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Neural Tube Birth Defect: Encephalocele

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Encephalocele (en – sef – a – lo – seal) is a rare neural tube defect (NTD) that occurs when the bones of the skull do not completely close and leave a hole. Brain tissues, spinal fluid, and membrane (meninges) squeeze through this hole.

Encephalocele presents as either a “groove down the middle of the skull, between the forehead and the nose, or on the back of the skull.” 

According to the Centers for Disease Control, 1 in every 10,000 live births in the United States have encephalocele. The March of Dimes estimates that NTDs affect 3,000 pregnancies each year.

How does encephalocele affect my baby?

According to Children’s Hospital of Wisconsin only half of unborn babies with encephalocele actually survive to birth. This defect occurs more in girls than boys, though researchers don’t know why yet.

Most encephaloceles are diagnosed on routine ultrasound.

Click here to see what encephalocele looks like on the back of the skull. For ultrasound and x-ray pictures go here.

If the defect is covered by skin, alpha-fetoprotein levels will come back normal on amniocentesis, although the amniocentesis will be able to detect if there are any chromosomal abnormalities.

A mom-to-be will likely be referred to a perinatologist, a specialist in the high-risk births, and scheduled for a fetal MRI to better assess the extent of the defect. If the encephalocele is too large, delivery by cesarean section may be recommended.

Children’s Hospital of Wisconsin says that 100 percent of babies born with a frontal encephalocele survive, while only 55 percent survive encephalocele in the back.

Treatment and Prevention of Encephalocele

Treatment usually involves surgery to place the brain tissue and fluid back inside the skull and to close the defect in the bone, usually before 4 months of age. 

However, if there is no skin covering the defect then the surgery is performed immediately after birth. Palliative care may be the only treatment option if the defect is too large to fix.

There is no cure and no clear cause of neural tube defects. But the March of Dimes reports that up to 70 percent of NTDs could be prevented if all women of childbearing years maintained the recommended amount of folic acid before and during early pregnancy.

Since many pregnancies are unplanned and the development and closing of the neural tube happens before a woman even knows she’s pregnant, the CDC recommends that every woman of childbearing years consume at least 400 micrograms (µg or mcg) of folic acid per day.

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