Congenital Hydrocephalus: Treatment

Early treatment-within a baby's first 3 to 4 months-is important to help limit or prevent brain damage. The long-term effects of congenital hydrocephalus depend largely on the cause of the condition, its severity, and the response to treatment.

Treatment generally consists of surgically inserting a flexible tube (shunt) in the brain to drain the cerebrospinal fluid. The shunt will remain in the brain permanently but may need to be repaired or replaced if a problem develops.

Endoscopic third ventriculostomy (ETV) is sometimes performed instead of surgical shunt placement. ETV is a surgical procedure in which a small hole is made in a ventricle in the brain, allowing CSF to flow freely. It is often done when hydrocephalus seems to be a result of a blockage between ventricles. If used as treatment for congenital hydrocephalus, it is more likely to be tried after a shunt malfunction or infection. ETV was once thought to be a permanent solution for redirecting CSF flow, but it has been shown to fail over time. Babies younger than 6 months of age usually are not good candidates for this procedure.

Children with congenital hydrocephalus are at increased risk for developmental disabilities and may require treatment such as speech therapy or physical therapy.

Congenital Hydrocephalus: Diagnosis

Most cases of congenital hydrocephalus are diagnosed during a physical exam soon after birth based on the larger-than-normal size of the baby's head. In rare cases, a diagnosis is made later in childhood. There may be reasons other than congenital hydrocephalus for why a baby has a larger-than-normal head.
With sophisticated imaging technologies, congenital hydrocephalus can be detected in a fetus as early as the third or fourth month of pregnancy. By the fifth or sixth month, abnormal dilation of brain cavities is more clearly detectable. Tests to identify the condition include:

• Ultrasound -- Performed by a radiologist or perinatologist, this test will establish if there is an abnormal collection of fluid but may not show the obstruction. Imaging tests are usually done to see whether congenital hydrocephalus is a possibility.

• Amniocentesis -- This, or a needle aspiration of intrauterine fluid, may be performed to detect the presence of other birth defects associated with hydrocephalus.

Congenital hydrocephalus, however, is frequently diagnosed at birth or shortly after. Sometimes it's not diagnosed until after birth.

A computed tomography (CT) scan, magnetic resonance imaging (MRI), or ultrasound may be done to help confirm the diagnosis or to provide a more detailed picture of the brain and its structures.

Congenital Hydrocephalus: Signs and Symptoms

Signs and Symptoms

The most common cause of congenital hydrocephalus is obstruction of the cerebral aqueduct -- the long, narrow passageway between the third and fourth ventricle or cavity of the brain. This condition may result from a blockage, infection, hemorrhage, tumor or arachnoid cyst. Other medical problems associated with this form of hydrocephalus are:

* Chiari malformations, an abnormality at the base of brain where the spinal column joins the skull
* Craniosynostosis, when the bones in the skull fuse together before the brain has stopped growing
* Dandy-Walker syndrome, when the fourth ventricle is enlarged because of partial or complete closure of its outlets
* Hydranencephaly, a rare condition in which the brain's cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid
* Neural tube defects or spina bifida, when the spinal cord is exposed at birth and is often lacking cerebrospinal fluid
* Schizencephaly, an extremely rare disorder characterized by abnormal slits, or clefts, in the brain's cerebral hemispheres
* Vein of Galen malformations, abnormal connections between arteries and the deep draining veins of the brain that develop before birth

Symptoms in infants may include:

* A very large head or a head that's growing very quickly in relation to the rest of the body
* Vomiting
* Sleepiness
* Irritability
* Downward deviation of the eyes, called "sunsetting"
* Seizures

Source: UCSF