Hydrocephalus: What is it, Causes, Symptoms, Diagnosis and Treatment

Hydrocephalus is a disease that can be seen at any age, including in the womb.

Hydrocephalus is a disease that can be seen at any age, including in the womb. It is also known as accumulation of water in the brain. Hydrocephalus is a combination of the words hydro = water and cephali = head. It means ‘head filled with water’ in Greek.

What is Hydrocephalus?

Hydrocephalus occurs as a result of the accumulation of cerebrospinal fluid (CSF, Cerebrospinal Fluid) above normal in the head, brain ventricles and cavities, and in this disease, head growth generally occurs in the childhood age group.

Cerebrospinal fluid should not be considered as normal water. Because it has vital functions such as protecting the brain in case of severe impacts that can negatively affect our brain. This liquid contains nutritious content, just as it does in our blood. Approximately half a liter of CSF is produced in the brain per day, and this CSF is absorbed by the brain. In other words, CSF constantly goes through production, circulation and absorption processes in our body. It circulates in the brain cavities and between the sheaths covering the brain and spinal cord. Fluid accumulation may occur due to abnormal CSF production, absorption problems, and circulation problems as a result of stenosis or obstruction in the channels through which the fluid circulates. As a result of the accumulation of this fluid, pressure occurs in the brain, and this pressure is known to cause many damages in the brain. Various types of brain damage can lead to developmental, physical and mental impairments.

It is not a disease that can heal spontaneously or be treated with medication under the supervision of a doctor, and the only solution is surgery. After the diagnosis of the disease, surgical treatment should be performed as soon as possible. The disease can be fatal if treatment is delayed or not given at all. Diagnosis can be made even in the womb with various radiological techniques, and early diagnosis is very important.

The disease can be seen in any period, starting from infancy in the womb to advanced ages. However, the symptoms of this disease vary across age groups.

What are the causes of hydrocephalus?

Sometimes the cause of hydrocephalus cases cannot be clearly understood. The causes of hydrocephalus can be divided into two: congenital or acquired.

Causes of congenital hydrocephalus:

When a baby is born with hydrocephalus, it can be understood that there is a problem with CSF flow. This problem can also be seen in babies born prematurely, and some premature babies may even develop a brain hemorrhage.

• Some health problems such as spina bifida

• Some mutations seen due to the X chromosome

• Some rare genetic disorders (such as Dandy Walker malformation)

• Arachnoid cysts are fluid-filled sacs located between the brain or spinal cord and the arachnoid membrane.

Causes of hydrocephalus in children and adults:

The hydrocephalus that affects these children or adults is usually caused by disease, trauma, or injury that affects the brain. It is called acquired hydrocephalus.

• Infections in the membrane covering the brain and spinal cord (such as meningitis)

• Tumors in the brain

• Stroke

• Traumas and injuries to the head due to certain reasons

• Blood clots in the brain, a condition called venous thrombosis

• Bleeding conditions in the brain

• Congenital stenosis in the channels through which the cerebrospinal fluid circulates can sometimes cause symptoms in the late stages.

Causes of hydrocephalus in the elderly:

Hydrocephalus may develop in the elderly as a result of brain damage, brain hemorrhage, and some infections. It may develop in connection with chronic conditions that negatively affect blood flow, such as diabetes, heart disease or high cholesterol. These conditions are factors that may cause hydrocephalus, but the exact cause is unknown. We can especially see normal pressure hydrocephalus in these patients.

Hydrocephalus Symptoms

Hydrocephalus may cause different symptoms depending on its type and the age at which the disease occurs.

Symptoms of congenital hydrocephalus:

• The head is larger than normal due to blistering,

• Scalp so thin and shiny that the veins are easily visible

• Swelling or tightness due to blistering in the fontanel (the soft area on top of the baby’s head)

• Large cavities in the skull

• Nutritional disorder and imbalance

• state of being angry

• Vomiting

• Having a seizure (Epilepsy, epilepsy)

• Persistent drowsiness

• Stiffness and spasms in the muscles in the feet and legs

• Eye strain, vision problems

Symptoms of hydrocephalus in adults or children:

• Pain in the head (Brain fluid cannot drain very well due to staying in a lying position for a long time. For this reason, people may feel a more severe headache when they wake up in the morning. Sitting constantly can also increase the headache, and as the disease progresses, these headaches may become permanent unless treated.)

• Neck pain

• Feeling sick, tired and weak

• Frequent sleepiness (in advanced cases, even coma may occur)

• Mental fogginess and frequent confusion

• Vision problem, often blurry or double vision

• Having difficulty walking

• Urinary incontinence and fecal incontinence

• Short and high-pitched cries in children

• Changes in facial appearance along with changes in head size

• Developmental delay in children

• Slowing down and limiting movements

• Irritability and uncontrolled mood

• Having a seizure (Epilepsy, epilepsy)

Symptoms of normal pressure hydrocephalus:

It is usually seen in adults over the age of 60. The main symptom of this condition is sudden falls without loss of consciousness.

• Impairment in walking, inability to make turns easily (Typically, they walk as if there is a magnet on the ground)

• Problems in memory and forgetfulness due to impairment of mental functions

• Feeling pain in the head

• Inability to hold urine or feces

How to Diagnose Hydrocephalus?

Neuroradiological techniques developed thanks to the advancement of technology and medicine are used in diagnosis. Examples of these techniques are MR imaging (CSF flow MRI) and Computed Tomography (CT). Thanks to these techniques, disease-related enlargements of the brain cavities and changes in the brain tissue can be detected, and a definitive diagnosis of hydrocephalus can be made by combining the symptoms observed with the patient examination.

This disease can also be encountered in babies in the womb before birth. MRI and ultrasonography are techniques that help determine and monitor changes in the brain development and brain spaces of the baby in the womb. The cause of the hydrocephalus seen with the MRI performed after the birth of the baby is determined.

How is Hydrocephalus Treated?

Early diagnosis is very important in hydrocephalus, as in other diseases. It is not possible to treat hydrocephalus with medication. The only treatment method is surgical interventions. Only neurosurgeons can perform these surgical interventions. There are many methods in surgical interventions, and the most accurate method is chosen according to the factors causing hydrocephalus.

If the cause of hydrocephalus is inadequate CSF circulation and the obstruction in the circulation channels is the reason, a surgical method should be chosen to remove the obstruction. The cause of these blockages may be conditions such as tumors or cysts. In cases where the blockage cannot be opened, the circulation channels of CSF in the brain can be replaced by surgical interventions.

shunt surgery

Generally, the circulation of this fluid cannot be restored to its former and healthy state and this fluid is transferred to any space in the body. In general, it is preferred to transfer the cerebrospinal fluid to a cavity in the abdomen or heart where it can be easily absorbed. A thin, long, elastic and silicone tube known as a “shunt” is used for this transfer process. This process must work in one direction and at a controlled speed. For this, a pump and thin tube located under the scalp are required. Thanks to this process, the pressure inside the brain is prevented from increasing. Considering that the fluid in the brain is produced every day, it can be understood that this system must work constantly. Parts of this system are under the skin but can be felt by touch or during a physical examination. In babies, it can be seen from the outside. The shunt does not need a power source such as a battery. If the necessary checks are carried out after this procedure, patients can continue their lives without any problems.

Endoscopic Ventriculostomy

Endoscopic (interventions performed with a camera system) systems are the preferred treatment method in suitable patients today. There is no need for a shunt system in this method, known as endoscopic third ventriculostomy (opening a new path for the fluid to circulate) and aquaductoplasty (opening the blocked area). In addition, the endoscopic method may be preferred in patients who have a shunt installed and have recurrent problems such as permanent shunt blockage, or in patients whose shunt is removed due to infection problems.

During the operation, after a small skin incision of 1-2 cm in length is made, the third ventricle is accessed with an endoscope (camera) through a small hole opened in the skull. A path is opened here for the spinal fluid to circulate in the lower front part. Thus, the accumulated liquid begins to drain from here. The surgery takes approximately 1 hour. The patient is discharged the next day.

This method, which is much more advantageous than shunt surgery in suitable patients, does not involve any plastic hose or system placed in our body. The risk of infection is less. Problems such as shunt blockage and malfunction are not observed. In addition, there are some surgery risks. In rare cases, the opening that is opened surgically and allows the fluid to drain may close again on its own, and the optic nerves may be affected.

In general, this method is not preferred for babies under 1 year old.

If the diagnosis is made in the womb, the baby should be delivered as soon as possible and the necessary surgical intervention for the disease should be performed as soon as possible.

Hydrocephalus is a disease that can be fatal if diagnosed late. Therefore, if you experience symptoms, do not forget to get checked at the nearest health institution.