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Hydrocephalus: A build-up of fluid in the cavities deep within the brain ( water in the brain )

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Hydrocephalus– increased accumulation of cerebrospinal fluid in the cerebrospinal fluid system of the brain. Hydrocephalus accompanies many congenital and acquired neurological diseases. Clinically, it is manifested by signs of increased intracranial pressure (headache, nausea, pressure on the eyes), symptoms of compression of brain structures (vestibular ataxia, visual impairment, mental disorders, epileptic seizures) and symptoms characteristic of the disease that caused it. Diagnosis of hydrocephalus includes x-ray of the skull, ophthalmologic examinations, Echo-EG (in infants, neurosonography), MRI or CT of the brain. Surgical treatment of hydrocephalus makes it possible to correct congenital anomalies of the CSF system, to remove intracranial formations that violate CSF circulation, and to establish an outflow of CSF from the cranial cavity.

 

General information

Hydrocephalus in literal translation means “dropsy of the head.” In modern neurology , this is a frequently occurring clinical syndrome that can be observed in many diseases, congenital anomalies, or post-traumatic conditions of the brain. The occurrence of hydrocephalus is associated with certain disorders in the CSF system of the brain. Hydrocephalus affects people of all ages. Hydrocephalus can occur in newborns, be congenital, develop in children and adults, accompany atrophic processes occurring in the brain in the elderly. However, it is most often found in pediatric practice.

 

The reasons

3 pathological mechanisms lead to the accumulation of an excess amount of cerebrospinal fluid in the cerebrospinal fluid system: the production of an excess amount of cerebrospinal fluid, impaired absorption, or disorder of cerebrospinal fluid circulation. Hydrocephalus may be based on one of these mechanisms or their combination. Causes that cause disturbances in the functioning of the CSF system can act during fetal development and cause congenital hydrocephalus or affect the brain after birth and cause the appearance of the so-called acquired hydrocephalus. Causes of hydrocephalus include:

1. Congenital hydrocephalus :

  • malformations of the CSF system (atresia of the holes of Magendie and Luschka
  • defects in the structure of the subarachnoid space
  • stenosis of the sylvian aqueduct, Dandy-Walker syndrome , etc.)
  • craniovertebral anomalies ( Chiari anomaly , congenital basilar impression )
  • intrauterine infections (toxoplasmosis, congenital syphilis , cytomegaly , rubella), birth trauma .

2. Acquired hydrocephalus :

  • inflammatory processes in the brain and its membranes ( encephalitis , arachnoiditis , meningitis)
  • traumatic brain injury
  • vascular disorders ( bleeding into the ventricles , hemorrhagic stroke or  intracerebral hematomas  with a breakthrough of blood into the ventricles).
  • germination of the ventricles and compression of the CSF pathways against the background of a colloid cyst of the III ventricle and intracerebral tumors ( astrocytomas , germinomas , ganglioneuromas , etc.). In this case, the normal circulation of the cerebrospinal fluid and its outflow from the cranial cavity are disturbed.

Separately, an atrophic (replacement) form of hydrocephalus is distinguished, which occurs as a result of post-traumatic death or age-related atrophy of brain tissues. In this case, cerebrospinal fluid fills the space that is formed inside the cranium as a result of a decrease in the volume of the brain. Atrophic hydrocephalus in old age can develop against the background of a violation of the blood supply to the brain with atherosclerosis of the cerebral vessels, hypertension , diabetic macroangiopathy .

 

Pathogenesis

Normally, CSF (cerebrospinal fluid) is produced by the choroid plexuses of the interconnected ventricles of the brain. Its largest amount is formed in the lateral ventricles, from where the cerebrospinal fluid enters the third ventricle, and from it through the Sylvian aqueduct into the fourth ventricle. Then the cerebrospinal fluid enters the subarachnoid (subarachnoid) space, which extends over the entire surface of the brain, and in the caudal direction passes the region of the craniovertebral junction and further surrounds the spinal cord throughout its entire length. The cerebrospinal fluid located in the subarachnoid space is constantly absorbed by the arachnoid (arachnoid) membrane of the spinal cord and brain and enters the bloodstream. The above etiological factors that disrupt the production, movement and absorption of cerebrospinal fluid,

Classification

According to the etiological principle, congenital and acquired hydrocephalus are distinguished. According to the mechanism of occurrence, hydrocephalus is classified into open and closed forms.

  • Open hydrocephalus is associated with hyperproduction of CSF or impaired absorption during normal CSF circulation.
  • Closed hydrocephalus is caused by a violation of the outflow of cerebrospinal fluid due to compression, partial or complete obstruction of any part of the CSF system of the brain.

Depending on where the excess accumulation of CSF occurs, internal and external hydrocephalus are distinguished. Internal hydrocephalus is accompanied by the accumulation of cerebrospinal fluid in the ventricles of the brain. External hydrocephalus is characterized by an excess of CSF in the subarachnoid and subdural space.

According to the features of the course, hydrocephalus is classified as acute, subacute and chronic. Acute hydrocephalus is characterized by rapid development, in which a few days after the onset of the first signs of the disease, decompensation occurs. Subacute hydrocephalus develops within a month, and chronic – more than six months.

Of great clinical importance is the division of hydrocephalus into stabilized (compensated) and progressive (increasing).

  • Stabilized hydrocephalus does not increase and usually proceeds with normal CSF pressure.
  • Progressive hydrocephalus is characterized by aggravation of symptoms, accompanied by an increase in CSF pressure, poorly amenable to conservative therapy and leads to atrophy of brain tissue.

Symptoms of hydrocephalus

Hydrocephalus in adults

The accumulation of excess amounts of cerebrospinal fluid in a limited space of the cranium leads to an increase in intracranial pressure, which causes the most typical symptoms of hydrocephalus. In adults and older children, these include: intense headache not relieved by analgesics, nausea , vomiting, a feeling of pressure on the eyeballs. These symptoms may occur acutely or increase gradually, having a transient character at the beginning of the disease. Atrophic hydrocephalus often occurs without signs of increased intracranial pressure and is detected only during an additional examination of the patient.

In most cases, hydrocephalus is accompanied by neurological symptoms, which are caused both by compression of the brain structures by enlarged cerebrospinal fluid spaces, and by the underlying disease that causes the development of hydrocephalus. Most often with hydrocephalus, vestibular and visual disturbances are noted. The first includes vestibular ataxia , manifested by dizziness, gait instability, tinnitus and head noise, nystagmus . On the part of vision, there may be a significant decrease in visual acuity, loss of certain parts of the visual fields, congestive optic discs; with a long course of hydrocephalus, atrophy of the optic nerves may develop .

Hydrocephalus can occur with disorders of the motor and sensory spheres: paresis and paralysis, increased tendon reflexes and muscle tone, a decrease or complete loss of all types of sensitivity, and the formation of spastic contractures of the limbs. Occlusive hydrocephalus , caused by a violation of the CSF circulation in the region of the posterior cranial fossa, is characterized by symptoms of cerebellar ataxia : impaired coordination and gait, large-scale disproportionate movements, changes in handwriting, etc.

In some cases, hydrocephalus is accompanied by mental disorders, which in adults are more often manifested by violations of the emotional-volitional sphere: emotional instability, neurasthenia , causeless euphoria with a rapid transition to a state of indifference and apathy. With a sharp increase in intracranial pressure, aggressive behavior is possible.

Hydrocephalus in children

In children, due to the great compliance of the skull bones, there is no increase in intracranial pressure; hydrocephalus in them is accompanied by an increase in the size of the skull. In newborns and young children, hydrocephalus is characterized by too large head size, bulging of the scalp veins, tension and lack of pulsation of the large fontanel, and edema of the optic discs.

Often there is a symptom of the “setting sun” – restriction of upward movement of the eyeballs. There may be a divergence of the sutures of the skull. Tapping on the skull is accompanied by a characteristic sound (“cracked pot” symptom). In children of the first year of life, hydrocephalus leads to developmental delays. They later begin to hold their heads, roll over, sit and walk.

Children who have severe hydrocephalus are distinguished by a spherical shape of the head, its too large size, deep-set eyes, protruding ears, and thinning of the scalp. There may be a decrease in vision, increased muscle tone in the lower extremities, disorders of the cranial nerves. Unlike adults, in childhood, hydrocephalus is more often accompanied not by emotional and volitional disorders, but by intellectual insufficiency.

Children with hydrocephalus are usually inactive and obese . They are apathetic, lack of initiative, do not have attachment to relatives characteristic of their peers. Reducing the degree of hydrocephalus often leads to an increase in the intellectual abilities and activity of the child.

In adolescence, hydrocephalus often occurs acutely against the background of an infectious disease, mental or physical trauma. At the same time, it is accompanied by intense headache, repeated vomiting, bradycardia . There may be bouts of loss of consciousness, sometimes convulsive seizures . In some cases, there are episodic psychoses with hallucinatory or delusional syndrome.

Diagnostics

The clinical symptoms of hydrocephalus are usually so characteristic that they allow the neurologist to suspect its presence at the first examination of the patient. To determine the degree and form of hydrocephalus, as well as to identify the underlying disease, additional examinations are carried out:

  • X-ray . An x-ray of the skull reveals thinning of the bones of the skull and the divergence of the sutures between them; on the inner surface of the cranial vault, a symptom of “finger impressions” is observed. Hydrocephalus due to stenosis of the aqueduct of the brain is accompanied by a decrease in the volume of the posterior cranial fossa on radiographs of the skull. Hydrocephalus in Dandy-Walker syndrome, on the contrary, is characterized by an increase in the volume of the posterior cranial fossa on craniograms. Hydrocephalus during the closure of one of the interventricular communications is manifested by the asymmetry of the skull visible on the craniogram.
  • Computed or MR-tomography . Tomographic diagnostic methods make it possible to determine the nature of hydrocephalus, to identify the site of occlusion of the CSF tract or an existing congenital anomaly, to diagnose the causative disease (tumor, cyst, hematoma, etc.). With hydrocephalus, the use of MRI of the brain is most informative . If vascular disorders are suspected, MRA of cerebral vessels is indicated.
  • Echography . Of the ultrasound diagnostic methods for hydrocephalus, echoencephalography is used , which allows you to determine the degree of increase in intracranial pressure. In children of the first year of life, an ultrasound scan of the brain through an open fontanelle using ultrasonography is possible .
  • Ophthalmologist’s consultation . An ophthalmologist evaluates visual disturbances and the condition of the optic discs. As a rule, the list of ophthalmological examinations for hydrocephalus includes ophthalmoscopy , determination of visual acuity and perimetry .
  • Lumbar puncture . In the absence of contraindications, a lumbar puncture may be performed to identify the causative disease, followed by examination of the cerebrospinal fluid. Congenital hydrocephalus of infectious etiology requires PCR diagnostics to determine the type of infection that caused it.

Treatment of hydrocephalus

The choice of treatment for hydrocephalus depends on its etiology. Conservative therapy is often carried out with acquired hydrocephalus due to inflammatory diseases, traumatic brain injury, hemorrhage into the ventricles. The underlying disease is treated, and diuretics (acetazolamide, furosemide) are prescribed to reduce the degree of hydrocephalus and increased intracranial pressure.

Congenital hydrocephalus usually requires surgery to correct the underlying malformation. If hydrocephalus is due to the presence of a volumetric process in the brain, then it is also treated with a surgical method. According to indications, removal of intracranial hematoma , surgery to remove tumors, opening or total excision of a brain abscess , separation of adhesions in arachnoiditis, etc.

In cases where it is not possible to eliminate the cause of hydrocephalus, bypass surgery is used:

  • cystoperitoneal shunting
  • endoscopic ventriculocisternostomy of the fundus of the third ventricle
  • ventriculoperitoneal shunting
  • lumboperitoneal shunting
  • external ventricular drainage

They are aimed at creating additional pathways for the outflow of cerebrospinal fluid from the cranial cavity. Bypass surgery can be performed as an addition to the surgical treatment of the underlying disease, if during the operation it is not possible to restore the normal circulation of the CSF.

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