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Congenital hydrocephalus is a buildup of excess
cerebrospinal fluid (CSF) in the brain at birth. The
extra fluid can increase pressure in the baby's brain, causing brain damage and
mental and physical problems. This condition is rare.
Finding the condition early and treating it
quickly can help limit any long-term problems. But long-term effects mostly
depend on what caused the fluid buildup, how bad it gets, and how the baby
responds to treatment.
hydrocephalus doesn't occur until later in life, it is called acquired
hydrocephalus. This topic focuses on hydrocephalus that is present at birth
condition is caused by an imbalance between how much fluid the brain makes and
how well the body is able to process it.
Normally, fluid flows
through and out of chambers of the brain called ventricles, and then around the
brain and spinal cord. The fluid is then absorbed by the thin tissue around the
brain and spinal cord. But with hydrocephalus, the fluid can't move where it
needs to or is not absorbed as it should be. And in rare cases the brain makes
too much fluid.
Congenital hydrocephalus may happen because
The clearest symptom of
hydrocephalus is a head that is larger than normal. You and your doctor may
notice it when the baby is born or within the first several months of life. It's normal for a baby's head to grow a lot during the first year. But with congenital hydrocephalus, the head may grow faster than the normal rate for a baby's height
condition may cause the
soft spot (fontanelle) on your baby’s head to feel
firm or bulge out. Also, the areas between the skull bones (sutures) may be larger than normal.
If pressure builds in the brain, your baby may:
A fetal ultrasound can sometimes show the problem before birth. But most cases are found during a
physical exam soon after birth. Your
doctor may suspect that your baby has congenital hydrocephalus if your baby’s
head is larger than normal.
Your baby may need imaging tests,
such as a
CT scan, an
MRI, or an
ultrasound, that can give a picture of the brain with
more detail. Genetic tests may be done in some cases.
Early treatment—before age 4 months—is important to help limit or prevent brain
damage. Treatment focuses on reducing the amount of fluid in the brain to relieve
In most cases, the doctor places a flexible tube, called a shunt, in the brain to drain the fluid. The shunt carries fluid to another part of the body (usually the belly or the heart), which then absorbs the fluid. The shunt may stay in the brain
for life, though it may be have to be fixed or replaced later if it becomes blocked or infected.
A surgery called endoscopic third ventriculostomy (ETV) may be used instead of a shunt in some cases. In ETV, a small hole is made in the deep part of the brain so that the fluid in the brain can flow freely.
Sometimes emergency treatment is needed to reduce the fluid. This may include medicines, a lumbar puncture (sometimes called a spinal tap), or a procedure to drain fluid from the brain until a shunt can be put in.
If your child has any developmental problems or delays caused by damage to the brain, your doctor can help you find the care you need. Treatment will focus on the specific problems your child has. For example, speech therapy can help with speech delays. Physical therapy can help with motor skill problems.
No matter what kind of treatment your child has had, you and your child's doctors will need to watch your child closely to make sure that the fluid in the brain continues to drain properly. Pressure can build up in the brain again. Shunts can become blocked or infected. These problems need to be treated right away to prevent permanent damage.
Symptoms such as irritability, poor appetite, sleeping too much, and vomiting often may be signs that fluid has built up again in your child's brain. After early childhood, there may be other symptoms to watch for, such as headaches, vision problems, confusion, slurred speech, or problems walking. Shunt infections may also cause a fever and redness along the shunt tract or valve.
As your child grows, you'll need to watch for problems with brain development. These could include things like delayed learning, problems with motor skills, and speech problems. Talk to your doctor about any new problems or changes you notice.
It can be hard to wait and see if symptoms will return. And if your child has special needs, it can be a challenge to take care of him or her. Try to take good care of yourself. And ask your
doctor about support groups and organizations that can help.
Learning about congenital hydrocephalus:
Living with congenital hydrocephalus:
The National Institute of Neurological Disorders and
Stroke (NINDS), a part of the National Institutes of Health, is the leading
U.S. federal government agency supporting research on brain and nervous system
disorders. It provides the public with educational materials and information
about these disorders.
NeurosurgeryToday.org is the public Web site of the American Association of Neurological Surgeons. You'll find information about the prevention, diagnosis, treatment, and rehabilitation of conditions that affect the entire nervous system including the spinal column, spinal cord, brain, and peripheral nerves.
The goal of the Hydrocephalus Association is to ensure that
families and individuals dealing with the complex issues of hydrocephalus
receive personal support, comprehensive educational materials, and ongoing
quality health care. This nonprofit group promotes the importance of medical
research. And it has many services and resources for both members and
The National Hydrocephalus Foundation (NHF) is a not-for-profit
public service organization that aims to increase public awareness and
knowledge of hydrocephalus. The organization also promotes and supports
research on the cause, treatment, and prevention of hydrocephalus. In addition
to providing the public with informational pamphlets, the NHF maintains a
reference library and videos on hydrocephalus, offers support groups, and
publishes the quarterly newsletter LIFE LINE.
Other Works ConsultedFeldstein NA, Anderson R (2006). Diagnosis and management of hydrocephalus. In FD Burg et al., eds., Current Pediatric Therapy, 18th ed., pp. 374–377. Philadelphia: Saunders Elsevier.Kinsman SL, Johnston MV (2011). Congenital anomalies of the central nervous system. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 19th ed., pp. 1998–2013. Philadelphia: Saunders.Prockop LD (2010). Hydrocephalus. In LP Rowland, TA Pedley, eds., Merritt's Neurology, 12th ed., chap. 53, pp. 349–355. Philadelphia: Lippincott Williams and Wilkins.Ropper AH, Samuels MA (2009). Disturbances of cerebrospinal fluid and its circulation, including hydrocephalus, pseudotumor cerebri, and low-pressure syndromes. In Adams and Victor's Principles of Neurology, 9th ed., pp. 591–611. New York: McGraw-Hill.Seiff ME (2003). Hydrocephalus section of Developmental, neurocutaneous, and genetic metabolic disorders. In RW Evans, ed., Saunders Manual of Neurologic Practice, pp. 889–895. Phildelphia: Saunders.Ulrich NJ (2011). Hydrocephalus and pseudotumor cerebri. In CD Rudolph et al., eds., Rudolph’s Pediatrics, 22nd ed., pp. 2174–2178. New York: McGraw-Hill.
January 24, 2012
John Pope, MD - Pediatrics & Christian G. Zimmerman, MD, FACS, MBA - Neurological Surgery
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