they should.” (CHB)
No one knows precisely why these defects happen, however, there are several risk factors that increase a woman’s chance of having a baby born with omphalocele:
- Consumption of alcohol
- Heavy smoking habits (more than a pack a day)
- Taking certain serotonin-reuptake inhibitors (SSRIs) during pregnancy
Treatment of Omphalocele
An omphalocele can be seen on a routine ultrasound. Amniocentesis is often recommended when a defect is found to evaluate for chromosomal abnormalities. If the herniation is small, a vaginal delivery may be possible. In the case of giant omphalocele, however, a cesarean section is recommended to prevent trauma to any of the organs.
In the case of a small omphalocele, the exposed organs are returned to the abdomen and the herniation is surgically closed.
With a giant omphalocele, “the exposed organs are covered with a protective wrap, and the organs gradually return to the abdominal cavity with the aid of gravity. In some cases, a stages surgical repair might be required.” (CHP) The staging is necessary to allow the abdomen to stretch to accommodate the organs, as would have normally happened in utero. The entire process can take approximately 6 to 12 months for the abdomen to grow large enough for full closure.
Babies with omphalocele and undergoing treatment are fed intravenously. Once bowel movements start happening then feeding via naso-gastric tube is added, for which expressed breast milk is ideal, so long as the baby tolerates it.
Please read about this one family’s journey through the treatment/surgery process.
Darlene Oakley is a freelance writer for EmpowHER.com.
Omphalocele Surgery and Repair. Children’s Hospital of Philadelphia. Web. Mar 26, 2012.
Omphalocele. Children’s Hospital Boston. Web. Mar 26, 2012.
Birth Defects: Facts about Omphalocele. Centers for Disease Control and Prevention. Web. Mar 26, 2012.