Hydrocephalus (from Greek hydro-, meaning "water", and kephalos, meaning "head") is a medical condition in which there is an abnormal accumulation of cerebrospinal fluid (CSF) in the brain. This causes increased intracranial pressure inside the skull and may cause progressive enlargement of the head if it occurs in childhood, potentially causing convulsion, tunnel vision, and mental disability. It was once informally called "Water on the brain."
Two types of hydrocephalus are commonly described non-communicating hydrocephalus and communicating hydrocephalus, although there is evidence that communicating forms can lead to obstruction of CSF flow in many instances.
In non-communicating hydrocephalus, the CSF in the ventricles can not reach the subarachnoid space. This results from obstruction of interventricular foramina, cerebral aqueduct, or the outflow foramens of the fourth ventricle (median and lateral apertures). The most common obstruction is in the cerebral aqueduct. A block at any of these sites leads rapidly to dilatation of one or more ventricles. If the skull is still pliable, as it is in children younger than 2 years, the head may enlarge.
In communicating hydrocephalus, the obstruction of CSF flow is in the subarachnoid space from prior bleeding or meningitis. This causes thickening of the arachnoid leading to blockage of the return-flow channels. In some patients, the spaces filled by CSF are uniformly enlarged without an increase in intercranial pressure. This special form of communicating hydrocephalus is called normal pressure hydrocephalus (NPH), which results specifically from impaired CSF reabsorption at the arachnoid granulations. NPH's clinical manifestations are gait abnormality, dementia, and involuntary urination. NPH usually occurs in elderly patients.
The clinical presentation of hydrocephalus varies with chronicity. Acute dilatation of the ventricular system is more likely to manifest with the nonspecific signs and symptoms of increased intracranial pressure. By contrast chronic dilatation (especially in the elderly population) may have a more insidious onset presenting, for instance, with Hakim's triad (Adams triad).
Symptoms of increased intracranial pressure may include headaches, vomiting, nausea, papilledema, sleepiness or coma. Elevated intracranial pressure may result in uncal and/or cerebellar tonsill herniation, with resulting life-threatening brain stem compression.
Hakim's triad of gait instability, urinary incontinence and dementia is a relatively typical manifestation of the distinct entity normal pressure hydrocephalus (NPH). Focal neurological deficits may also occur, such as abducens nerve palsy and vertical gaze palsy (Parinaud syndrome due to compression of the quadrigeminal plate, where the neural centers coordinating the conjugated vertical eye movement are located). The symptoms depend on the cause of the blockage, the person's age, and how much brain tissue has been damaged by the swelling.
In infants with hydrocephalus, CSF builds up in the central nervous system, causing the fontanelle (soft spot) to bulge and the head to be larger than expected. Early symptoms may also include:
- Eyes that appear to gaze downward;
- Separated sutures;
Symptoms that may occur in older children can include:
- Brief, shrill, high-pitched cry;
- Changes in personality, memory, or the ability to reason or think;
- Changes in facial appearance and eye spacing;
- Crossed eyes or uncontrolled eye movements;
- Difficulty feeding;
- Excessive sleepiness;
- Irritability, poor temper control;
- Loss of bladder control (urinary incontinence);
- Loss of coordination and trouble walking;
- Muscle spasticity (spasm);
- Slow growth (child 0–5 years);
- Slow or restricted movement;
- Vomiting .
Because hydrocephalus can injure the brain, thought and behavior may be adversely affected. Learning disabilities including short-term memory loss are common among those with hydrocephalus, who tend to score better on verbal IQ than on performance IQ, which is thought to reflect the distribution of nerve damage to the brain. However the severity of hydrocephalus can differ considerably between individuals and some are of average or above-average intelligence. Someone with hydrocephalus may have motion and visual problems, problems with coordination, or may be clumsy. They may reach puberty earlier than the average child (see precocious puberty). About one in four develops epilepsy.
The cranial bones fuse by the end of the third year of life. For head enlargement to occur, hydrocephalus must occur before then. The causes are usually genetic but can also be acquired and usually occur within the first few months of life, which include 1) intraventricular matrix hemorrhages in premature infants, 2) infections, 3) type II Arnold-Chiari malformation, 4) aqueduct atresia and stenosis, and 5) Dandy-Walker malformation.
In newborns and toddlers with hydrocephalus, the head circumference is enlarged rapidly and soon surpasses the 97th percentile. Since the skull bones have not yet firmly joined together, bulging, firm anterior and posterior fontanelles may be present even when the patient is in an upright position.
The infant exhibits fretfulness, poor feeding, and frequent vomiting. As the hydrocephalus progresses, torpor sets in, and the infant shows lack of interest in his surroundings. Later on, the upper eyelids become retracted and the eyes are turned downwards ("sunset eyes") (due to hydrocephalic pressure on the mesencephalic tegmentum and paralysis of upward gaze). Movements become weak and the arms may become tremulous. Papilledema is absent but there may be reduction of vision. The head becomes so enlarged that the child may eventually be bedridden.
About 80-90% of fetuses or newborn infants with spina bifida—often associated with meningocele or myelomeningocele—develop hydrocephalus.
This condition is acquired as a consequence of CNS infections, meningitis, brain tumors, head trauma, intracranial hemorrhage (subarachnoid or intraparenchymal) and is usually extremely painful.
The prognosis for patients diagnosed with hydrocephalus is difficult to predict, although there is some correlation between the specific cause of hydrocephalus and the patient's outcome. Prognosis is further complicated by the presence of associated disorders, the timeliness of diagnosis, and the success of treatment. Affected individuals and their families should be aware that hydrocephalus poses risks to both cognitive and physical development.
Hydrocephalus treatment is surgical, generally utilizing various types of cerebral shunts. It involves the placement of a ventricular catheter (a tube made of silastic), into the cerebral ventricles to bypass the flow obstruction/malfunctioning arachnoidal granulations and drain the excess fluid into other body cavities, from where it can be resorbed. Most shunts drain the fluid into the peritoneal cavity (ventriculo-peritoneal shunt), but alternative sites include the right atrium (ventriculo-atrial shunt), pleural cavity (ventriculo-pleural shunt), and gallbladder. A shunt system can also be placed in the lumbar space of the spine and have the CSF redirected to the peritoneal cavity (Lumbar-peritoneal shunt). An alternative treatment for obstructive hydrocephalus in selected patients is the endoscopic third ventriculostomy (ETV), whereby a surgically created opening in the floor of the third ventricle allows the CSF to flow directly to the basal cisterns, thereby shortcutting any obstruction, as in aqueductal stenosis. This may or may not be appropriate based on individual anatomy.
Examples of possible complications include shunt malfunction, shunt failure, and shunt infection, along with infection of the shunt tract following surgery (the most common reason for shunt failure is infection of the shunt tract). Although a shunt generally works well, it may stop working if it disconnects, becomes blocked (clogged), infected, or it is outgrown. If this happens the cerebrospinal fluid will begin to accumulate again and a number of physical symptoms will develop (headaches, nausea, vomiting, photophobia/light sensitivity), some extremely serious, like seizures. The shunt failure rate is also relatively high (of the 40,000 surgeries performed annually to treat hydrocephalus, only 30% are a patient's first surgery) and it is not uncommon for patients to have multiple shunt revisions within their lifetime.
Another complication can occur when CSF drains more rapidly than it is produced by the choroid plexus, causing symptoms -listlessness, severe headaches, irritability, light sensitivity, auditory hyperesthesia (sound sensitivity), nausea, vomiting, dizziness, vertigo, migraines, seizures, a change in personality, weakness in the arms or legs, strabismus, and double vision - to appear when the patient is vertical. If the patient lies down, the symptoms usually vanish quickly. A CT scan may or may not show any change in ventricle size, particularly if the patient has a history of slit-like ventricles. Difficulty in diagnosing overdrainage can make treatment of this complication particularly frustrating for patients and their families. Resistance to traditional analgesic pharmacological therapy may also be a sign of shunt overdrainage or failure.
The diagnosis of cerebrospinal fluid buildup is complex and requires specialist expertise. Diagnosis of the particular complication usually depends on when the symptoms appear - that is, whether symptoms occur when the patient is upright or in a prone position, with the head at roughly the same level as the feet.
Because the shunt systems are too expensive for most people in developing countries, such people often die without getting a shunt. Worse, the rate of revision in shunt systems adds to the cost of shunting many times. Looking at this point, a study done by Dr. Benjamin C. Warf compares shunt systems and highlights the role of low-cost shunt systems in most of the developing countries. This study was published in Journal of Neurosurgery: Pediatrics May 2005 issue. It compares the Chhabra shunt system to shunt systems from developed countries. The study was done in Uganda and the shunts were donated by the International Federation for Spina Bifida and Hydrocephalus.