Hydrocephalus
What is it?
Hydrocephalus is the presence of excess cerebrospinal fluid (CSF) in the ventricular system that washes the brain. The pathology can be congenital and acquired, occurring in various neurological diseases and injuries to the central nervous system, both in children and adults. In some cases, mainly in everyday life, this disease is also called dropsy of the brain.
Disease
The brain is the entire system of chambers and water tanks producing the CSF. All produced cerebrospinal fluid is absorbed into the subarachnoid space, from where it is transferred to the general circulation.
The CSF system contains 140 to 270 ml of cerebrospinal fluid, which is located in 4 chambers. Approximately 600 ml of CSF is produced daily, supporting the metabolism between brain tissues and blood. The production, circulation, and absorption of CSF are continuous and ongoing processes, but sometimes, for certain reasons, they are disrupted. In such cases, characteristic symptoms appear, and experts talk about hydrocephalus.
Types of hydrocephalus
There are three main forms of pathology:
- open when all the ventricles are dilated, but there are no obstructions to the flow of CSF;
- occlusive, where a barrier occurs during the movement of cerebrospinal fluid, hence the pathways are blocked;
- non-resorptive, when there is insufficient absorption in the subarachnoid space.
Depending on where the fluid is localized, there is internal hydrocephalus, excessive CSF in the ventricles, and external, when CSF accumulates in the subarachnoid space.
In the open type of hydrocephalus, the ventricles stretch and compress the brain substance, which causes thinning and degeneration of the nervous tissue. If there are blockages in the CSF, the pressure drops in different brain parts. Tumors, injuries, and inflammation can cause these barriers. In some forms of stroke, the brain tissue dissolves, and fluid takes its place. Roughly the same processes occur in old age.
Considering the causes of development, the disease can be congenital, when brain disorders result from peculiarities of intrauterine fetal development, and acquired, resulting from injuries and diseases during life.
Depending on the nature of the course, hydrocephalus can be of two types:
- stable – CSF pressure is slightly elevated, but it does not constantly increase.
- progressive – therapeutic measures do not lead to stabilization of the amount of cerebrospinal fluid, atrophy of the brain substance is possible.
The risk of experiencing hydrocephalus of the brain continues at any age in both adults and children. As a rule, the highest proportion of cases affects babies.
Symptoms of hydrocephalus
Symptoms of the disease vary in children and adults. The signs of hydrocephalus depend on the degree of pathology, the patient’s age, and the form and extent of the disease.
The disease is severe in young patients, almost always leading to delayed development. Due to the fact that the bones of the skull are not yet fused, the head of the child with hydrocephalus is huge, with swollen veins showing through the skin. The large fontanel is tense and does not pulsate. In severe cases, skull sutures split up. Characteristic appearance: thin skin on the head, protruding ears, difficulty looking (setting-sun phenomenon), deep-set eyes. Children lag behind in development, turn and sit down later than their peers, and have little interest in the environment.
Elimination of pathology in the first year of life restores normal mental and physical development of the child.
In young people, hydrocephalus can be complicated by infectious diseases, seizures, loss of consciousness, and bradycardia.
The main manifestation in adults is persistent and excruciating headaches, with a lack of response to painkillers. It is characterized by the feeling of pressure in the eyes, pain while moving the eyes, nausea, and vomiting.
Other neurological symptoms of brain hydrocephalus in older children and adults may include:
- loss of visual acuity;
- loss of field of vision;
- dizziness and staggering when walking;
- increased muscle tone;
- paresis and paralysis;
- sweeping, disproportionate movements;
- change of the handwriting;
- emotional and mental disorders.
Some patients have a sharp shift from indifference to euphoria, from pleasure to apathy, etc. In some cases, a person who has not previously shown anger begins to behave aggressively.
Causes of hydrocephalus
There are three main reasons for the development of pathology:
- excessive production of a cerebrospinal fluid;
- blood circulation disorders;
- malabsorption.
All these infringements occur for different reasons, such as:
- deformities of the nervous system;
- intrauterine infections;
- inflammation of the brain tissue and membranes;
- traumatic brain injuries;
- bleeding in the brain;
- cysts;
- tumours.
In infants, congenital hydrocephalus can also be caused by infections of mother during intrauterine fetal development, for example, HIV, syphilis, toxoplasmosis, and rubella.
Diagnosis
Feature signs of the disease are usually pointed out at a very first inspection. For further studies, the following methods are used:
- radiography – it shows thinned skull bones, their asymmetry, and an imprint on the brain, bones in the form of “fingerprints.”
- An MRI or CT scan will help identify developmental abnormalities and obstructions during the common pathways.
- Ultrasound (used mainly in children under one-year-old until the large fontanel does not overgrow); lumbar puncture, used for calculating the pressure of the cerebrospinal fluid and for studying CSF by laboratory methods, if necessary.
It is mandatory to consult an ophthalmologist, who will determine the condition of the vessels of the eye fundus and optic nerve, as well as a neurologist and several other specialists according to the indications.
Treatment of hydrocephalus
Treatment depends on the cause that caused the violation of the production or circulation of cerebrospinal fluid.
Treatment of hydrocephalus in adults and children can be conservative and operative. Treatment tactics is chosen by asupervising specialist, and in severe cases, a decision is made during a medical council.
Conservative treatment
It is only possible to achieve radical improvements conservatively in cases where the hydrocephalus has become a complication of another disease, for example, an infection, injury, or inflammation. The main focus is to eliminate the condition’s underlying cause; therefore, specialists select a regime of medication, physiotherapy procedures, and other methods.
The main medicines used are:
- diuretics;
- anti-inflammatory and antibacterial drugs;
- medications that prevent blood clotting.
Unfortunately, for those with congenital forms of pathology, conservative tactics are not very effective and may only become an addition to the main radical treatment.
Surgical treatment
- Ventriculoperitoneal (VP) Shunt. The most common surgical treatment for hydrocephalus is the insertion of a ventriculoperitoneal (VP) shunt. This involves placing a flexible tube with a valve into the brain’s ventricles. The tube diverts the excess CSF from the brain to the abdomen, where it can be absorbed. The valve regulates the flow of CSF and ensures it doesn’t drain too quickly.
- Endoscopic Third Ventriculostomy (ETV). Another surgical option is endoscopic third ventriculostomy (ETV). This procedure involves creating a hole in the floor of the third ventricle using an endoscope, allowing CSF to bypass the obstruction and flow directly towards the site of absorption at the surface of the brain. ETV is often preferred for non-communicating hydrocephalus.
- Shunt Systems with Programmable Valves. Advancements in technology have led to the development of shunt systems with programmable valves. These systems allow neurosurgeons to adjust the rate of CSF drainage without additional surgery, providing a more customized treatment approach based on the patient’s changing needs.
All these surgical procedures are performed in 640 hospitals worldwide (https://doctor.global/results/diseases/hydrocephalus). For example, endoscopic third ventriculostomy (ETV) can be done in 14 clinics across Turkey for approximate price of $5.5 K(https://doctor.global/results/asia/turkey/all-cities/all-specializations/procedures/endoscopic-third-ventriculostomy-etv).
Prevention
To reduce the risk of developing hydrocephalus, experts recommend the following:
- carefully plan your pregnancy without forgetting preventive check-ups and necessary treatment;
- avoid traumatic brain injuries, spinal cord injuries, and severe blows to the head.
- minimize stress;
- do not overwork yourself emotionally and mentally.
Performing timely and regular vaccination against diseases that can affect the brain (meningitis, encephalitis) and cause fetal hydrocephalus (toxoplasmosis, measles, rubella, etc.) is highly desirable.
Rehabilitation services
The level of recovery and the prognosis depend on many factors, including:
- patient’s age.
- type and degree of pathology.
- concomitant diseases;
- general health condition;
- patient’s compliance to doctor’s prescriptions;
Potential complications can include brain swelling, reduced or complete loss of hearing and vision, impaired cognitive function, and even death.
