Management of Hydrocephalus

Definition, History & Frequency

Hydrocephalus Definition, History & Frequency

Hydrocephalus: Greek word means: hydor = water & kephale = head. (Lisowski &
Oxnard 2007)

Thus, hydrocephalus is loosely translated as water in the head. (Frim and Gupta, 2006) Definition of hydrocephalus: Hydrocephalus is an abnormal enlargement of the ventricles due to an excessive accumulation of CSF resulting from a disturbance of its flow, absorption or, secretion. (Kaye, 2005) Hydrocephalus is a condition where the ventricles of the brain or the subarachnoid spaces (in external hydrocephalus) accumulate an excessive amount of CSF. In the neonate this is most frequently a result of occlusion of the cerebral aqueduct that prevents the flow of CSF from the 3rd into the 4th ventricle. In very extensive cases, the continued formation of CSF leads to an increase in fluid volume which causes enlargement of the head, reduction in the thickness of the bones of the cranium, and compression of the walls of the ventricles. Such compression may cause lamination of the cerebral cortex resulting in severe mental retardation. (Patestas & Gartner, 2006) Hydrocephalus and ventriculomegaly: All are terms used to describe dilatation of the lateral ventricles. However, they should be distinguished: 1. Hydrocephalus signifies dilated lateral ventricles resulting from an increased amount of CSF inside the ventricles and increased intracranial pressure.
(Vasiljevic, etal, 2012) 2. Ventriculomegaly is a dilatation of the lateral ventricles without increased intracranial pressure (Vasiljevic, etal, 2012). It may occur in the situation of

decreased brain volume from atrophy or brain injury (also known as hydrocephalus ex vacuo), or may be a normal variation in some individuals.
(Frim and Gupta, 2006)

Normal-pressure hydrocephalus (NPH): Normal-pressure hydrocephalus was first described as a clinical entity by Hakim and Adams in 1965. They described a group of patients with symptoms of dementia, ataxia and incontinence, where the radiological studies showed hydrocephalus but the lumbar CSF pressure was normal. (Kaye, 2005) History:

Management of Hydrocephalus

Definition, History & Frequency

hydrocephalic skulls can be found in ancient Egyptian medical literature from 2500 BC to 500 AD (Aschoff et al., 1999). The knowledge about CSF originates since the time of Hippocrates (466-377 BC), Galen (in the 2nd century AD), Herophilus (300 BC) and Arabian physicians (Athanasakis & Ermidou, 2011), Andreas Vesalius (1514 1564) presented the concept of hydrocephalus as accumulation of water inside the ventricles (Trojanowski, 2009). Also, references to hydrocephalic skulls can be found in ancient Egyptian medical literature from 2500 BC to 500 AD (Athanasakis & Ermidou, 2011). In 1701 Pacchioni discovered arachnoid granulations but wrongly associated them with secretion of CSF. In the beginning of 20th century Dandy W. and Blackfan K. studied hydrocephalus in an animal model and showed that removal of the choroid plexus, before occlusion of aqueduct or of the foramina, prevented the development of hydrocephalus (Trojanowski, 2009). The evolution of surgery of hydrocephalus divided into three stages. The first period was related to the antiquity, the second period is to the 19th century. The last period started at 1950 and is linked with the development of silicone catheters with a valve. (Athanasakis & Ermidou, 2011) Frequency: The incidence of infantile hydrocephalus is approximately 3-4 per 1000 births and most cases are due to congenital abnormalities. The incidence of hydrocephalus occurring as a single congenital disorder is 1-1.5 per 1000 births. Hydrocephalus occurring with spina bifida and myelomeningocele varies from 1.5 to 2.9 per 1000 births, but with prenatal screening and folate supplementation the incidence of spina bifida is decreasing (Kaye, 2005). Couples with a previously affected child have a recurrence risk of 4%. ( Vasiljevic, etal, 2012) The frequency of hydrocephalus among CNS congenital anomalies Congenital CNS anomalies Ventriculomegaly Closed spinal dysraphisms Hydrocephalus Myelomeningoceles Holoprosencephaly Microcephaly Agenesis of the corpus callosum DWM Arachnoid cysts 2 The frequency 1.3 :1000 0.9 :1000 0.5 :1000 0.4 :1000 0.1 :1000 0.1 :1000 0.07 : 1000 0.07 :1000 0.03 :1000

Management of Hydrocephalus Encephaloceles Spinal lipoma Mega cistern magna Schizencephaly Vein of Galen aneurysm

Definition, History & Frequency 0.03 :1000 0.03 :1000 0.03 :1000 0.03 :1000 0.03 :1000
(Adapted from Alastair, 2012)