Hydrocephalus

Hydrocephalus (Greek hydro- ὑδρο, water '; kephale κεφαλή, head '), also written Hydrocephalus is an abnormal enlargement of the liquorgefüllten fluid spaces (ventricles ) of the brain. He is also known as water on the brain or obsolete, brain dropsy.

Development of hydrocephalus

The cerebrospinal fluid ( CSF) is produced in specific glomeruli ( the choroid plexus ), especially in the two lateral ventricles, but also in the third and fourth ventricles. After passage of the ventricular CSF passes into the cisterna cerebellomedullary and thus into the outer CSF space. There it is distributed throughout the brain and spinal cord surrounding space, and is finally absorbed by the arachnoid villi in the area of ​​the superior sagittal sinus and the Spinalnervenaustritten back into the blood. Alternative conveyance routes exist in the area of exits of the spinal nerves in Venous Plexus, or the Lymphysystem. The constant total amount of cerebrospinal fluid in the adult amounts to approximately 150 milliliters. Since the choroid plexus per day produce about a pint of liquor, the liquor is replaced within 24 hours three times.

Absorption and production adhere normally in balance. But if too much CSF is produced, the connection is closed between the CSF spaces, or if too little liquid is absorbed, a hydrocephalus may develop.

Runoff and absorption can be usually the result of meningitis, or a congenital or neonatal malformation of the brain. Also, prenatal infections of the brain in fetuses (eg, lymphocytic choriomeningitis ) can cause hydrocephalus. In addition, bleeding into liquorführende brain structures and tumors come into consideration. Also, by increasing the pressure in the brain itself ( for example, by bleeding ), there is a Resorptionswiderstand. In very rare cases, choroid plexus may lead to a Liquorüberproduktion.

Hydrocephalus is distinguished from these forms of hydrocephalus ex vacuo, which arise enlarged subarachnoid spaces by the loss of brain tissue. Here there is no disturbance of CSF circulation, only the CSF total volume is increased.

Congenital causes

The following congenital diseases are often associated with the development of hydrocephalus:

  • Monosomy 4p (absence of the short arm of chromosome 4, resulting in severe brain damage with hydrocephalus and microcephaly )
  • Dysraphische malformation ( myelomeningocele, encephalocele, Syringomyelozele )
  • Arnold -Chiari malformation ( circulation of small parts of the brain and medulla oblongata through the foramen magnum into the spinal canal )
  • Dandy- Walker syndrome ( developmental disorder that is characterized by cystic enlargement of the 4th ventricle, often with dysplasia or cystic changes in the cerebellum, agenesis of the corpus callosum and, optionally, other malformations of the CNS )
  • Complex brain malformations
  • Bony abnormalities (eg, Klippel -Feil syndrome)

Acquired causes

Acquired causes are, for example:

  • Toxoplasmosis ( Toxoplasma gondii infection )
  • Meningitis and / or encephalitis ( inflammation of the brain)
  • Intracranial hemorrhage ( " posthämorrhagischer hydrocephalus " ) ( cerebral hemorrhage )
  • Exogenous causes (e.g., operations )
  • Brain tumors
  • Trauma ( " post-traumatic hydrocephalus " ) ( damage to the brain )
  • Liquorüberproduktion (eg, choroid plexus papilloma )
  • Liquorresorptionsstörungen
  • Increased protein concentration of the cerebrospinal fluid (increased viscosity of the cerebrospinal fluid and impairment of the osmotic balance )
  • Thrombosis in the head
  • Blood group incompatibility / rhesus incompatibility

Symptoms

In fetuses and infants, the head can be like a balloon distended because of the still unfixed cranial bones. Side effects can be, for example, headache, surge -like vomiting, double vision, and epilepsy. More intracranial pressure and neurological deficits are possible.

Since the skull after closure of the sutures and fontanelles by increase in size of the intracranial volume increase can not compensate, there is a rise in pressure in the skull with brain shift from shares in the direction of the great occipital foramen ( foramen magnum ). With this displacement vital parts of the brain can be jammed. Since these vital parts of the brain are essential for the function of the cardiovascular and respiratory system, performs a herniation with damage to these parts of the brain to the heart and circulatory and respiratory failure, and thus to death.

Forms of hydrocephalus

  • Hydrocephalus: extension only of the cerebral ventricles Occlusivus: disability of Liquorabflusses from the ventricle
  • Malresorptivus: in delayed Liquorrückresorption

In the latter form of hydrocephalus there is, in contrast to other forms of hydrocephalus no intracranial pressure.

Therapy

In case of increased intracranial pressure earliest possible relief by means of a Liquorableitung drainage is desired. Here, the cerebrospinal fluid is a shunt, for example, in the abdominal cavity, often derived in the right atrium of the heart or less in the pleural space. Depending on the location of the passage obstacle is the ventriculostomy a possible form of therapy in which the operational base of the lateral ventricle is punctured and so the outflow of cerebrospinal fluid is made possible in the basal cisterns.

Part of a therapy in utero is already possible, so a prenatal intervention taking place.

Congenital disorder Klippel–Feil syndrome Exogeny Osmosis Drain (surgery)
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