Hydrocephalic syndrome

Hydrocephalic syndrome - 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 as a disorder of liquor hydrodynamics.

[1], [2], [3], [4], [5], [6]

Epidemiology

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

One Swedish study, which was conducted for ten years, showed that the prevalence of congenital hydrocephalus is 0.82 per 1000 live births.

[7], [8], [9], [10], [11]

Causes of the hydrocephalic syndrome

The causes of hydrocephalic syndrome can be congenital and acquired.

Congenital obstructive hydrocephalic syndrome

• Bickers-Adams syndrome (stenosis of the Sylvian aqueduct, characterized by severe difficulties in learning and deformation of the thumb).
• Dandy Walker's malformation (atresia of the holes of Magendie and Lushka).
• The Arnold Chiari defect is 1 and 2 types.
• Underdevelopment of the Monroe orifice.
• Aneurysms of the veins of Galena.
• Congenital toxoplasmosis.

Acquired obstructive hydrocephalus syndrome

• Acquired stenosis of the aqueduct (after infection or bleeding).
• Supratentorial tumors that cause tentorial hernias.
• Intraventricular hematoma.
• intraventricular tumors, tumors of the pineal gland and posterior cranial fossa, for example, ependymoma, astrocytomas, choroid papillomas, craniopharyngiomas, pituitary adenomas, hypothalamus or optic nerve gliomas, hamartomas, 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 vascular malformation).
• Infections - meningitis, cysticercosis.
• Increased venous pressure in the sinuses (may be associated with achondroplasia, craniostenosis, venous thrombosis).
• Iatrogenic causes are hypervitaminosis A.
• Idiopathic.

Other causes of hydrocephalic syndrome in adults

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

[12], [13], [14]

Risk factors

• Absence of any prenatal care.
• Arterial hypertension in the mother during pregnancy.
• Preeclampsia.
• Drinking alcohol during pregnancy.
• Heredity.

[15], [16], [17], [18]

Pathogenesis

Owing to the increase in the volume of the cerebrospinal fluid, the ventricles of the brain are dilated, afterwards, the cerebrospinal fluid penetrates through the ependymal openings into the white matter of the parenchymal space, which leads to its damage with the formation of later scarring. An untreated hydrocephalic syndrome can lead to death.

[19], [20], [21], [22], [23], [24], [25]

Symptoms of the hydrocephalic syndrome

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

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

Features of symptoms in children

Symptoms of hydrocephalic syndrome in children vary depending on whether it has an acute or gradual onset. An acute onset is characterized by irritability, vomiting, and impaired consciousness. The gradual beginning is manifested by a delay in the child's physical and mental development. Other signs include:

• Rapid increase in the circumference of the head.
• The widening of the veins of the head, the strained fontanel.
• Symptom of the setting sun (when looking down, a white strip of sclera can be seen).
• Symptom Macewen (sound when percussing the head there is a pronounced sound of an empty pot).
• An increase in the tone of the limbs.

Features of the symptoms of hydrocephalic syndrome in older children and adults

Sharp beginning:

• Headache and vomiting.
• Papillohedema and impaired vision.

Gradual beginning:

• Disturbance of gait due to spasticity in the legs.
• A large head (although the seams are closed, the skull is still enlarged due to a chronic increase in intracranial pressure.
• One-sided or bilateral palsy of the sixth nerve.

Other features specific to adults

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

[26], [27], [28], [29], [30]

Stages

By severity:

• easy;
• moderate.

By the degree of compensation and reversibility of pathological changes:

• compensated;
• subcompensated;
• decompensated.

[31], [32], [33]

Forms

Distinguish the following forms of pathology: hypertensive-hydrocephalic, hydrocephalic-atrophic syndromes.

[34], [35], [36], [37], [38]

Complications and consequences

Congenital hydrocephalus often has a fatal outcome during the first four years of life. Epilepsy, learning disorders and general development of the child are some of the most common complications of hydrocephalic syndrome.

[39], [40], [41], [42]

Diagnostics of the hydrocephalic syndrome

The characteristic symptoms of hydrocephalus allow the doctor-neurologist to accurately diagnose the disease and to diagnose it. In order to determine the degree and form of the disease, radiography, echoencephalography and computer or magnetic resonance imaging are additionally performed.

[43], [44], [45]

Differential diagnosis

Differentiate the hydrocephalic syndrome with the following pathologies:

• Tumors of the brain stem (gliomas, craniopharyngiomas, glioblastomas, meningiomas, oligodendrogliomas, pituitary tumors, primary CNS lymphoma).
• Migraine.
• Bleeding: epidural hematoma, intracranial hemorrhage, subdural hematoma.
• Epilepsy.
• Frontal and temporal dementia.
• Infectious Disease:
  • intracranial epidural abscess,
  • subdural empyema.

Treatment of the hydrocephalic syndrome

The methods of treatment of hydrocephalus depend on the etiology, intensity of severity of symptoms.

Medications can help delay surgery until the patient's condition is completely stabilized.

Drug treatment of the acquired hydrocephalic syndrome consists in prescribing diuretics for removing excess fluid and suppressing the secretion of cerebrospinal fluid (Diakarb, Veroshpiron), drugs for improving brain trophism (Actovegin, Piracetam, Asparcum), a common vitamin complex, sedatives (Diazepam).

In case of acute deterioration of the patient's condition, spinal puncture can be performed.

Treatment of congenital hydrocephalus often requires surgical intervention, which will be aimed at eliminating the cause of the disease (for example, removing the tumor, bruising, abscess), shunting operations (introducing a system of silicone tubules to drain the cerebrospinal fluid).

Treatment of this disease can drag on for many years. Shunts, under constant supervision, can stand for a long time. As necessary, they can be replaced.

Prevention

Some congenital forms of hydrocephalic syndrome can be diagnosed antenatally.

[46], [47]

Forecast

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

In order to improve the physical condition of the patient, massage and physiotherapy are prescribed. Such procedures must be performed only by specialists who have experience 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 be a prerequisite for severe consequences for patients of different ages. If untimely to provide medical care and this syndrome will cause complete loss of vision, paralysis, coma, mental retardation, including dementia. In adults, the disease is most often complicated by paralysis, coma development and brain atrophy. If the hydrocephalic syndrome is late detected or if treatment is wrong, it can even lead to death.

[48], [49], [50]