Hydrocephalus

Changed by Benjamin Layton, 29 Mar 2018

Updates to Article Attributes

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Definition

Hydrocephalus merely denotes is an increase in the volume of CSF and thusactive distension of the cerebral ventricles (ventriculomegaly (ventriculomegaly) of the brain resulting from inadequate passage of cerebrospinal fluid (CSF) from its point of production within the cerebral ventricles to its point of absorption into the systemic circulation: it is caused by a mismatch between CSF production and absorption.5

Although raised intracranial pressure often co-exists with hydrocephalus it doesn’t have to be present.

Hydrocephalus is typically referred to as either being "obstructive"either obstructive (non-communicating) or "communicating",non-obstructive (communicating):

Obstructive refers to a barrier to flow between two or more CSF spaces (eg aqueductal stenosis blocking the cerebral aqueduct of sylvius causing dilatation of the 3rd and lateral ventricles).

Non-obstructive is broken down into over production of CSF (a CSF producing coroid plexus tumour) and under-absorption of CSF (for example sub-arachnoid haemorrhage blocking the absorption pathway).

There has been a recent effort to change the classification system into one which classes under-absorption of CSF (technically an obstruction) as an obstructive hydrocephalus and therefore which does not consider communicating and non-obstructive as the same (likewise with non-communicating and obstructive). Thus:

1.    Non-obstructive hydrocephalus:

  • Choroid plexus papilloma
  • Normal pressure hydrocephalus (NPH)

2.     Obstructive hydrocephalus:

  • All other causes

The intention of this can leadclassification is to confusion as toclear up the underlying cause of ventriculomegaly as the terms are referring to different aspects of the underlying pathophysiology (namely "why" and "where").

For example, acute subarachnoid haemorrhage confined confined to the basal cisterns can result in ventriculomegaly by obstructing the normal flow of CSF through the basal cisterns, and by filling the arachnoid granulations. Given that this is mechanistically an obstruction to CSF flow outside of the ventricular system should it be considered communicating or obstructive hydrocephalus? The technically correct answer is that is actually a communicating obstructive hydrocephalus.

As such a more precise terminology is to divide hydrocephalus into:

  1. communicating

    1.     Communicating and non-communicating: addressingAddressing "where" the obstruction is located

  2. obstructive

    2.     Obstructive and non-obstructive: onOn the grounds of whether or not there is obstruction of CSF pathways in the ventricles or the subarachnoid space 1-3

This nomenclature leads to the following types of hydrocephalus (see figures 1 & 2):

  • communicating (i.e. CSF
    Communicating (CSF can exit the ventricular system):
    • withWith obstruction to CSF absorption
      • usuallyUsually referred to merely as communicating hydrocephalus
      • passagePassage of CSF from the ventricular system into the subarachnoid space is unimpeded but at some point between the basal cisterns and the arachnoid granulations, normal flow is impeded
      • Ssubarachnoidubarachnoid haemorrhage (obstruction can be acute when filling the basal cisterns with blood clot, or chronic due to scarring of the subarachnoid space and arachnoid granulations)
      • infectiveInfective meningitis (both during infection and chronic) 
      • TB meningitis is is typically basal filling the basal cisterns
      • bacterialBacterial meningitis is typically also over the convexities
    • Lleptomeningealeptomeningeal carcinomatosis
  • withoutWithout obstruction to CSF absorption
  • additionally

    Additionally

    , other conditions with large ventricles fall into this group although they are often not thought ofclassified as hydrocephalus. These include
  • non
  • Non
    -communicating (i.e. CSF(CSF cannot exit the ventricular system, and thus there is by definition obstruction to CSF absorption):
    Regarding CSF Absorption

    It is important to note that recent research suggests that the arachnoid villi are not physiologically implicated in CSF absorption. The exact mechanism of absorption is unclear but is intracranial lymphatics are implicated.6

    • -<p><strong>Hydrocephalus</strong> merely denotes an increase in the volume of CSF and thus of the <a href="/articles/ventricular-system">cerebral ventricles</a> (<a href="/articles/ventriculomegaly">ventriculomegaly</a>).</p><p>Although hydrocephalus is typically referred to as either being "obstructive" or "communicating", this can lead to confusion as to the underlying cause of ventriculomegaly as the terms are referring to different aspects of the underlying pathophysiology (namely "why" and "where").</p><p>For example, acute <a href="/articles/subarachnoid-haemorrhage">subarachnoid haemorrhage</a> confined to the basal cisterns can result in ventriculomegaly by obstructing the normal flow of CSF through the basal cisterns, and by filling the arachnoid granulations. Given that this is mechanistically an obstruction to CSF flow outside of the ventricular system should it be considered communicating or obstructive hydrocephalus? The correct answer is that is actually a communicating obstructive hydrocephalus.</p><p>As such a more precise terminology is to divide hydrocephalus into:</p><ol>
    • -<li>communicating and non-communicating: addressing "where" the obstruction is located</li>
    • -<li>obstructive and non-obstructive: on the grounds of whether or not there is obstruction of CSF pathways in the ventricles or the subarachnoid space <sup>1-3</sup>
    • -</li>
    • -</ol><p>This nomenclature leads to the following types of hydrocephalus (see figures 1 &amp; 2):</p><ul>
    • -<li>communicating (i.e. CSF can exit the ventricular system)<ul>
    • -<li>with obstruction to CSF absorption<ul>
    • -<li>usually referred to merely as <a href="/articles/communicating-hydrocephalus">communicating hydrocephalus</a>
    • -</li>
    • -<li>passage of CSF from the ventricular system into the subarachnoid space is unimpeded but at some point between the basal cisterns and the arachnoid granulations, normal flow is impeded<ul>
    • -<li>
    • -<a href="/articles/subarachnoid-haemorrhage">subarachnoid haemorrhage</a> (obstruction can be acute when filling the basal cisterns with blood clot, or chronic due to scarring of the subarachnoid space and arachnoid granulations)</li>
    • -<li>infective <a href="/articles/meningitis">meningitis</a> (both during infection and chronic) <ul>
    • +<h5>Definition</h5><p>Hydrocephalus is an active distension of the cerebral ventricles (ventriculomegaly) of the brain resulting from inadequate passage of cerebrospinal fluid (CSF) from its point of production within the cerebral ventricles to its point of absorption into the systemic circulation: it is caused by a mismatch between CSF production and absorption.<sup>5</sup></p><p>Although raised intracranial pressure often co-exists with hydrocephalus it doesn’t have to be present.</p><p>Hydrocephalus is typically referred to as either being either obstructive (non-communicating) or non-obstructive (communicating):</p><p><strong>Obstructive </strong>refers to a barrier to flow between two or more CSF spaces (eg aqueductal stenosis blocking the cerebral aqueduct of sylvius causing dilatation of the 3<sup>rd</sup> and lateral ventricles).</p><p><strong>Non-obstructive</strong> is broken down into over production of CSF (a CSF producing coroid plexus tumour) and under-absorption of CSF (for example sub-arachnoid haemorrhage blocking the absorption pathway).</p><p>There has been a recent effort to change the classification system into one which classes under-absorption of CSF (technically an obstruction) as an obstructive hydrocephalus and therefore which does not consider communicating and non-obstructive as the same (likewise with non-communicating and obstructive). Thus:</p><p><!--[if !supportLists]-->1.    <strong> <!--[endif]-->Non-obstructive hydrocephalus:</strong></p><ul>
    • +<li>Choroid plexus papilloma</li>
    • +<li>Normal pressure hydrocephalus (NPH)</li>
    • +</ul><p><!--[if !supportLists]-->2.     <!--[endif]--><strong>Obstructive hydrocephalus:</strong></p><ul><li>All other causes</li></ul><p>The intention of this classification is to clear up the underlying cause of ventriculomegaly as the terms are referring to different aspects of the underlying pathophysiology (namely "why" and "where").</p><p>For example, acute <a href="/articles/subarachnoid-haemorrhage">subarachnoid haemorrhage</a> confined to the basal cisterns can result in ventriculomegaly by obstructing the normal flow of CSF through the basal cisterns. Given that this is mechanistically an obstruction to CSF flow outside of the ventricular system should it be considered communicating or obstructive hydrocephalus? The technically correct answer is that is actually a communicating obstructive hydrocephalus.</p><p>As such a more precise terminology is to divide hydrocephalus into:</p><p><!--[if !supportLists]-->1.     <!--[endif]--><strong>Communicating and non-communicating: </strong>Addressing "where" the obstruction is located</p><p><!--[if !supportLists]-->2.     <!--[endif]--><strong>Obstructive and non-obstructive: </strong>On the grounds of whether or not there is obstruction of CSF pathways in the ventricles or the subarachnoid space <sup>1-3</sup></p><p>This nomenclature leads to the following types of hydrocephalus (see figures 1 &amp; 2):</p><h5>Communicating (CSF can exit the ventricular system):</h5><ul>
    • +<li>With obstruction to CSF absorption<ul>
    • +<li>Usually referred to merely as <a href="/articles/communicating-hydrocephalus">communicating hydrocephalus</a>
    • +</li>
    • +<li>Passage of CSF from the ventricular system into the subarachnoid space is unimpeded but at some point between the basal cisterns and the arachnoid granulations, normal flow is impeded</li>
    • +<li>S<a href="/articles/subarachnoid-haemorrhage">ubarachnoid haemorrhage</a> (obstruction can be acute when filling the basal cisterns with blood clot, or chronic due to scarring of the subarachnoid space and arachnoid granulations)</li>
    • +<li>Infective <a href="/articles/meningitis">meningitis</a> (both during infection and chronic) </li>
    • -<a href="/articles/tuberculous-meningitis">TB meningitis</a> is typically basal filling the basal cisterns</li>
    • -<li>bacterial meningitis is typically also over the convexities</li>
    • -</ul>
    • +<a href="/articles/tuberculous-meningitis">TB meningitis</a> is typically basal filling the basal cisterns</li>
    • +<li>Bacterial meningitis is typically also over the convexities</li>
    • +<li>L<a href="/articles/leptomeningeal-metastases">eptomeningeal carcinomatosis</a>
    • -<li><a href="/articles/leptomeningeal-metastases">leptomeningeal carcinomatosis</a></li>
    • -</ul>
    • +<li>Without obstruction to CSF absorption<ul>
    • +<li>A particular group of conditions with disparate, and often poorly understood, abnormal CSF dynamics, including:</li>
    • +<li>N<a href="/articles/normal-pressure-hydrocephalus">ormal pressure hydrocephalus (NPH)</a>
    • -<li>without obstruction to CSF absorption<ul>
    • -<li>a particular group of conditions with disparate, and often poorly understood, abnormal CSF dynamics, including:<ul>
    • -<li><a href="/articles/normal-pressure-hydrocephalus">normal pressure hydrocephalus (NPH)</a></li>
    • -<li>
    • -<a href="/articles/choroid-plexus-papilloma-1">choroid plexus papillomas</a> (part of the associated hydrocephalus is thought to be due to overproduction of CSF <sup>1</sup>. An obstructive component in larger masses is often also present)</li>
    • +<li>C<a href="/articles/choroid-plexus-papilloma-1">horoid plexus papillomas</a> (part of the associated hydrocephalus is thought to be due to overproduction of CSF <sup>1</sup>. An obstructive component in larger masses is often also present)</li>
    • -<li>additionally, other conditions with large ventricles fall into this group although they are often not thought of as hydrocephalus. These include<ul><li>
    • -<a href="/articles/hydrocephalus-ex-vacuo">hydrocephalus ex-vacuo</a> and <a href="/articles/colpocephaly">colpocephaly</a> (ventricles are enlarged due to loss of adjacent brain parenchyma)</li></ul>
    • -</li>
    • -</ul>
    • +</ul><p> </p><p>Additionally, other conditions with large ventricles fall into this group although they are not classified as hydrocephalus. These include <!--[endif]--><a href="/articles/hydrocephalus-ex-vacuo">hydrocephalus ex-vacuo</a> and <a href="/articles/colpocephaly">colpocephaly</a> (ventricles are enlarged due to loss of adjacent brain parenchyma)</p><h5>Non-communicating (CSF cannot exit the ventricular system):</h5><ul>
    • +<li>Up-stream ventricles are dilated and exert mass effect upon adjacent brain (e.g. effacement of sulci)</li>
    • +<li>Numerous causes including<ul>
    • +<li>F<a href="/articles/foramen-of-monro">oramen of Monro</a>: <a href="/articles/colloid-cyst-of-the-third-ventricle">colloid cyst</a>
    • -</ul>
    • -</li>
    • -<li>non-communicating (i.e. CSF cannot exit the ventricular system, and thus there is by definition obstruction to CSF absorption)<ul>
    • -<li>often merely referred to as <a href="/articles/obstructive-hydrocephalus">obstructive hydrocephalus</a>
    • -</li>
    • -<li>up-stream ventricles are dilated and exert mass effect upon adjacent brain (e.g. effacement of sulci)</li>
    • -<li>numerous causes including<ul>
    • -<li>
    • -<a href="/articles/foramen-of-monro">foramen of Monro</a>: <a href="/articles/colloid-cyst-of-the-third-ventricle">colloid cyst</a>
    • -</li>
    • -<li>
    • -<a href="/articles/cerebral-aqueduct-of-sylvius">aqueduct of Sylvius</a>: <a href="/articles/aqueduct-stenosis">aqueduct stenosis</a>, <a href="/articles/tectal-glioma">tectal glioma</a>  </li>
    • -<li>fourth ventricle: <a href="/articles/posterior-fossa-tumours">posterior fossa tumour</a>, or <a href="/articles/cerebellar-infarction">cerebellar infarct</a> or <a href="/articles/cerebellar-haemorrhage">cerebellar haemorrhage</a>
    • -</li>
    • -</ul>
    • +<li>A<a href="/articles/cerebral-aqueduct-of-sylvius">queduct of Sylvius</a>: <a href="/articles/aqueduct-stenosis">aqueduct stenosis</a>, <a href="/articles/tectal-glioma">tectal glioma</a>  </li>
    • +<li>Fourth ventricle: <a href="/articles/posterior-fossa-tumours">posterior fossa tumour</a>, or <a href="/articles/cerebellar-infarction">cerebellar infarct</a> or <a href="/articles/cerebellar-haemorrhage">cerebellar haemorrhage</a>
    • -</ul>
    • +</ul><h5>Regarding CSF Absorption</h5><p>It is important to note that recent research suggests that the arachnoid villi are not physiologically implicated in CSF absorption. The exact mechanism of absorption is unclear but is intracranial lymphatics are implicated.<sup>6</sup></p>

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