Recent advances in the understanding and treatment of hydrocephalus

doi:10.1016/S1071-9091(97)80035-6

Seminars in Pediatric Neurology

Volume 4, Issue 3, September 1997, Pages 167-178

https://doi.org/10.1016/S1071-9091(97)80035-6 Get rights and content

Advances in the management of patients with hydrocephalus and other abnormalities of cerebrospinal fluid dynamics and intracranial pressure have come from a variety of sources including an improved understanding of the pathophysiology of the various subtypes of the problem, development of alternative methods of treating the condition without reliance on implantable shunting devices, use of neuroendoscopy, and the development of newer types of shunt valves. The purpose of this review is to put into perspective the relative importance of each of these advances to the overall management of our patients.

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A unifying hypothesis for hydrocephalus and the Chiari malformations part two: The hydrocephalus filling mechanism
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Citation Excerpt :
This allows more compliance so that remaining neurones have sufficient space with adequate volumes of CSF to allow for the required arterial supply over time. CSF production is down regulated when pressure is raised [14,35,69]. CSF production that exceeds absorption requires increase in ventricle or head size (or both), a shunt or an external drain.
It is proposed that negative central nervous system (CNS) pressure is one of the filling mechanisms of the fluid spaces of the CNS. Negative CNS pressure is caused by the combination of gravitational force and body movement. The venous system imposes pressure fluctuations on the CNS due to changes in posture and body cavity pressure. It is proposed here that filling of veins, arteries and cerebrospinal (CSF) spaces are all assisted by negative CNS pressure. Hyperemia in the CNS in response to pressure changes with movement was described in the first part of this hypothesis. By this means parenchyma water levels may increase (Williams, 2008). In the developmental forms of hydrocephalus expansion of CSF spaces is a more prominent feature than parenchyma water changes. This feature is explained by this second part of the hypothesis where the negative pulsatility of pressure that can accompany positive pressure pulsatility, which occurs with body movement, is described as the pathological force that leads to cavity filling. When CNS compliance is lost there is overrepresentation of low and well as high pressure pulsations in response to body movements. Pressure that leads to the development of hydrocephalus can be described as being abnormally labile. Negative CNS pressure causes cavity filling in an analogous way to pleural cavity filling, with water passing from parenchyma tissue. Positive pressure within the pressure profile may cause expansion of regions of the CNS skeletal system that are able to grow such as the cranial vault so that large head size is a frequent accompaniment to hydrocephalus that is caused by this mechanism. Hydrocephalic disorders that are characterised by negative pressure filling mechanism often have a skeletal anatomical abnormality that causes reduced CNS compliance and adversely affect neural development. This is often in the form of obstruction to CSF flow around the base of the brain that then leads to vault expansion by means of high pressure pulsation and ventricle enlargement by means of low pressure pulsation. In health pressure pulsatility does not lead to enduring changes in water distribution within the CNS compartments but it assists physiological water balance.
Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2
2014, Neuroscience
Citation Excerpt :
Congenital hydrocephalus is a birth defect occurring with a frequency of approximately 1–3 in 1000 newborn children that is caused by an accumulation of cerebrospinal fluid (CSF) in the cerebral ventricular system (Rekate, 1997; Del Bigio, 2001, 2004; Mataro et al., 2001; Perez-Figares et al., 2001; Chiafery, 2006; Zhang et al., 2006).
Hydrocephalus is caused by the accumulation of cerebrospinal fluid (CSF) in the cerebral ventricular system which results in an enlargement of the cranium due to increased intraventricular pressure. The increase in pressure within the brain typically results in sloughing of ciliated ependymal cells, loss of cortical gray matter, and increased gliosis. Congenital hydrocephalus is associated with several syndromes including primary ciliary dyskinesia (PCD), a rare, genetically heterogeneous, pediatric syndrome that results from defects in motile cilia and flagella. We have examined the morphological and physiological defects in the brains of two mouse models of PCD, nm1054 and bgh, which have mutations in Pcdp1 (also known as Cfap221) and Spef2, respectively. Histopathological and immunohistochemical analyses of mice with these mutations on the C57BL/6J and 129S6/SvEvTac genetic backgrounds demonstrate strain-dependent morphological brain damage. Alterations in astrocytosis, microglial activation, myelination, and the neuronal population were identified and are generally more severe on the C57BL/6J background. Analysis of ependymal ciliary clearance ex vivo and CSF flow in vivo demonstrate a physiological defect in nm1054 and bgh mice on both genetic backgrounds, indicating that abnormal cilia-driven flow is not the sole determinant of the severity of hydrocephalus in these models. These results suggest that genetic modifiers play an important role in susceptibility to severe PCD-associated hydrocephalus.
Mechanisms of mammalian ciliary motility: Insights from primary ciliary dyskinesia genetics
2011, Gene
Citation Excerpt :
In the absence of proper flow, excessive pressure from CSF accumulation in the lateral ventricles commonly results in sloughing of the ependymal cells, cell death in the underlying white matter, and loss of cortical neurons, which can cause thinning of the cerebral cortex (Del Bigio, 2004). Congenital hydrocephalus is debilitating and even fatal if untreated, and the only current method of treatment is surgical shunting (Clewell, 1988; Del Bigio, 2004; Rekate, 1997). Cilia on the embryonic node or organizer play a critical role in left–right patterning in the early embryo (Afzelius, 2004; Bisgrove and Yost, 2006; Ibanez-Tallon et al., 2003; Leigh et al., 2009; McGrath and Brueckner, 2003; Sutherland and Ware, 2009).
Motile cilia and flagella are organelles that, historically, have been poorly understood and inadequately investigated. However, cilia play critical roles in fluid clearance in the respiratory system and the brain, and flagella are required for sperm motility. Genetic studies involving human patients and mouse models of primary ciliary dyskinesia over the last decade have uncovered a number of important ciliary proteins and have begun to elucidate the mechanisms underlying ciliary motility. When combined with genetic, biochemical, and cell biological studies in Chlamydomonas reinhardtii, these mammalian genetic analyses begin to reveal the mechanisms by which ciliary motility is regulated.
Hydrocephalus in Dogs and Cats
2010, Veterinary Clinics of North America - Small Animal Practice
Citation Excerpt :
The major site of CSF absorption is at the arachnoid villi located in the venous sinuses and cerebral veins. Absorption of CSF is a passive process and not energy dependent.5 There is a pressure differential of 7 to 10 cm H2O across the arachnoid villi, which accounts for the normal intracranial pressure of 7 to 10 cm H2O.
Fluid and ion transfer across the blood-brain and blood-cerebrospinal fluid barriers; a comparative account of mechanisms and roles
2016, Fluids and Barriers of the CNS
MR elastography can be used to measure brain stiffness changes as a result of altered cranial venous drainage during jugular compression
2015, American Journal of Neuroradiology

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Recent advances in the understanding and treatment of hydrocephalus

Surg Neurol

Über veränderungen des Kleinhirns, des Pons und der Medulla oblongata in Folge von congenitales Hydrocephalie des Grosshirns

Denkschrift Akademic Wissenshaft, Wein

Über veränderungen des Kleinhirns in Folge von Hydrocephalie des Grosshirns

Deutsche Medicinische Wochenschrift

Internal Hydrocephalus. An experimental, clinical and pathological study

Am J Dis Child

Circulation of the cerebraospinal fluid: Demonstration of the choroid plexuses as the generator of the force for flow of fluid and ventricular enlargement

J Neurosurg

Effects of Prolonged cerebrospinal fluid shunting on the skull and brain

J Neurosurg

Cerebrospinal fluid production by the choroid plexus and brain

Science

Extirpation of the choroid plexus of the lateral ventricles in communicating hydrocephalus

Ann Surg

Treatment of hydrocephalus by direct shunt from ventricle to jugular vein

Surg Forum

Spontaneously arrested hydrocephalus: Results of the re-examination of 82 survivors from a series of 182 unoperated cases

Dev Med Child Neurol

Secretion of cerebrospinal fluid by the choroid plexus of the rabbit

Am J Physiol

Non-communicating syringomyelia following occlusion of the central canal in rats: Experimental model and histologic findings

J Neurosurg

Cerebrospinal fluid drainage in dead Rabbits

Formation and absorption of cerebrospinal fluid in man

Brain

Relationship between intracranial and sagittal sinus pressure in normal and hydrocephalic dogs

Pediatr Neurosc

Cerebrospinal fluid drainage as influenced by ventricular pressure in the rabbit

J Neurosurg

Recent research into the nature of CSF formation and absorption

J Neurosurg

Resistance elements within the cerebreospinal fluid circulation

Circuit diagram of the circulation of cerebrospinal fluid

Concepts in Pediatric Neurosurgery

Reexpansion of previously collapsed ventricles: The slit ventricle syndrome

J Neurosurg

Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

J Neurosurg

Low spinal fluid pressure syndromes

Neurology

Spontaneous Intracranial Hypotension: Headache with a reversible Arnold-Chiari malformation

Headache

The application of mathematical modeling to hydrocephalus research

Concepts in Pediatric Neurosurgery

The usefulness of mathematical modeling in hydrocephalus research

Childs Nerv Syst

Brain turgor (Kb): Intrinsic property of the brain to resist distortion

Pediatr Neurosurg

Low-pressure hydrocephalic stat and viscoelastic changes is the brain

Neurosurgery

Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies

Neurology

Preliminary experience with controlled external lumbar drainage in diffuse pediatric head injury

Pediatr Neurosurg

Controlled lumbar drainage in pediatric head injury

J Neurosurg

Neuropathological changes caused by hydrocephalus

Acta Neuropathol

Progression of experimental infantile hydrocephalus and effects of ventriculoperitoneal shunts: an analysis correlating magnetic resonance imaging with gross morphology

Neurosurgery