Hydrocephalus
A hydrocephalus ( Greek ὕδωρ hydōr 'water', κεφαλή kephalē 'head'), also written hydrocephalus , is a pathological expansion of the fluid spaces ( cerebral ventricles ) of the brain filled with liquor . It is also called water head or, outdated, cerebral dropsy .
| Classification according to ICD-10 | |
|---|---|
| Q03.- | Congenital hydrocephalus |
| Q05.0 | Cervical spina bifida with hydrocephalus |
| G91.0 | Communicating hydrocephalus |
| G91.1 | Hydrocephalus occlusus |
| G91.2 | Normal pressure hydrocephalus |
| G91.3 | Post-traumatic hydrocephalus |
| G91.9 | Hydrocephalus, unspecified |
| ICD-10 online (WHO version 2019) | |
Development of hydrocephalus
The cerebrospinal fluid ( liquor cerebrospinalis ) is generated in certain blood vessels (the choroid plexus ), especially in the two lateral ventricles , but also in the third and fourth ventricles. After flowing through the ventricles, the CSF enters the cerebellomedullary cistern and thus into the external CSF space . There it is distributed in the entire space surrounding the brain and spinal cord and is finally absorbed back into the blood through the arachnoid villi in the superior sagittal sinus and the spinal nerve exits. Alternative drainage routes exist in the area of the branches of the spinal nerves in the venous plexus or the lymphatic system. The constant total amount of liquor in adults is approx. 150 milliliters. Since the choroid plexus produce about half a liter of liquor per day, the liquor is exchanged three times within 24 hours. Absorption and production are normally in equilibrium. However, if too much CSF is produced, the connection between the individual CSF spaces is blocked or if too little fluid is absorbed, hydrocephalus can develop.
Drainage and resorption obstacles are usually the result of meningitis or a congenital or early childhood malformation of the brain. Prenatal brain infections in fetuses (e.g. lymphocytic choriomeningitis ) can also cause hydrocephalus. Bleeding into cerebrospinal fluid-carrying brain structures and tumors are also possible . Resorption resistance also arises from the increase in intracranial pressure itself (for example due to bleeding). In very rare cases, plexus papillomas can lead to overproduction of CSF.
Hydrocephalus ex vacuo is distinguished from these forms of hydrocephalus , the enlarged cerebrospinal fluid space of which is caused by the loss of brain tissue. There is no disturbance of the CSF circulation, only the total CSF 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; leads to severe brain damage with hydrocephalus and microcephaly )
- Dysraphic malformations ( myelomeningocele , encephalocele , syringomyelocele )
- Arnold-Chiari malformation (displacement of cerebellar parts and medulla oblongata through the foramen magnum into the spinal canal )
- Dandy Walker syndrome (developmental disorder characterized by cystic enlargement of the fourth 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 malformations (e.g. Klippel-Feil syndrome )
- Hereditary forms such as autosomal recessive hydrocephalus
Acquired causes
Acquired causes are for example:
- Toxoplasmosis (infection with Toxoplasma gondii)
- Meningitis and / or encephalitis (inflammation of the brain)
- Intracranial haemorrhage ( "posthemorrhagic hydrocephalus" ) (cerebral haemorrhage)
- Exogenous causes (e.g. operations)
- Brain tumors
- Trauma ( "post-traumatic hydrocephalus" ) (damage to the brain)
- CSF overproduction (e.g. in plexus papilloma )
- CSF resorption disorders
- Increased protein concentration in the liquor (increased viscosity of the liquor and impairment of the osmotic balance)
- Thrombosis in the head area
- Blood group intolerance / Rh intolerance
Symptoms
In fetuses and infants , the head can be balloon-like due to the still unstable skull bones . Side effects can be B. Headache , gushing vomiting , sunset phenomena , double vision and epilepsy . Further intracranial pressure and deficiency symptoms are possible.
Since the skull cannot compensate for the increase in intracranial volume by increasing its size after the bone sutures and fontanelles have been closed, there is an increase in pressure in the skull and parts of the brain shift towards the large occipital opening ( foramen magnum ). During this shift, vital parts of the brain can be pinched. Since these vital parts of the brain are indispensable for the functioning of the cardiovascular and respiratory systems, entrapment with damage to these parts of the brain leads to cardiovascular and respiratory arrest and thus to death.
Forms of hydrocephalus
-
Internal hydrocephalus: enlargement of the cerebral ventricles only
- Occlusion: when the flow of liquor from the ventricle is obstructed
- Malresorptivus: with delayed CSF reabsorption
- External hydrocephalus: enlargement of the external liquor spaces (i.e. the subarachnoid space )
- Hydrocephalus externus et internus (corresponds to hydrocephalus communicans - continuous connection from the ventricles to the subarachnoid space, disturbed liquor circulation due to adhesions of the meninges (inflammatory genesis or after a subarachnoid hemorrhage))
- Normal pressure hydrocephalus : enlarged cerebrospinal fluid spaces with only an intermittent increase in intracranial pressure. A symptom triad (dementia, gait disorder , incontinence)is typical for this form of hydrocephalus
- Hydrocephalus e (or: ex ) vacuo : internal and external hydrocephalus as an expression of primary brain tissue atrophy
In the latter form of hydrocephalus, in contrast to the other forms of hydrocephalus, there is no increase in intracranial pressure.
therapy
In the case of increased intracranial pressure, the earliest possible relief is sought through liquor drainage by means of a drainage . Here, the cerebrospinal fluid is passed through a shunt z. B. into the abdominal cavity , into the right atrium of the heart or less often into the pleural space. Depending on the location of the obstacle, the ventriculostomy is also a possible form of therapy, in which the floor of the third ventricle is surgically pierced, thus enabling the liquor to drain into the basal cisterns.
In some cases, therapy in utero is already possible, i.e. a prenatal intervention.
Trivia
Well-known personalities who suffered from hydrocephalus (e.g. as a result of meningitis) were Hermann von Helmholtz , Georges Cuvier and Adolph Menzel .
Web links
Individual evidence
- ^ A b A. Aschoff: In vitro testing of hydrocephalus valves. Habilitation thesis, Heidelberg 1994.
- ↑ H. Collmann, N. Sörensen, J. Krauss, J. Mühling: Hydrocephalus in craniosynostosis . In: Child's Nerv Syst. , October 1988. pp. 279-285.
- ^ Steven J. Schiff et al .: Rainfall drives hydrocephalus in East Africa. In: Journal of Neurosurgery: Pediatrics. Volume 10, No. 3, 2012, pp. 161-167, doi: 10.3171 / 2012.5.PEDS11557
- ↑ Ulf Lagerqvist: Pioneers of Microbiology and the Nobel Prize. World Scientific Publishing, New Jersey / London / Singapore / Hong Kong 2003, p. 21.
- ^ KM Laurence, Stephen Coates: The natural history of hydrocephalus. Detailed analysis of 182 unoperated case. In: Archives of Disease in Childhood. August 1, 1962, pp. 345-362, here: p. 360.
- ↑ Ruth Tesmar: The third eye. Imagination and insight . Inaugural lecture June 28, 1995 (Humboldt University Berlin. Philosophical Faculty III, Seminar for Artistic-Aesthetic Practice), p. 18.
- ↑ Karl Burger: Textbook of Obstetrics. Springer-Verlag, Berlin / Göttingen / Heidelberg 1950, p. 377.
- ↑ Ferdinand Sauerbruch : That was my life. Kindler & Schiermeyer, Bad Wörishofen 1951; Licensed edition for Bertelsmann Lesering, Gütersloh 1956, p. 82.
