Hydrocephalus

The signs and symptoms of hydrocephalus vary by age of onset.

Common signs and symptoms of hydrocephalus in infants include:

• An unusually large head, or rapid increase in head size
• A bulging or tense soft spot (fontanel) on the top of the head
• Vomiting
• Sleepiness
• Irritability
• Poor feeding
• Seizures
• Eyes fixed downward 
• Deficits in muscle tone and strength
• Poor responsiveness to touch
• Poor growth

If hydrocephalus has progressed by the time of birth, it may result in significant intellectual, developmental and physical disabilities.

In toddlers and older children, signs and symptoms may also include:

• Headache
• Blurred or double vision
• Sleepiness or lethargy
• Nausea or vomiting
• Unstable balance
• Poor coordination
• Poor appetite
• Urinary incontinence
• Behavioural and cognitive changes
• Irritability or personality changes
• Decline in school performance
• Delays or problems with previously acquired skills, such as walking or talking

Common signs and symptoms in young and middle-aged adults include:

• Headache
• Lethargy
• Loss of coordination or balance
• Loss of bladder control or a frequent urge to urinate
• Impaired vision
• Decline in memory, concentration and other thinking skills 

Among adults 60 years of age and older, the more common signs and symptoms of hydrocephalus are:

• Loss of bladder control or a frequent urge to urinate
• Memory loss
• Progressive loss of other thinking or reasoning skills
• Difficulty walking, often described as a shuffling gait or the feeling of the feet being stuck
• Poor coordination or balance

Long-term complications of hydrocephalus can vary widely and are often difficult to predict. Less severe cases, when treated appropriately, may have few, if any, serious complications.

Adults who have experienced a significant decline in memory or other thinking skills generally have poorer recoveries and persistent symptoms after treatment of hydrocephalus.

The severity of complications depends on other medical or developmental problems, severity of initial symptoms and the promptness of diagnosis and treatment.

Because the symptoms associated with hydrocephalus can be also caused by other conditions, it is important to get a timely diagnosis and appropriate care.

Seek emergency medical care for infants and toddlers experiencing these signs and symptoms:

• A high-pitched cry
• Problems with sucking or feeding
• Unexplained, recurrent vomiting
• An unwillingness to move the head or lay down
• Breathing difficulties
• Seizures

Seek prompt medical attention for other signs or symptoms in any age group.

Cerebrospinal fluid normally flows in the spaces around the brain and spinal column and plays an important role in brain function.

Hydrocephalus is caused by an imbalance between the volume of cerebrospinal fluid which is produced and how much is absorbed into the bloodstream.

Excess cerebrospinal fluid in the ventricles occurs for one of the following reasons:

• Obstruction of the normal flow of cerebrospinal fluid
• Poor absorption by blood vessels in tissues near the base of the brain, often related to inflammation of brain tissues from disease or injury, is a less common cause
• Overproduction, when cerebrospinal fluid is created more quickly than it can be absorbed, is rare

In many cases, the exact event leading to hydrocephalus is unknown. Some developmental or medical problems have been found to contribute to or trigger hydrocephalus.

In new-borns:

• Abnormal development of the central nervous system that can obstruct the flow of cerebrospinal fluid, such as open myelomeningocele (a form of spina bifida)
• Bleeding within the ventricles, a possible complication of premature birth
• Infection in the uterus during a pregnancy, such as rubella or syphilis, that can cause inflammation in foetal brain tissues

Other factors that can contribute to hydrocephalus among any age group include:

• Lesions or tumours of the brain or spinal cord
• Central nervous system infections, such as bacterial meningitis or mumps
• Bleeding in the brain from a stroke or head injury
• Other traumatic injury to the brain.

Medical history and assessment of symptoms

General physical examination

Neurological examination to assess muscle condition, movement, sensation and well-being.

Brain imaging, often with ultrasound or MRI, can help diagnose hydrocephalus and identify underlying causes of the symptoms.

Ultrasound may also detect hydrocephalus prior to birth when the procedure is used during routine prenatal examinations.

Surgical treatments may be used to restore and maintain normal cerebrospinal fluid levels in the brain.

1. Surgical insertion of a shunt is the most common treatment for hydrocephalus. 

A long, flexible tube with a valve that keeps fluid from the brain flowing in the right direction and at the proper rate. The excess cerebrospinal fluid is drained to another part of the body, such as the abdomen or a chamber in the heart, where it can be more easily absorbed.

Shunt systems can stop draining cerebrospinal fluid or poorly regulate drainage because of mechanical malfunctions, blockage or infections. People who have hydrocephalus usually need a shunt system for the rest of their lives, and regular monitoring is required.

1. Endoscopic third ventriculostomy is less commonly used.

In the procedure, a small video camera is inserted to guide the surgeon to make a hole in the bottom of one of the ventricles or between the ventricles to enable cerebrospinal fluid to flow out of the brain. Complications of ventriculostomy include bleeding and infections.

Patients require close, regular monitoring for complications following surgery. Complications or failures require prompt attention, surgical revisions or other interventions. 

• A multidisciplinary team is often required to provide therapies and manage symptoms or functional impairments resulting from hydrocephalus.

  Children may be seen by:

  • Paediatrician 
  • Paediatric neurologist 
  • Occupational therapist
  • Developmental therapist 
  • Mental health provider
  • Social worker
  • Special education teacher

  Adults with more-severe complications also may require the services of occupational therapists, social workers, specialists in dementia care or other medical specialists.

  With the help of rehabilitative therapies and educational interventions, many people with hydrocephalus live with few limitations.

   Parents of children with hydrocephalus may access local resources available to provide emotional and medical support.