Hydrocephalus classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Syed Ahsan Hussain, M.D.[2]

Hydrocephalus is classified based upon the underlying cause such as impaired cerebrospinal fluid (CSF) flow, impaired CSF reabsorption, or excessive CSF production.

• Based on its underlying mechanisms, hydrocephalus can be classified into:^[1][2]
  • Communicating (non-obstructive).
  • Non-communicating (obstructive).

• Both communicating and non-communicating forms can be either:
  • Congenital.
  • Acquired.

• The mechanism of hydrocephalus is given below:^[1][3]
  • It is caused by impaired cerebrospinal fluid resorption in the absence of any CSF-flow obstruction.
  • It has been theorized that this is due to functional impairment of the arachnoid granulations.
  • Arachnoid granulations are located along the superior sagittal sinus and is the site of cerebrospinal fluid resorption back into the venous system.
  • Various neurologic conditions may result in communicating hydrocephalus, including:
  • Subarachnoid/intraventricular hemorrhage.
  • Meningitis.
  • Chiari malformation.
  • Congenital absence of arachnoidal granulations (Pacchioni’s granulations).

Sub-types of communicating hydrocephalus:

(a) Normal pressure hydrocephalus (NPH):

• NPH is a particular form of communicating hydrocephalus, characterized by enlarged cerebral ventricles, with only intermittently elevated cerebrospinal fluid pressure.
• The diagnosis of NPH can be established only with the help of continuous intraventricular pressure recordings (over 24 hours).

• It refers to an enlargement of cerebral ventricle
• Hydrocephalus ex vacuo occurs as a result of compensatory enlargement of the CSF-spaces in response to brain parenchyma loss.

• Non-communicating hydrocephalus, or obstructive hydrocephalus, is caused by a CSF-flow obstruction (either due to external compression or intraventricular mass lesions) which leads to:^[1][4]
  • Foramen of Monro obstruction may lead to dilation of one or, if large enough (e.g., in colloid cyst), both lateral ventricles.
  • The aqueduct of Sylvius, normally narrow to begin with, may be obstructed by a number of genetically or acquired lesions (e.g., atresia, ependymitis, hemorrhage, tumor) and lead to dilatation of both lateral ventricles as well as the third ventricle.
  • Fourth ventricle obstruction will lead to dilatation of the aqueduct as well as the lateral and third ventricles.
  • The foramina of Luschka and foramen of Magendie may be obstructed due to congenital failure of opening (e.g., Dandy-Walker malformation).
  • The subarachnoid space surrounding the brainstem may also be obstructed due to inflammatory or hemorrhagic fibrosing meningitis, leading to widespread dilatation, including the fourth ventricle.

• The cranial bones fuse by the end of the third year of life. For head enlargement to occur, hydrocephalus must occur before then. The causes are usually genetic but can also be acquired and usually occur within the first few months of life, which include:^[1][5]
  • Intraventricular matrix hemorrhages in premature infants
  • Infections
  • Type II Arnold-Chiari malformation
  • Aqueduct atresia and stenosis
  • Dandy-Walker malformation.
  • About 80-90% of fetuses or newborn infants with spina bifida which are often associated with meningocele or myelomeningocele, develops hydrocephalus.

The acquired condition is the result of^[5][1]:

• Infections.
• Meningitis.
• Brain tumors.
• Head trauma.
• Intracranial hemorrhage (subarachnoid or intraparenchymal) and is usually extremely painful for the patient.

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