Agenesis of the Corpus Callosum
What is agenesis of the corpus callosum?
The corpus callosum is a collection of nerve fibers that connect the two sides (hemispheres) of the brain. In agenesis of the corpus callosum (ACC), these nerve fibers are partially missing or completely absent. This is caused by a disruption in normal fetal development and can occur as an isolated defect or in conjunction with other abnormalities in the brain or any part of the body. Outcomes for children with ACC vary greatly, ranging from normal intelligence to mild learning disabilities to severe mental retardation and seizure disorders.
Agenesis of the corpus callosum occurs in approximately 30-70 of every 10,000 live births in the United States. In most cases, the exact cause of ACC is unknown, though it has been linked to certain genetic factors, prenatal infections, and chromosomal abnormalities.
What will happen during pregnancy?
If ACC is suspected, a series of additional tests will be conducted to confirm the diagnosis and identify any associated problems. These tests can include a fetal brain MRI and amniocentesis for chromosomal analysis.
Based on the results of these tests, your team of doctors will help you prepare for the care of your child after birth, including identifying specialists, such as a pediatric neurologist or neurosurgeon, to help treat and manage anticipated health problems.
Will a fetal treatment be required?
There are currently no fetal treatments for ACC.
What special considerations should be made for delivery?
Type of delivery - Typically, ACC cases do not require cesarean delivery. The delivery plan will be carefully discussed with you and your obstetrician.
Place of delivery - If the baby's ACC is an isolated defect, delivery does not need to occur at a specialized hospital with neonatal intensive and pediatric surgery services. Most infants can be born at the hospital of your choice and then seen at a specialty center after delivery. If other serious complications are present, delivery at a hospital with a neonatal intensive care unit and pediatric neurosurgical services may be recommended.
Time of delivery - There is usually no reason to intentionally induce early delivery for babies with ACC, and mothers can usually carry the child to term.
What will happen at birth?
Most infants will undergo a normal delivery. Mothers and family should be able see and hold their newborn. Immediately following delivery, doctors will carefully evaluate the baby. The baby will be stabilized in an intensive care unit, if needed. In most cases, there are no immediate needs after delivery except for identifying and treating potential associated problems. Your child may undergo a series of tests to examine the brain such as MRI. Your pediatric neurosurgeon will perform a thorough evaluation of your child.
What are the long-term outcomes and considerations?
The severity of neurological problems vary in children with ACC. In mild cases, children can grow up to have normal intelligence, while some may have learning difficulties. Those with severe disease may develop mental retardation and cerebral palsy. These children can be completely dependent on their parents and family for every day function. Most children will undergo many tests that examine the structure, function, and development of the brain and will be followed by a pediatric neurologist and neurosurgeon during childhood. The Center will coordinate an entire team of specialists to help you care for your child.
Contact the Texas Fetal Center to make an appointment with a high-risk pregnancy doctor.
Note: This information regarding your condition is meant to be a helpful, informative introduction. Because every newborn is different, this may not be applicable to all cases, especially if there are additional defects. Your team of experts at the Center will provide you with information specific to your pregnancy.