Congenital Hydrocephalus

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

Diagnosis

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.

• 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. At UCSF Children's Hospital, our team of doctors and other staff members will thoroughly evaluate your child's condition. Our staff will ask you about the medical history of your child as well as of your immediate family. If your pediatrician has sent us your child's medical records, we will review this information. Be sure to tell your doctors about any medications your child is currently using, including over-the-counter medications, vitamins, nutritional supplements or herbal remedies.

Our medical team will examine your child to attempt to discover the cause of the medical problem and to identify the best treatment. This evaluation includes imaging techniques such as ultrasonography, computer tomography (CT), magnetic resonance imaging (MRI) or pressure-monitoring techniques.

Treatment

Congenital hydrocephalus generally isn't treated until after birth, even if diagnosed in the womb. The condition most often is treated by either removing the cause of the condition, such as a tumor or other blockage, or inserting a shunt. A shunt is a small flexible plastic tube that diverts excess cerebrospinal fluid (CSF) from the brain to another part of the body where the fluid can be reabsorbed.

Some patients can be treated with an alternative procedure called endoscopic third ventriculostomy (ETV). In this procedure, a small hole is made in the floor of the third ventricle of the brain, allowing the cerebrospinal fluid to bypass the obstruction and flow toward the area where it can be reabsorbed by the body.

Your medical team will explain any possible risks or complications from the treatments your child  receives at UCSF Children's Hospital.

Your child's team, along with your family pediatrician, will design a treatment plan tailored to your child's condition, state of health and individual needs. Your child may need more than one kind of treatment or treatment requiring several visits to UCSF Children's Hospital. You and your child may be referred to additional doctors or other medical professionals.

Hydrocephalus can pose risks to both mental and physical development. Many children diagnosed with the disorder benefit from rehabilitation and educational programs, and go on to lead normal lives.

Don't be afraid to ask your doctors, nurses or therapists about the treatments your child will receive. If treatment includes medication, be sure your child takes it exactly as your doctor has ordered. And remember to bring your child to all follow-up appointments requested by our doctors or other medical staff.