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HydrocephalusAka: Chronic Hydrocephalus

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  1. See Also
    1. Normal Pressure Hydrocephalus
  2. Definitions
    1. Acute Hydrocephalus
      1. Neurologic Emergency
      2. Complete Obstructive Hydrocephalus
    2. Chronic Hydrocephalus
      1. Described below
      2. Results in Dementia
  3. Predisposing factors
    1. Meningitis
    2. Intraventricular hemorrhage
  4. Pathophysiology
    1. Children
      1. Ventricular obstruction (noncommunicating)
        1. Aqueductal stenosis
        2. Incomplete Magendie or Luschka foramina development
      2. Associated with other neurologic abnormalities
        1. Microgyria or Macrogyria
        2. Porencephaly
        3. Agenesis of corpus callosum or cerebellar vermis
        4. Fusion of cerebral hemispheres
        5. Spina bifida, Meningocele or Encephalocele
        6. Syringomyelia or Hydromyelia
        7. Arnold-Chiari Malformation
    2. Adults
      1. Extraventricular obstruction (communicating)
        1. Occurs from subarachnoid space blockage
      2. Rarely Noncommunicating from aqueductal stenosis
  5. Causes
    1. Nonobstructive (ex vacuo)
      1. Alzheimer's Disease
      2. Pick's Disease
      3. Multiple Cerebral Infarctions
      4. Huntington's Disease
    2. Obstructive (Incomplete except in Acute Hydrocephalus)
      1. Adult: Communicating (Extraventricular Blockage)
        1. Post-Subarachnoid Hemorrhage
        2. Post-Meningitis
        3. Idiopathic Normal Pressure Hydrocephalus
      2. Child: Noncommunicating (Interventricular Blockage)
        1. Aqueductal stenosis
        2. Masses compressing the fourth ventricle
          1. Cerebellar tumor
        3. Foramen magnum malformation
          1. Arnold-Chiari Malformation
          2. Dandy-Walker Syndrome
  6. Symptoms
    1. Headache
    2. Vomiting
    3. Limb weakness
    4. Incoordination
  7. Signs
    1. Child
      1. Rapid increase in Head Circumference
      2. Bulging Anterior Fontanelle
    2. Adult
      1. Classic Triad of Normal Pressure Hydrocephalus
        1. Dementia of subcortical type
        2. Gait disturbance
        3. Incontinence
      2. Abulia
      3. Papilledema
      4. Eyes displaced downward
  8. Imaging
    1. Cranial Ultrasound (Infants)
      1. Requires open Anterior Fontanelle
      2. Shows ventricular enlargement
    2. CT Head
    3. MRI Head (preferred)
  9. Diagnosis
    1. CSF Flow study
      1. Radioiodinated Serum Albumin or radioactive indium
      2. Identifies obstruction site
  10. Management: Surgical Shunt (Ventriculoperitoneal shunt)
    1. Needs to be done before irreversible neurologic loss
    2. Predictors of shunt efficacy
      1. Symptoms for less than 6 months
      2. Lumbar Puncture efficacious
        1. Lowers CSF Pressure <100 mm
        2. Improves gait
    3. Complications of Ventriculoperitoneal shunt
      1. Infection
      2. Mechanical malfunction
      3. Cannula dislodged with inadequate drainage
      4. Ascites
      5. Peritonitis
      6. Ruptured Viscus
      7. Subdural Hematoma
  11. Management: Non-surgical
    1. Indicated when surgery not possible
    2. Acetazolamide (Diamox): Decreases CSF production
      1. Child: 10-25 mg/kg/day PO divided tid
      2. Adult: 250 mg PO tid
    3. Serial Lumbar Puncture (Temporize until surgery)
  12. Resources
    1. All About Normal Pressure Hydrocephalus
      1. http://www.allaboutnph.com

Hydrocephalus (C0020255)

Definition (MSH)Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, INTRACRANIAL HYPERTENSION; HEADACHE; lethargy; URINARY INCONTINENCE; and ATAXIA (and in infants macrocephaly). This condition may be caused by obstruction of cerebrospinal fluid pathways due to neurologic abnormalities, INTRACRANIAL HEMORRHAGES; CENTRAL NERVOUS SYSTEM INFECTIONS; BRAIN NEOPLASMS; CRANIOCEREBRAL TRAUMA; and other conditions. Impaired resorption of cerebrospinal fluid from the arachnoid villi results in a communicating form of hydrocephalus. Hydrocephalus ex-vacuo refers to ventricular dilation that occurs as a result of brain substance loss from CEREBRAL INFARCTION and other conditions.
Definition (CSP)excessive accumulation of cerebrospinal fluid within the cranium which may be a congenital or acquired disorder; hydrocephalus ex-vacuo refers to ventricular dilation that occurs as a result of brain substance loss from cerebral infarction and other conditions.
Definition (NCI)(hye-dro-SEF-uh-lus) The abnormal buildup of cerebrospinal fluid in the ventricles of the brain.
ConceptsDisease or Syndrome (T047)
MSHD006849
EnglishHydrocephalus, Hydrocephaly
Spanish1 hidrocefalia, hidrocefalia
Parent ConceptsCNS disorder (C0007682), Degenerative brain disorder (C0154671), Congenital disease of brain (C0596364), Congenital Abnormality (C0000768), Head (C0018670), Brain Diseases (C0006111), Neonatal disorder (C0021290), Intracranial Hypertension (C0151740), Disorders of cerebrospinal fluid and intracranial pressure (C0393974), Duplicate concept (C1274013)
SourcesAOD, COSTAR, CSP, CST, DXP, LCH, MEDLINEPLUS, MSH, MTH, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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