- Abilash Haridas, MD
Abilash Haridas, MD
- Pediatric & Cerebrovascular Neurosurgery, Children’s Hospital of Michigan
- Assistant Professor of Neurosurgery, Wayne State University
- Tadanori Tomita, MD
Tadanori Tomita, MD
- Yeager Professor and Division Head of Pediatric Neurosurgery,
- Director, Falk Brain Tumor Center
- Ann & Robert H. Lurie Children’s Hospital of Chicago;
- Professor and Vice-Chair, Department of Neurosurgery,
- Northwestern University Feinberg School of Medicine
- Section Editors
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
- Leonard E Weisman, MD
Leonard E Weisman, MD
- Section Editor — Neonatology
- Professor of Pediatrics
- Baylor College of Medicine
Hydrocephalus is a disorder in which an excessive amount of cerebrospinal fluid (CSF) accumulates within the cerebral ventricles and/or subarachnoid spaces, which are dilated [1,2].
In children, hydrocephalus is almost always associated with increased intracranial pressure (ICP). In most cases, this is caused by excess CSF accumulating in the cerebral ventricles due to disturbances of CSF circulation (known as obstructive or non-communicating hydrocephalus). Less often, the CSF accumulates because of impaired absorption (known as communicating hydrocephalus). These types of hydrocephalus will be the focus of this topic review.
By contrast, in normal pressure hydrocephalus, the cerebral ventricles are pathologically enlarged, but the ICP is within the normal range. This condition is usually caused by impaired CSF absorption. This type of hydrocephalus is more often seen in adults and is discussed separately. (See "Normal pressure hydrocephalus".)
These forms of hydrocephalus are distinct from two radiographic findings that include the same word. The term "hydrocephalus ex-vacuo" refers to dilatation of the ventricles secondary to brain atrophy or loss of brain tissue secondary to an insult; hydrocephalus ex-vacuo is not accompanied by increased ICP. The term "external hydrocephalus" or "benign enlargement of the extra-axial spaces" refers to excessive fluid, usually CSF, in the subarachnoid spaces and is associated with familial macrocephaly [3,4].
The prevalence of congenital and infantile hydrocephalus in the United States and Europe has been estimated as 0.5 to 0.8 per 1000 live and still births [5-7]. Approximately 15 to 25 percent of these cases are associated with myelomeningocele (spina bifida) [6,8].
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- Kulkarni AV, Donnelly R, Shams I. Comparison of Hydrocephalus Outcome Questionnaire scores to neuropsychological test performance in school-aged children. J Neurosurg Pediatr 2011; 8:396.
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- Adams-Chapman I, Hansen NI, Stoll BJ, et al. Neurodevelopmental outcome of extremely low birth weight infants with posthemorrhagic hydrocephalus requiring shunt insertion. Pediatrics 2008; 121:e1167.
- CSF production
- CSF circulation
- CSF absorption
- Impaired absorption
- Excessive production
- - Neural tube defects
- - Isolated hydrocephalus
- - X-linked hydrocephalus
- - CNS malformations
- - Syndromic forms
- - Intrauterine infection
- - Choroid plexus papilloma or carcinoma
- Acquired hydrocephalus
- - Infections and tumors
- - Posthemorrhagic hydrocephalus
- Low pressure hydrocephalus
- CLINICAL FEATURES
- Physical examination
- - Obstructive versus communicating hydrocephalus
- - Hydrocephalus versus atrophy
- - Antenatal MRI
- Lumbar puncture
- - Complications
- Third ventriculostomy
- Medical therapy
- - Diuretics and fibrinolytics
- - Serial lumbar punctures
- Functional outcome
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS