External hydrocephalus of the brain: diagnosis and treatment

Hydrocephalus (from the ancient Greek. ωδωρ “water” + κεφαλή “head”), brain water - a disease characterized by excessive accumulation of cerebrospinal fluid in the ventricular system of the brain as a result of the difficulty of its movement from the secretion site (ventricles of the brain) to the place of absorption into the circulatory system (subarachnoid space) - occlusive hydrocephalusor as a result of impaired absorption - isorbate hydrocephalus.


skull of a patient with hydrocephalus
ICD-10G 91 91., Q 03 03.
ICD-10-KMG91 and G91.9
ICD-9331.3 331.3 , 331.4 331.4 , 741.0 741.0 , 742.3 742.3
OMIM123155, 236600, 236635, 307000, 615219, 236600, 615219, 123155, 236635 and 307000
eMedicineneuro / 161

Hydrocephalus - a person suffering from hydrocephalus.

In ancient medical literature, hydrocephalus was rarely described, although its existence and symptoms were well known. The father of medicine Hippocrates is considered the first doctor to attempt to document the treatment of hydrocephalus. He recommended trepanation for the treatment of epilepsy, blindness, and possibly hydrocephalus. The Greeks reportedly treated hydrocephalus by wrapping the patient’s head in the bark and inserting it into the trepanation holes.

Hydrocephalus occurs at any age, but most often in early childhood, due to various reasons: tumors, inflammation, traumatic brain injury, congenital anomalies.

Hydrocephalus in a newborn can be caused by a generic craniocerebral trauma, which the mother transferred during pregnancy to infectious diseases (cytomegalovirus infection), leading to disruption of the ventricular system of the fetal brain. This, in turn, leads to obstruction of the circulation of cerebrospinal fluid and / or its excess production. In addition to congenital hydrocephalus, it can develop (most often in the first months of a newborn's life) and acquired hydrocephalus after suffering meningitis, meningoencephalitis, head injuries, intoxication, etc.

Violation of the circulation of cerebrospinal fluid leads to an increase in intracranial pressure and the so-called hypertension-hydrocephalic syndrome. As a result of the pressure exerted on brain areas, vision begins to decrease, convulsions appear, compression of the brain stem is manifested by oculomotor disorders (squint, paresis of gaze up (symptom of the setting sun)), weakness in the upper and lower limbs. This can lead to death, gross neurological disorders, intellectual impairment.

Forms of hydrocephalus are divided into: open (communicating) and closed (occlusal), external and internal, with the course of acute and chronic, compensated and decompensated. The open form is characterized by the expansion of all ventricular systems of the brain and the absence of an obstacle to the flow of cerebrospinal fluid throughout the cerebrospinal fluid system. With a closed form, a violation of the cerebrospinal fluid inside the ventricular system can be associated with abnormalities of its development, adhesions, neoplasms. Disruption of cerebrospinal fluid communication is possible at different levels: the level of the Monroe opening, III ventricle, the brain water supply, the holes of Lushka and Mazhandi, the large occipital opening. When the outer form of the fluid accumulates in the subarachnoid space, with the internal - in the ventricles of the brain. The open form of hydrocephalus can be external and internal, and the occlusal form is usually internal. With an open external form, subarachnoid spaces overflow and expand. With an open internal form, a sharp dilatation of the ventricular system and thinning of the substance of the brain.

The most characteristic sign of hydrocephalus in newborns is an outstripping growth of the head circumference, leading to a visually well-defined hydrocephalic shape of the skull, greatly increased in volume. A sign of hydrocephalus is a bulging, strained fontanel, frequent tipping of the head, and displacement of eyeballs to the bottom. In places where a normal accretion of the bones of the skull has not occurred, rounded pulsating protrusions may form. Strabismus and nystagmus often occur. High irritability due to headaches is noted, the child eats poorly, often cries, vomits, he is lethargic. Sometimes you may notice a decrease in vision and hearing.

Hydrocephalus at an older age is characterized by headaches, especially in the morning, nausea and vomiting at the height of a headache, dizziness, head sizes are not increased. On radiographs of the skull, the destruction of the Turkish saddle, an increase in finger impressions, are noted. The most informative magnetic resonance and computed tomography, detecting sharply enlarged ventricles of the brain.

The main treatment for hydrocephalus is surgical. To reduce intracranial pressure, diuretics are prescribed as a therapeutic measure.

Shunting Edit

Since the 50s of the XX century, a bypass surgery to restore the movement of cerebrospinal fluid has been the standard treatment for any form of hydrocephalus. After craniotomy, one end of the shunt, ending with a radiopaque catheter, is inserted into the cavity of the dilated ventricle. The intermediate, longest part made of silicone is located subcutaneously. The distal end, also having a catheter, opens in the abdominal or chest cavity, which provides drainage. The shunt is equipped with a pump that automatically regulates cerebrospinal fluid pressure .. Starting from the mid-80s, endoscopic operations began to occupy a significant place in the treatment of hydrocephalus.

Treatment of occlusal hydrocephalus with bypass surgery is quite effective, however, according to various sources, complications during this operation are 40-60% of cases. At the same time, depending on the cause of the dysfunction, the entire shunt or its parts must be replaced. Experience shows that the most common complications requiring revision of the shunt, occur in the period from six months to one year after surgery. Most patients who underwent shunt surgery have to undergo several surgical interventions throughout their lives. In any case, at least two or more revisions should be expected - because the child is growing. After shunt operations, the patient becomes shunt-dependent, that is, his entire future life will depend on the operation of the shunt.

Complications of Shunt Operations Edit

  • Occlusion (occlusion) in the ventricles of the brain and in the abdominal cavity.
  • Infection of the shunt, ventricles of the brain, meninges.
  • Mechanical damage to the shunt.
  • Hyperdrainage (rapid discharge of cerebrospinal fluid from the ventricles) is often accompanied by a rupture of convexital veins and the formation of hematomas.
  • Hypodrainage (slow outflow from the ventricles) - the operation in this case is ineffective.
  • The development of epileptic syndrome, pressure sores of the abdominal cavity, etc.

Endoscopic treatment for hydrocephalus Edit

Currently, endoscopic treatment of hydrocephalus is a priority in the world practice of neurosurgery. Types of surgical endoscopic interventions for hydrocephalus:

  • endoscopic ventriculocysternostomy of the bottom of the third ventricle,
  • aqueductoplasty
  • ventriculocystocysternostomy,
  • septostomy
  • endoscopic removal of an intraventricular brain tumor,
  • endoscopic shunt system installation.

The remaining types of surgical interventions have not yet found wide application in clinical practice.

Endoscopic ventriculocysternostomy of the bottom of the third ventricle

This operation has found wide application and occupies about 80% of neuroendoscopic operations in hydrocephalus. The purpose of the operation is to create pathways for the outflow of fluid from the ventricular system of the brain (III ventricle) into the tanks of the brain, through the paths of which the reabsorption (absorption) of fluid occurs in a healthy person.

Indications for surgery:

  • primary operation for occlusal hydrocephalus with an occlusion level from the posterior parts of the third ventricle and distal,
  • an alternative operation for complications of shunt operations with the removal of a previously installed shunt system (instead of the operation “revision of the shunt system”),
  • post-traumatic hydrocephalus,
  • mixed hydrocephalus (internal and external),
  • selection operation when removing the shunt system to achieve shunt independence,

The advantages of surgery compared to classic shunts:

  • the operation restores the physiological (as in a healthy person) liquor flow from the ventricular system of the brain to the basal cisterns,
  • there is no implantation of a foreign body (shunt system) into the body and thus the problems associated with it (infection, malfunction, the need for revisions) are eliminated,
  • significantly lower risk of over-training and related complications (subdural hematomas, hydromas, etc.),
  • less invasive surgery,
  • surgery is more cost-effective for hospitals,
  • improving the quality of life.

Surgery is actually the only way to combat the disease. In most cases, medical methods can only slow down the course of the disease, but do not eliminate the root cause of the disease. In the event of a successful operation, an almost complete recovery is likely with a return to normal.

The prognosis for hydrocephalus depends on the cause and time of diagnosis and the appointment of adequate treatment. Children who receive treatment are able to live a normal life with small, if any, restrictions. In some cases, speech impairment may occur. Problems with a shunt infection or a malfunction may require a surgical reinstallation of the shunt.

Prevention of infectious diseases of the mother during pregnancy, extreme caution when taking any medications during pregnancy, and the prevention of neuroinfections in childhood. Screening ultrasound examination of pregnant women and newborns.

Currently, the diagnosis of “hypertension-hydrocephalic syndrome” is one of the most common diagnoses made by pediatric neuropathologists. However, in most cases, excessive diagnosis takes place, and the child does not require any medical treatment. When making such a diagnosis, constant monitoring of the development of the child is required: first of all, it is necessary to regularly monitor both the growth of the head circumference and the size of the ventricles of the brain (using neurosonography). A significant deviation of the measurement indices should be an immediate reason for a detailed examination using an MRI of the brain. However, it must be borne in mind that it is the dynamics of development that are the fundamental factor, insignificant static deviations from the norm can often be caused by the individual development of the child and should not cause panic. At the same time, the range of 33.0-37.5 cm is considered normal for the head circumference of a newborn. Most intensively, the head circumference increases in the first 3 months (about 1.5 cm every month). Further, the growth rate decreases, and by the year the circumference is on average 44.9 - 48.9 cm).

The concept and types of the disease

Hydrocephalus is characterized by an excessive content of cerebrospinal (cerebrospinal) fluid in the space under the meninges - the ventricular system of the brain, due to a violation of its circulation or production.

The result of these disorders is an increase in the ventricles and a "softening" of the medulla.

Hydrocephalus species

There are several types of disease:

  1. congenital - occurs due to developmental pathology, intrauterine infections,
  2. acquired - a complication after various kinds of injuries, inflammatory processes, vascular pathology, disorders in the cervical spine and so on.

According to the shape of the course, several types of the disease are divided:

  • Open external hydrocephalus of the brain is a violation of the absorption of cerebrospinal fluid, but cerebrospinal spaces that provide nutrition to the brain system with cerebrospinal fluid are freely reported.
  • Closed - there is no communication of liquor-bearing spaces.
  • External replacement hydrocephalus of the brain is allocated in a separate form, since in this case the volume of the gray matter itself decreases, and the freed space is filled with the above-mentioned liquid - there is a “substitution”.

The last form of the disease is the most dangerous - it can be completely asymptomatic for a long time due to compensation of the brain substance by the spinal contents.

That is, there are no pressure drops in the skull box and, as a result, there are no symptoms characteristic of this disease.

Like any other disease, hydrocephalus can occur in two stages: acute and chronic (moderate or severe).

Treatment of the acute stage should be started immediately (it is the result of congenital pathologies of the central nervous system), while the moderate one does not require radical actions and quite often is the result of injuries of various kinds.

But before proceeding with the treatment, we will deal with its symptoms.

All diseases of the human brain

The brain is an organ of the central nervous system. It coordinates all the vital functions of a person, controls his behavior. Speaking figuratively, this is the most complicated control room that receives information, analyzes it and selects the optimal scheme of action so that the human body can successfully function in constantly changing conditions. Brain diseases can bring down the settings in this complex system, disrupt or completely destroy the body control system. What can this lead to? To a violation of certain functions of the body, a change in behavior, deformation, or even the destruction of a person’s personality. In severe cases, “breakdown” of the brain means death.

The modern world is changing and complicating rapidly, and today, as never before, it is important to recognize and cure the disease of the brain at an early stage.

The brain suffers from diseases of various types:

  • - genetic (defects in DNA as a whole or in its individual parts),
  • - traumatic (penetrating or dull injury),
  • - immune (the immune system begins to attack and destroy brain tissue),
  • - infectious (exposure to viruses, fungi, parasites, bacteria, etc.),
  • - vascular (strokes, weakening of the walls or abnormal growth of blood vessels),
  • - malignant and benign tumors,
  • - other (death of brain cells, radiation damage, etc.).

Is it possible to prevent brain diseases?

It is impossible to prevent genetic factors of brain damage; in all other cases, it is realistic to reduce the likelihood of a disease by avoiding actions that are regarded as risk factors. Anything that increases the chances of getting a head injury, exposure to infections (toxoplasma, HIV, rabies, etc), radiation, chemicals, alcohol consumption, poor nutrition, smoking (and passive including) are all risk factors, and worth avoiding.

When should I sound the alarm?

What symptoms a brain disease manifests itself depends on its type. But there are a number of manifestations that are very likely to indicate that this organ is affected:

  • persistent or intermittent headache
  • - mood swings,
  • - loss of appetite,
  • - changes in behavior and personality,
  • - problems with hearing, balance, vision, smell,
  • - weakness
  • - impossibility to concentrate,
  • - numbness or paralysis of different parts of the body,
  • - memory loss,
  • - convulsions
  • - confusion,
  • - coma.

If any of these symptoms occur with you or your loved ones, medical advice is required!

Diagnosis and treatment

When diagnosing a brain disease, the doctor will be interested in the patient’s family history of the disease. Various blood tests are required, in many cases a brain scan needs to be done, and a definitive diagnosis for some types of lesions can only be made after a biopsy of brain tissue.

On the basis of the disease, as well as the severity of the lesion, treatment may be conservative (antibiotics, other drugs, chemotherapy, radiation therapy) or operative. Sometimes a combination of conservative and surgical treatment is effective. Unfortunately, there is no optimal treatment for some types of brain damage, and patients are prescribed supportive therapy, which reduces the onset of symptoms and inhibits the development of the disease.

Meningitis (inflammation of the lining of the brain), stroke (circulatory disorder), angiospasm or occlusion of cerebral vessels, hydrocephalus (fluid accumulation), concussion and contusion of the brain - not a complete list of problems that everyone can face. At the slightest suspicion of impaired brain function, you should seek medical advice.

Mild hydrocephalus

At the first stage, the disease may not manifest itself. This is the so-called mild degree of external hydrocephalus. If the degree of damage to the brain system is insignificant, the body can independently restore fluid circulation. Thus, mild external hydrocephalus of the brain has the most optimistic prognoses in terms of treatment and consequences.

For an adult, if the disease is not diagnosed, the pressure on the brain system increases and there is severe external hydrocephalus of the brain, which has certain symptoms:

  • headaches and migraines
  • nausea,
  • drowsiness,
  • visual impairment (double vision),
  • weakness,
  • increased fatigue.

If you experience similar symptoms as a result of a disease or injury, you should immediately consult a doctor.

External hydrocephalus of the brain

When the external form of hydrocephalus occurs, excessive formation of cerebrospinal fluid occurs, normally circulating in the structures of the brain and spinal cord and protecting it from mechanical damage. In addition, the cerebrospinal fluid supplies the brain with nutrition and removes metabolic products into the blood. As a result of pathological pressure, brain tissue atrophies, negative symptoms appear, with complications, the risk of coma and death is increased.

Classification of disease forms

External hydrocephalus is a form of dropsy of the brain, consisting in the excessive accumulation of cerebrospinal fluid (cerebrospinal fluid) in the subarachnoid and subdural spaces of the brain. The subarachnoid cavity is located between the soft and arachnoid membrane of the brain and spinal cord. A narrow slit-like subdural cavity is located between the solid and arachnoid membrane of the brain. It is penetrated by connective tissue fibers.

Types of external hydrocephalus:

Open form - communication of the cerebrospinal fluid circulation paths is not broken, changes affect the mechanism of cerebrospinal fluid absorption,

Closed form - between the spaces in which liquor is concentrated, and the ways of its circulation there is no message,

Hypersecretory form - the production of cerebrospinal fluid is excessive,

The external substitution form - the brain substance (parenchyma) is transformed, undergoing atrophy, and cerebrospinal fluid takes its place.

Classification of external hydrocephalus according to the pace of the course:

Acute - from the first signs to the initial decompensation, no more than 3 days pass,

Subacute - lasts for a month,

Chronic - occurs from 3 weeks to six months or more.

According to the level of cerebrospinal fluid pressure, it is divided into hypotensive, hypertensive and normotensive forms. The latent course of the disease, which lasts long enough under normal intracranial pressure, is most dangerous, since the symptoms of the pathology can grow suddenly and avalanche without any apparent prerequisites. Most often, this situation occurs with external replacement hydrocephalus.

There are congenital and external forms of dropsy of the brain. The congenital form occurs during fetal development, acquired hydrocephalus - as a result of trauma or inflammation of the brain structures.

Symptoms of external hydrocephalus

The human body has impressive compensatory abilities. A mild form of external hydrocephalus can pass almost unnoticed for the patient - the circulation of cerebrospinal fluid is restored independently. The prognosis of such a disease is most optimistic, and its consequences are minimal.

An increase in intracranial pressure leads to the appearance of the following symptoms:

Headache worse after prolonged horizontal position

Visual impairment (double vision)

All these manifestations are the result of a decrease in the density of brain structures due to the fact that they are saturated with cerebrospinal fluid, narrowing of the subarachnoid and subdural spaces, and incorrect resorption of the cerebrospinal fluid. When replacing the cerebral parenchyma with cerebrospinal fluid, the symptoms of the disease are exacerbated.

Symptoms of replacement external hydrocephalus:

Violation of large and fine motor skills,

Uncertain shaky gait

Involuntary urination and fecal incontinence,

Violations of intellectual activity, memory, attention,

If external hydrocephalus is diagnosed in an infant, the following symptoms occur:

The divergence of the joints between the bones of the skull,

Enlargement of the frontal part of the skull,

Swelling of the veins on the skull, clearly visible under thin, stretched skin in the form of a venous network,

Excessive increase in the circumference of the child’s head.

Causes of disease

Any disturbances in the normal functioning of brain structures can lead to the development of the disease.

Causes of external hydrocephalus:

Brain injuries

The consequences of injuries and fractures of the spine,

Tumors of any etiology,

Stroke, hematoma, cerebral hemorrhage,

Consequences of inflammatory processes or neuroinfections (meningitis, meningoencephalitis, tuberculosis, herpes, toxoplasmosis),

Pathology of the cervical vertebrae,

Circulatory system diseases

Age-related changes in brain structures,

Congenital anatomical changes in the central nervous system.

Diagnosis of external hydrocephalus

The most informative diagnostic method is magnetic resonance imaging, or MRI. A little less information can be obtained as a result of CT, or computed tomography.

What can be seen during the MRI:

Contours of the skull, ventricles, subarachnoid space, brain,

The presence of anomalies - cysts, tumors, hematomas, aneurysms,

The shape and size of the ventricles.

In addition to tomographic diagnostic methods, the following studies are prescribed:

X-ray of the skull base tanks - allows you to specify the type of hydrocephalus, circulating cerebrospinal fluid circulation,

Angiography, or x-ray of blood vessels - diagnoses the pathology of the blood supply to the brain,

A blood test for the presence of antibodies to possible pathogens of infections that have an impact on the development of the disease.

In addition to the appointment of instrumental methods, the doctor conducts a neuropsychological examination, allowing to clarify the symptoms of the disease, the time of their appearance.

Possible treatment options for external hydrocephalus

If the diagnosis of the disease has revealed a moderate degree of external hydrocephalus, the neurologist determines the drug treatment regimen. The goal of therapy is to restore the functioning of cerebral vessels and the activity of the central nervous system.

The patient takes diuretics (Diacarb) in combination with drugs that compensate for the excessive withdrawal of potassium and magnesium from the body, vitamin complexes, nootropics, vasodilators.

Additional treatments:

Special diet and diet

If within 2-3 months there is no improvement in performance, or the patient's condition worsens, surgical treatment is carried out.

Methods of surgical intervention:

Bypass surgery to remove excess cerebrospinal fluid,

An endoscopic operation to create additional pathways for the outflow of cerebrospinal fluid into the tanks of the brain, designed to naturally absorb this fluid.

The consequence of these operations is the restoration of normal circulation of cerebrospinal fluid, improving the quality of life of the patient, in some cases - complete compensation of the condition.

Education: In 2005, he completed an internship at the First Moscow State Medical University named after I. M. Sechenov and received a diploma in the specialty “Neurology”. In 2009, graduate school in the specialty "Nervous Diseases".

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Hydrocephalus replacement

If the necessary treatment is not carried out in time, then replacement brain hydrocephalus may occur, which has more tragic consequences:

  • signs of dementia
  • impaired coordination and gait,
  • arbitrary urination.

Often these symptoms are attributed to age-related changes, thereby complicating the consequences of the disease.

Conservative treatment

If a diagnosis is made: minor external hydrocephalus of the brain, then medical treatment is sufficient.

It is aimed at restoring the normal functioning of the central nervous system and blood vessels.

The age data of the patient, the degree of neglect of the disease and the root cause are necessarily taken into account.

The main methods of traditional treatment include:

  • gymnastics,
  • observance of a special diet,
  • taking anti-inflammatory, vasodilator, diuretic and other drugs.

Surgical treatment

If the progression of the disease has led to the development of external hydrocephalus replacement, then operative endoscopic intervention is possible, which is aimed at removing excess fluid from the brain.

Such operations are not uncommon and have great efficiency, in contrast to the bypass procedure.

Open external hydrocephalus of the brain somewhat simplifies the process of treatment and subsequent rehabilitation, in contrast to the closed form.

Nevertheless, practice shows that the appropriate observance of all the instructions of the doctor has approximately the same chances of recovery for any form of the disease.

As we found out, "dropsy" is not as harmless as it may seem at first. It has serious consequences that can cause irreparable damage to a person’s physical and mental health.

In advanced cases, even death is possible. Although, with timely access to specialists, the probability of living a normal full life in the future is very high.

Good health is the most valuable gift of nature, do not neglect it. Monitor your well-being and try to avoid injuries. And in case of disturbing “calls”, immediately consult a doctor. As the saying goes: "forewarned, then armed."

Watch the video: Hydrocephalus and its treatment. Boston Childrens Hospital (December 2019).