Herniation syndrome

Alternative names
Brain herniation; Transtentorial herniation; Uncal herniation; Subfalcine herniation; Tonsillar herniation; Herniation - brain

Definition

A brain herniation is the displacement of brain tissue, cerebrospinal fluid, and blood vessels outside the compartments in the head that they normally occupy. A herniation can occur through a natural opening at the base of the skull (called the foramen occipitalis) or through surgical openings created by a craniotomy procedure.

Herniation can also occur between compartments inside the skull, such as those separated by a rigid membrane called the “tentorium”.

Causes, incidence, and risk factors

A brain herniation occurs when pressure inside the skull (intracranial pressure) increases and displaces brain tissues. This is commonly the result of brain swelling from a Head injury. It can also be caused by space-occupying lesions such as primary brain tumor, metastatic brain tumor, and hemorrhages or strokes that produce swelling within the brain. Hydrocephalus (accumulation of fluid in the brain) can also lead to brain herniation.

A brain herniation itself often causes massive stroke. This results from poor blood supply to some areas of the brain and compression of vital structures that regulate your breathing and circulation. This can rapidly lead to death or brain death.

Brain herniations are the most common secondary effect of expanding masses in the brain.

Symptoms

     
  • Progressive loss of consciousness  
  • Coma  
  • Irregular breathing  
  • Respiratory arrest (no breathing)  
  • Irregular pulse  
  • Cardiac arrest (no pulse)  
  • Loss of all brainstem reflexes (blink, gag, pupillary reaction to light)

Signs and tests

A neurologic examination would show an impaired level of consciousness. Depending on the severity of the herniation, one or several brainstem reflexes and cranial nerve functions will be impaired. The patient would show an inability to breathe consistently, and heart rhythms would be irregular.

Treatment

Brain herniation is a medical emergency! The goal of treatment is to save the patient’s life.

To help reverse or prevent a brain herniation, the medical team will treat increased swelling and pressure in the brain. This can be accomplished by:

     
  • Corticosteroids such as dexamethasone, especially in cases where a tumor is involved  
  • Mannitol or other diuretics  
  • A drain placed into the brain to drain off fluid, in the case of a mechanical obstruction causing herniation  
  • Removing the blood if a massive hemorrhage is present and causing herniation, although the outlook in these cases is poor  
  • Placing a tube in the airway (Endotracheal intubation) and mechanically ventilating (forced breathing) at a rapid rate to reduce the levels of carbon dioxide (CO2) in the blood

Expectations (prognosis)

Once herniation in the brain’s temporal lobe or the cerebellum occurs, death is often inevitable. Herniation of other areas of the brain areas has a more variable outlook.

Complications

     
  • Permanent and significant neurologic problems  
  • Brain death

Calling your health care provider

If decreased alertness or other symptoms develop suddenly, particularly if there is a known Head injury or brain lesion, go to the emergency room or call 911. A neurologist or neurosurgeon will ultimately be involved in the patient’s care.

Prevention

Prompt treatment of increased intracranial pressure and related disorders may reduce the risk of brain herniation.

Johns Hopkins patient information

Last revised: December 3, 2012
by Martin A. Harms, M.D.

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