Hydrocephalus is an abnormal expansion of cavities (ventricles) within the brain that is caused by the accumulation of cerebrospinal fluid. Hydrocephalus comes from two Greek words: hydros means water and cephalus means head.
There are two main varieties of hydrocephalus: congenital and acquired. An obstruction of the cerebral aqueduct (aqueductal stenosis) is the most frequent cause of congenital hydrocephalus. Acquired hydrocephalus may result from spina bifida, intraventricular hemorrhage, meningitis
, head trauma, tumors, and cysts.
Hydrocephalus is the result of an imbalance between the formation and drainage of cerebrospinal fluid (CSF). Approximately 500 milliliters (about a pint) of CSF is formed within the brain each day, by epidermal cells in structures collectively called the choroid plexus. These cells line chambers called ventricles that are located within the brain. There are four ventricles in a human brain. Once formed, CSF usually circulates among all the ventricles before it is absorbed and returned to the circulatory system. The normal adult volume of circulating CSF is 150 ml. The CSF turn-over rate is more than three times per day. Because production is independent of absorption, reduced absorption causes CSF to accumulate within the ventricles.
There are three different types of hydrocephalus. In the most common variety, reduced absorption occurs when one or more passages connecting the ventricles become blocked. This prevents the movement of CSF to its drainage sites in the subarachnoid space just inside the skull. This type of hydrocephalus is called "noncommunicating." In a second type, a reduction in the absorption rate is caused by damage to the absorptive tissue. This variety is called "communicating hydrocephalus."
Both of these types lead to an elevation of the CSF pressure within the brain. This increased pressure pushes aside the soft tissues of the brain. This squeezes and distorts them. This process also results in damage to these tissues. In infants whose skull bones have not yet fused, the intracranial pressure is partly relieved by expansion of the skull, so that symptoms may not be as dramatic. Both types of elevated-pressure hydrocephalus may occur from infancy to adulthood.
A third type of hydrocephalus, called "normal pressure hydrocephalus," is marked by ventricle enlargement without an apparent increase in CSF pressure. This type affects mainly the elderly.
Hydrocephalus has a variety of causes including:
- congenital brain defects
- hemorrhage, either into the ventricles or the subarachnoid space
- infection of the central nervous system (syphilis, herpes, meningitis, encephalitis, or mumps)
Hydrocephalus is believed to occur in approximately one to two of every 1,000 live births. The incidence of adult onset hydrocephalus is not known. There is no known way to prevent hydrocephalus.
Causes and symptoms
Hydrocephalus that is congenital (present at birth) is thought to be caused by a complex interaction of genetic and environmental factors. Aqueductal stenosis, an obstruction of the cerebral aqueduct, is the most frequent cause of congenital hydrocephalus. As of 2001, the genetic factors are not well understood. According to the British Association for Spina Bifida
and Hydrocephalus, in very rare circumstances, hydrocephalus is due to hereditary factors, which might affect future generations.
Signs and symptoms of elevated-pressure hydrocephalus include:
- nausea and vomiting, especially in the morning
- disturbances in walking (gait)
- double vision
- subtle difficulties in learning and memory
- delay in children achieving developmental milestones
Irritability is the most common sign of hydrocephalus in infants. If this is not treated, it may lead to lethargy. Bulging of the fontanelles, or the soft spots between the skull bones, may also be an early sign. When hydrocephalus occurs in infants, fusion of the skull bones is prevented. This leads to abnormal expansion of the skull.
Symptoms of normal pressure hydrocephalus include dementia
, gait abnormalities, and incontinence (involuntary urination or bowel movements).
Imaging studies—x ray, computed tomography scan (CT scan), ultrasound, and especially magnetic resonance imaging
(MRI)—are used to assess the presence and location of obstructions, as well as changes in brain tissue that have occurred as a result of the hydrocephalus. Lumbar puncture (spinal tap) may be performed to aid in determining the cause when infection is suspected.
The primary method of treatment for both elevated and normal pressure hydrocephalus is surgical installation of a shunt. A shunt is a tube connecting the ventricles of the brain to an alternative drainage site, usually the abdominal cavity. A shunt contains a one-way valve to prevent reverse flow of fluid. In some cases of non-communicating hydrocephalus, a direct connection can be made between one of the ventricles and the subarachnoid space, allowing drainage without a shunt.
Installation of a shunt requires lifelong monitoring by the recipient or family members for signs of recurring hydrocephalus due to obstruction or failure of the shunt. Other than monitoring, no other management activity is usually required.
Some drugs may postpone the need for surgery by inhibiting the production of CSF. These include acetazolamide and furosemide. Other drugs that are used to delay surgery include glycerol, digoxin, and isosorbide.
Some cases of elevated pressure hydrocephalus may be avoided by preventing or treating the infectious diseases which precede them. Prenatal diagnosis of congenital brain malformation is often possible, offering the option of family planning.
The prognosis for elevated-pressure hydrocephalus depends on a wide variety of factors, including the cause, age of onset, and the timing of surgery. Studies indicate that about half of all children who receive appropriate treatment and follow-up will develop IQs greater than 85. Those with hydrocephalus at birth do better than those with later onset due to meningitis. For individuals with normal pressure hydrocephalus, approximately half will benefit by the installation of a shunt.
Toporek, Chuck, and Kellie Robinson. Hydrocephalus: A Guide for Patients, Families & Friends. Cambridge, Mass.: O'Reilly & Associates, 1999.
"Hydrocephalus." Review of Optometry 137, no. 8 (August 15, 2000): 56A.
Association for Spina Bifida and Hydrocephalus. 42 Park Rd., Peterborough, PE1 2UQ. UK 0173 355 5988. Fax: 017 3355 5985. email@example.com. 〈http://www.asbah.demon.co.uk〉.
Hydrocephalus Foundation, Inc., (HyFI). 910 Rear Broadway, Saugus, MA 01906. (781) 942-1161. HyFI1@netscape.net. http://www.hydrocephalus.org.
"Hydrocephalus." American Association of Neurological Surgeons/Congress of Neurological Surgeons. 〈http://www.neurosurgery.org/pubpages/patres/hydrobroch.html〉.
"Hydrocephalus." Institute for Neurology and Neurosurgery. Beth Israel Medical Center, New York, NY. 〈http://nyneurosurgery.org/child/hydrocephalus/hydrocephalus.htm〉.
"Hydrocephalus." National Library of Medicine. MEDLINEplus. http://www.nlm.nih.gov/medlineplus/hydrocephalus.html.
— Spaces in the brain that are located between portions of the brain and filled with cerebrospinal fluid.
— Fluid that circulates throughout the cerebral ventricles and around the spinal cord within the spinal canal.
— Specialized cells located in the ventricles of the brain that produce cerebrospinal fluid.
— One of several "soft spots" on the skull where the developing bones of the skull have yet to fuse.
— A small tube placed in a ventricle of the brain to direct cerebrospinal fluid away from the blockage into another part of the body.
— The constricting or narrowing of an opening or passageway.
— The space between two membranes surrounding the brain, the arachnoid and pia mater.
a condition characterized by abnormal accumulation of cerebrospinal fluid within the cerebral ventricular system. As a consequence, the ventricles are enlarged and the brain is diminutive. Called also water on the brain.
Although hydrocephalus occurs occasionally in adults, it is usually associated with a congenital defect in offspring.
There are two types of hydrocephalus, the distinction being based on whether there is abnormal absorption of the cerebrospinal fluid or an obstruction to its flow. In communicating hydrocephalus there is some abnormality in the capacity to absorb fluid from the arachnoid space. There is no obstruction to the flow of fluid between the ventricles. In noncommunicating hydrocephalus there is an obstruction at some point in the ventricular system. The cause of noncommunicating hydrocephalus usually is a congenital abnormality, such as stenosis of the aqueduct of Sylvius, or congenital atresia of the foramina of the fourth ventricle. Infections, intraventricular hemorrhage, trauma and tumors can produce acquired communicating hydrocephalus.
There are three forms that occur in cattle: in one there is gross distention of the cranium with normal facial bones; in the second there is a similar enlargement of the cranium with an accompanying achondroplastic dishing of the face and foreshortening of the maxilla and a shortening of the limb bones—these are the classical 'bulldog' calves; in the third the cranium is normal in size but there is internal hydrocephalus and the calves are blind and imbecile. There are a number of inherited hydrocephalitides in cattle. The disease also occurs in pigs but the inheritance is complex in that it is exacerbated by a concurrent hypovitaminosis A.
In dogs and cats, hydrocephalus is common in some toy breeds such as the Chihuahua in which a domed cranium is a desirable feature of conformation. It also occurs less often in adults in association with brain tumors and from infections such as toxoplasmosis and feline infectious peritonitis.
Hydrocephalus in a calf. By permission from Parkinson TJ, England GCW, Arthur GH, Arthur's Veterinary Reproduction and Obstetrics, Saunders, 2001
cerebrospinal fluid occupies space vacated by brain parenchyma because of malformation or degeneration. Examples are seen in fetal infection by bluetongue virus or bovine virus diarrhea virus, severe polioencephalomalacia in cattle, and cerebral infarction in cats.
the excess fluid is in the arachnoid space; rare in animals.
accompanied by increased cerebrospinal fluid pressure.
the excess fluid is within the ventricular system; common in domestic animals and may be congenital or acquired.
accompanied by normal cerebrospinal fluid pressure.