Hydrocephalus
Hydrocephalus | |
---|---|
Other names | Water on the brain |
Diagnostic method | Based on symptoms and medical imaging[1] |
Treatment | Surgery[1] |
Prognosis | Variable, often normal life[1] |
Frequency | Varies throughout the world, from 1 per 256 live births to 1 per 9,000, depending on access to prenatal health care, prenatal tests, and abortion[1][3] |
Hydrocephalus is a condition in which an accumulation of
Hydrocephalus can occur due to
Hydrocephalus is typically treated by the surgical placement of a
About one to two per 1,000 newborns have hydrocephalus.
Signs and symptoms
The clinical presentation of hydrocephalus varies with
Symptoms of increased ICP may include
Hakim's triad of
In infants with hydrocephalus, CSF builds up in the central nervous system (CNS), causing the fontanelle (soft spot) to bulge and the head to be larger than expected. Early symptoms may also include:[12]
- Eyes that appear to gaze downward
- Irritability
- Seizures
- Separated sutures
- Sleepiness
- Vomiting
Symptoms that may occur in older children can include:[12]
- Brief, shrill, high-pitched cry
- Changes in personality, memory, or the ability to reason or think
- Changes in facial appearance and eye spacing (craniofacial disproportion)
- Crossed eyes or uncontrolled eye movements
- Difficulty feeding
- Excessive sleepiness
- Headaches
- 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)
- Delayed milestones
- Failure to thrive
- Slow or restricted movement
- Vomiting[13]
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.[1] Hydrocephalus that is present from birth can cause long-term complications with speech and language. Children can have issues such as nonverbal learning disorder, difficulty understanding complex and abstract concepts, difficulty retrieving stored information, and spatial/perceptual disorders. Children with hydrocephalus are often known in having the difficulty in understanding the concepts within conversation and tend to use words they know or have heard.[14][15] However, the severity of hydrocephalus can differ considerably between individuals, and some are of average or above-average intelligence. Someone with hydrocephalus may have coordination and visual problems, or clumsiness. They may reach puberty earlier than the average child (this is called precocious puberty). About one in four develops epilepsy.[16]
Cause
Congenital
Congenital hydrocephalus is present in the infant prior to birth, meaning the fetus developed hydrocephalus
Other causes of congenital hydrocephalus include
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, bulging, firm anterior and posterior fontanelles may be present even when the person is in an upright position.[citation needed]
The infant exhibits fretfulness, poor feeding, and frequent vomiting. As the hydrocephalus progresses,
About 80–90% of fetuses or newborn infants with
Acquired
This condition is acquired as a consequence of CNS
Type
The cause of hydrocephalus is not known with certainty and is probably multifactorial. It may be caused by impaired CSF flow, reabsorption, or excessive CSF production.[25]
- Obstruction to CSF flow hinders its free passage through the ventricular system and congenital malformations) and can cause increases in ICP.[26]
- Hydrocephalus can also be caused by overproduction of CSF (relative obstruction) (e.g., choroid plexus papilloma, villous hypertrophy).[27][28]
- Bilateral ureteric obstruction is a rare, but reported, cause of hydrocephalus.
Hydrocephalus can be classified into communicating and noncommunicating (obstructive). Both forms can be either congenital or acquired.[29]
Communicating
Communicating hydrocephalus, also known as nonobstructive hydrocephalus, is caused by impaired CSF reabsorption in the absence of any obstruction of CSF flow between the ventricles and subarachnoid space. This may be due to functional impairment of the
Noncommunicating
Noncommunicating hydrocephalus, or obstructive hydrocephalus, is caused by an obstruction to the flow of CSF.[31]
- Foramen of Monro obstruction may lead to dilation of one, or if large enough (e.g., in colloid cyst), both lateral ventricles.
- The aqueduct of Sylvius, normally narrow, may be obstructed by a number of genetic or acquired lesions (e.g., atresia, ependymitis, hemorrhage, or tumor) and lead to dilation of both lateral ventricles, as well as the third ventricle.
- Fourth ventricle obstruction leads to dilatation of the aqueduct, as well as the lateral and third ventricles (e.g., Chiari malformation).
- The foramina of Luschka and foramen of Magendie may be obstructed due to congenital malformation (e.g., Dandy–Walker malformation).
Other
- Normal pressure hydrocephalus (NPH) is a particular form of chronic communicating hydrocephalus, characterized by enlarged cerebral ventricles, with only intermittently elevated cerebrospinal fluid pressure. Characteristic triad of symptoms are; dementia, apraxic gait and urinary incontinence. The diagnosis of NPH can be established only with the help of continuous intraventricular pressure recordings (over 24 hours or even longer), since more often than not instant measurements yield normal pressure values. Dynamic compliance studies may be also helpful. Altered compliance (elasticity) of the ventricular walls, as well as increased viscosity of the cerebrospinal fluid, may play a role in the pathogenesis.[32]
- Hydrocephalus ex vacuo also refers to an enlargement of cerebral ventricles and subarachnoid spaces, and is usually due to brain atrophy (as it occurs in dementias), post-traumatic brain injuries, and even in some psychiatric disorders, such as schizophrenia.[33] As opposed to hydrocephalus, this is a compensatory enlargement of the CSF-spaces in response to brain parenchyma loss; it is not the result of increased CSF pressure.[33]
Mechanism
Hydrocephalus is usually due to blockage of CSF outflow in the ventricles or in the subarachnoid space over the brain. In a person without hydrocephalus, CSF continuously circulates through the brain, its ventricles and the spinal cord and is continuously drained away into the circulatory system. Alternatively, the condition may result from an overproduction of the CSF, from a congenital malformation blocking normal drainage of the fluid, or from complications of head injuries or infections.[35]
Compression of the brain by the accumulating fluid eventually may cause neurological symptoms such as
The elevated ICP may cause compression of the brain, leading to brain damage and other complications. A complication often overlooked is the possibility of hearing loss due to ICP. The mechanism of ICP on hearing loss is presumed that the transmission of CSF pressure to and from the Perilymphatic space through a patent cochlear aqueduct.[37][38] The cochlear aqueduct connects the Perilymphatic space of the inner ear with the subarachnoid space of the posterior cranial fossa.[39] A loss of CSF pressure can induce Perilymphatic loss or endolymphatic hydrops resembling the clinical presentation of Ménière's disease associated hearing loss in the low frequencies.[37]
CSF can accumulate within the ventricles, this condition is called internal hydrocephalus and may result in increased CSF pressure. The production of CSF continues, even when the passages that normally allow it to exit the brain are blocked. Consequently, fluid builds inside the brain, causing pressure that dilates the ventricles and compresses the
Treatments
Procedures
Hydrocephalus treatment is surgical, creating a way for the excess fluid to drain away. In the short term, an
A shunt system can also be placed in the lumbar space of the spine and have the CSF redirected to the peritoneal cavity (
External hydrocephalus
External hydrocephalus is a condition generally seen in infants which involves enlarged fluid spaces or subarachnoid spaces around the outside of the brain. This condition is generally
Shunt complications
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) or infected, or it is outgrown. If this happens, the CSF begins to accumulate again and a number of physical symptoms develop (headaches, nausea, vomiting, photophobia/light sensitivity), some extremely serious, such as seizures. The shunt failure rate is also relatively high (of the 40,000 surgeries performed annually to treat hydrocephalus, only 30% are a person's first surgery) and people not uncommonly have multiple shunt revisions within their lifetimes.[49]
Another complication can occur when CSF drains more rapidly than it is produced by the
Following placement of a ventriculoperitoneal shunt there have been cases of a decrease in post-surgery hearing. It is presumed that the cochlea aqueduct is responsible for the decrease in hearing thresholds. The cochlea aqueduct has been considered as a probable channel where CSF pressure can be transmitted. Therefore, the reduced CSF pressure could cause a decrease in Perilymphatic pressure and cause secondary endolymphatic hydrops.[39] In addition to the increased hearing loss, there have also been findings of resolved hearing loss after ventriculoperitoneal shunt placement, where there is a release of CSF pressure on the auditory pathways.[51]
The diagnosis of CSF 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 person is upright or in a prone position, with the head at roughly the same level as the feet.[52]
Standardized protocols for inserting cerebral shunts have been shown to reduce shunt infections.[53][54] There is tentative evidence that preventative antibiotics may decrease the risk of shunt infections.[55]
Epidemiology
The hydrocephalus disease burden are concentrated in the developing world while North America has the least number of cases. A systematic review in 2019 estimated that there are 180,000 childhood hydrocephalus cases from the African continent per year, followed by 90,000 cases from Southeast Asia and the Western Pacific. Latin America also has a high prevalence of hydrocephalus. However, data on hydrocephalus disease burden in adults are lacking.[56]
History
In the pre-historic area, there were various paintings or artifacts depicting children or adults with macrocephaly (large head) or clinical findings of hydrocephalus. However, due to lack of writing, it was unknown how the people thought of the disorder at that time and the ways to treat the disease.[57]
References to hydrocephalic skulls can be found in ancient Egyptian medical literature from 2,500 BC to 500 AD.[58] Hydrocephalus was described more clearly by the ancient Greek physician Hippocrates in the fourth century BC, while a more accurate description was later given by the Roman physician Galen in the second century AD.[58]
The first clinical description of an operative procedure for hydrocephalus appears in the
The skull of a newborn baby is often full of liquid, either because the matron has compressed it excessively or for other, unknown reasons. The volume of the skull then increases daily, so that the bones of the skull fail to close. In this case, we must open the middle of the skull in three places, make the liquid flow out, then close the wound and tighten the skull with a bandage.
In 1881, a few years after the landmark study of Retzius and Key, Carl Wernicke pioneered sterile ventricular puncture and external drainage of CSF for the treatment of hydrocephalus.[58] It remained an intractable condition until the 20th century, when cerebral shunt and other neurosurgical treatment modalities were developed.[citation needed]
It is a lesser-known medical condition; relatively little research is conducted to improve treatment, and still no cure has been found. In developing countries, the condition often goes untreated at birth. Before birth, the condition is difficult to diagnose, and access to medical treatment is limited. However, when head swelling is prominent, children are taken at great expense for treatment. By then, brain tissue is undeveloped and neurosurgery is rare and difficult. Children more commonly live with undeveloped brain tissue and consequential intellectual disabilities and restrictions.[59]
Society and culture
Name
The word hydrocephalus is from the Greek ὕδωρ, hydōr meaning 'water' and κεφαλή, kephalē meaning 'head'.[7] Other names for hydrocephalus include "water on the brain", a historical name, and "water baby syndrome".[1][60]
Awareness campaign
September was designated National Hydrocephalus Awareness Month in July 2009 by the
Exceptional case
One case of hydrocephalus was a man whose brain shrank to a thin sheet of tissue, due to a buildup of cerebrospinal fluid in his skull. As a child, the man had a shunt, but it was removed when he was 14. In July 2007, at age 44, he went to a hospital due to mild weakness in his left leg. When doctors learned of the man's medical history, they performed a CT and MRI scan, and were astonished to see "massive enlargement" of the lateral ventricles in the skull. Dr. Lionel Feuillet of Hôpital de la Timone in Marseille said, "The images were most unusual... the brain was virtually absent."[61] Intelligence tests showed the person had an IQ of 75, considered "Borderline intellectual functioning", just above what would be officially classified as intellectually disabled.[62][63]
The person was a married father of two children, and worked as a civil servant, leading an at least superficially normal life, despite having enlarged ventricles with a decreased volume of brain tissue. "What I find amazing to this day is how the brain can deal with something which you think should not be compatible with life", commented Dr. Max Muenke, a pediatric brain-defect specialist at the National Human Genome Research Institute. "If something happens very slowly over quite some time, maybe over decades, the different parts of the brain take up functions that would normally be done by the part that is pushed to the side."[64][65][66]
Notable cases
- Ice hockey player Colby Cave had acute obstructive hydrocephalus due to a colloid cyst.[67]
- Author Sherman Alexie, born with the condition, wrote about it in his semi-autobiographical junior fiction novel The Absolutely True Diary of a Part-Time Indian.[68]
- Prince William, Duke of Gloucester (1689–1700), probably contracted meningitis at birth, which resulted in this condition.[69]
- Emperor Ferdinand I of Austria (1793–1875) became emperor in 1835 despite various health issues including hydrocephalus and epilepsy.[citation needed]
- In the American folklore of the states of Ohio, Michigan, and Connecticut, an urban legend exists about the melon heads, the inbred descendants of families of people born with hydrocephaly.
- Masato Kudo, a professional soccer player, died of hydrocephalus on October 21, 2022.
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