What is hydrocephalus?

The term hydrocephalus is derived from the Greek words “hydro” meaning water and “cephalus” meaning head. Hydrocephalus is an abnormal accumulation of cerebrospinal fluid (CSF) within cavities in the brain called ventricles. CSF circulates through the ventricular system in the brain and is absorbed into the bloodstream. This fluid is in constant circulation and has many functions, including to surround the brain and spinal cord and act as a protective cushion against injury.

What are the different types of hydrocephalus?

Hydrocephalus is a condition, not a disease. It can develop for a variety of reasons, sometimes as part of another condition.

  • Congenital hydrocephalus means the condition is present at birth, caused by a complex interaction of genetic and environmental factors during fetal development. Congenital hydrocephalus is now often diagnosed before birth through routine ultrasound.
  • Compensated hydrocephalusis hydrocephalus that is diagnosed in adulthood, but may have existed since birth. It can still be considered congenital.
  • Acquired hydrocephalus develops after birth as a result of neurological conditions such as head trauma, brain tumor, cyst, intraventricular hemorrhage or infection of the central nervous system.
  • Normal pressure hydrocephalus occurs in older adults when the ventricles of the brain are enlarged, but there is little or no increase in the pressure within the ventricles. Sometimes the cause of NPH is known – but most often it is idiopathic, which means the cause is not known.

What causes hydrocephalus?

The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) or developmental disorders (such as those associated with neural tube defects including spina bifida and encephalocele).

Other possible causes include complications of premature birth such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage, which block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF within the cisterns

Who develops hydrocephalus?

Hydrocephalus affects a wide range of people, from infants and older children to young, middle-aged and older adults.

What are the symptoms?

Infants and Children

  • Abnormal head enlargement
  • Tense, bulging fontanel
  • Prominent scalp veins
  • Skull bones may feel separated
  • Vomiting, sleepiness, irritability
  • Headache, nausea, vomiting, vision
  • Downward deviation of eyes

Young and Middle Aged Adults

  • Chronic headaches
  • Difficulty walking/gait disturbances
  • Cognitive challenges or complaints
  • Urinary urgency or incontinence

Older Adults (NPH)

  • Difficulty walking/gait disturbances
  • Cognitive challenges/mild dementia
  • Urinary urgency or incontinence

How is hydrocephalus diagnosed?

Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, CT, MRI, or pressure-monitoring techniques. A physician selects the appropriate diagnostic tool based on an individual’s age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord.

What is the current treatment?

There is currently no known way to prevent or cure hydrocephalus and the only treatment option today requires brain surgery, which is most often treated by surgically inserting a shunt system. This system diverts the flow of CSF from the CNS to another area of the body where it can be absorbed as part of the normal circulatory process.

Management of Hydrocephalus

Managing medical treatment and care as well as maintaining quality of life for your loved one or yourself is part of living with a chronic neurologic condition. Hydrocephalus affects cognitive, social and physical development and abilities in diverse ways.