Hydrocephalus syndrome

Hydrocephalic syndrome is an increase in the volume of cerebrospinal fluid in the ventricles of the brain as a result of impaired absorption or excessive secretion.

The syndrome can be classified in various ways, but all forms are considered a disorder of cerebrospinal fluid fluid flow.

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Epidemiology

There is some evidence that the incidence of hydrocephalic syndrome in children has decreased significantly in many developed countries.

One Swedish study that was conducted over a ten-year period found the prevalence of congenital hydrocephalus to be 0.82 per 1,000 live births.

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Causes hydrocephalus syndrome

The causes of hydrocephalic syndrome can be congenital and acquired.

Congenital obstructive hydrocephalic syndrome

• Bickers-Adams syndrome (stenosis of the aqueduct of Sylvi, characterized by severe learning difficulties and deformity of the thumb).
• Dandy-Walker malformation (atresia of the foramina of Magendie and Luschka).
• Arnold-Chiari malformation types 1 and 2.
• Underdevelopment of the foramen of Monroe.
• Aneurysms of the veins of Galen.
• Congenital toxoplasmosis.

Acquired obstructive hydrocephalic syndrome

• Acquired aqueduct stenosis (after infection or bleeding).
• Supratentorial tumors causing tentorial hernias.
• Intraventricular hematoma.
• intraventricular tumors, tumors of the pineal gland and posterior cranial fossa, such as ependymoma, astrocytomas, choroid papillomas, craniopharyngiomas, pituitary adenomas, gliomas of the hypothalamus or optic nerve, hamartoma, metastatic tumors.

Acquired hydrocephalic syndrome in infants and children

• Tumor lesions (in 20% of all cases, for example, medulloblastoma, astrocytomas).
• Intraventricular hemorrhage (eg, prematurity, head trauma, or rupture of a vascular malformation).
• Infections - meningitis, cysticercosis.
• Increased venous pressure in the sinuses (may be associated with achondroplasia, craniosynostosis, venous thrombosis).
• Iatrogenic causes - hypervitaminosis A.
• Idiopathic.

Other causes of hydrocephalic syndrome in adults

• Idiopathic (one third of cases).
• Iatrogenic - surgical operations in the area of the posterior cranial fossa.
• All causes of hydrocephalus described in infants and children.

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Risk factors

• Lack of any prenatal care.
• Arterial hypertension in the mother during pregnancy.
• Preeclampsia.
• Alcohol consumption during pregnancy.
• Heredity.

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Pathogenesis

Due to the increase in the volume of cerebrospinal fluid, dilation of the cerebral ventricles occurs, subsequently, the cerebrospinal fluid penetrates through the ependymal openings into the white matter of the periventricular space, which leads to its damage with the subsequent formation of scars. Untreated hydrocephalic syndrome can lead to death.

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Symptoms hydrocephalus syndrome

Hydrocephalic syndrome can occur at any age in both adults and children.

Symptoms of hydrocephalic syndrome depend on age, disease progression, and individual tolerance to changes in cerebrospinal fluid pressure.

Peculiarities of symptoms in children

Symptoms of hydrocephalic syndrome in children vary depending on whether it has an acute or gradual onset. Acute onset is characterized by irritability, vomiting, and altered consciousness. Gradual onset is characterized by delays in the child's physical and mental development. Other signs include:

• Rapid increase in head circumference.
• Dilation of the veins of the head, tense fontanelle.
• Setting sun symptom (when looking down, a white stripe of sclera may be visible).
• Macewen's symptom (when percussing the head, the sound is a pronounced sound of an empty pot).
• Increased tone of the limbs.

Features of symptoms of hydrocephalic syndrome in older children and adults

Sharp onset:

• Headache and vomiting.
• Papilloedema and impaired upward gaze.

Gradual start:

• Gait disturbance due to spasticity in the legs.
• Large head (although the sutures are closed, the skull is still enlarged due to chronic increase in intracranial pressure.
• Unilateral or bilateral sixth nerve palsy.

Other characteristics typical for adults

• Cognitive impairment.
• Pain in the neck.
• Nausea and vomiting.
• Blurred and double vision.
• Urinary incontinence.

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Stages

By severity:

• easy;
• moderate.

According to the degree of compensation and reversibility of pathological changes:

• compensated;
• subcompensated;
• decompensated.

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Forms

The following forms of pathology are distinguished: hypertensive-hydrocephalic, hydrocephalic-atrophic syndromes.

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Complications and consequences

Congenital hydrocephalus is often fatal within the first four years of life. Epilepsy, learning disabilities, and general developmental disabilities are among the most common complications of hydrocephalic syndrome.

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Diagnostics hydrocephalus syndrome

Characteristic symptoms of hydrocephalus enable a neurologist to accurately determine the disease and make a diagnosis. In order to determine the degree and form of the disease, X-ray, echoencephalography and computed or magnetic resonance imaging are additionally performed.

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Differential diagnosis

Hydrocephalic syndrome is differentiated from the following pathologies:

• Brainstem tumors (gliomas, craniopharyngioma, glioblastoma, meningiomas, oligodendroglioma, pituitary tumors, primary CNS lymphoma).
• Migraine.
• Bleeding: epidural hematoma, intracranial hemorrhage, subdural hematoma.
• Epilepsy.
• Frontal and temporal dementia.
• Infectious diseases:
  • intracranial epidural abscess,
  • subdural empyema.

Treatment hydrocephalus syndrome

Treatment methods for hydrocephalus depend on the etiology, intensity and severity of symptoms.

Medications may help delay surgery until the patient's condition is fully stabilized.

Drug treatment of acquired hydrocephalic syndrome consists of prescribing diuretics to remove excess fluid and suppress the secretion of cerebrospinal fluid (Diacarb, Veroshpiron), drugs to improve brain trophism (Actovegin, Piracetam, Asparkam), a general vitamin complex, and sedatives (Diazepam).

In case of acute deterioration of the patient's condition, a lumbar puncture may be performed.

Treatment of congenital hydrocephalus most often requires surgical intervention, which will be aimed at eliminating the cause of the disease (for example, removal of a tumor, hematoma, abscess), shunting operations (introduction of a system of silicone tubes for drainage of cerebrospinal fluid).

Treatment of this disease can take many years. Shunts, under constant observation, can be installed for a long time. They can be replaced as needed.

Prevention

Some congenital forms of hydrocephalic syndrome can be diagnosed antenatally.

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Forecast

The prognosis of this disease depends on the form and degree of hydrocephalic syndrome. Children with this disease have a disability, although the degree of dysfunction can be either mild or severe.

In order to improve the patient's physical condition, massage and therapeutic exercise are prescribed. Such procedures must be performed only by specialists who have experience working with such patients.

If the diagnosis is correct and timely treatment is started, the prognosis is favorable. The prognosis in other cases depends on the cause of the syndrome.

Hydrocephalic syndrome can become a prerequisite for serious consequences for patients of different ages. If medical assistance is not provided in a timely manner, this syndrome will cause complete loss of vision, paralysis, coma, delayed mental development, including dementia. In adults, the disease is most often complicated by paralysis, coma, and brain atrophy. If hydrocephalic syndrome is detected late or the wrong treatment is prescribed, it can even lead to death.

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