Cerebrospinal Fluid Leaks And Encephalocele

What is Encephalocele?

Encephalocele is a condition in which a gap in the bone of the skull allows the brain to push through and possibly leak cerebrospinal fluid (CSF). Encephaloceles can happen anywhere from the base of the skull to the forehead, the nose, in the nasal cavity and sinuses, and within the ears. The result is clear drainage from the nose, down the back of the throat, or even from the ear. Encephaloceles and CSF leaks increase the risk of developing meningitis, a life-threatening intracranial infection.

Causes of Cerebrospinal Fluid Leaks

There are many causes of CSF leaks in children and adults, including head trauma, prior skull surgery, a developmental abnormality, or simply occurring spontaneously. Encephaloceles are also correlated with increased pressure in the brain and CSF.

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Symptoms of Encephalocele

The middle ear (the space behind the eardrum), mastoid, and nasal cavities are all located just below the base of the skull. Encephaloceles and CSF leaks can present symptoms such as ear fullness, hearing loss, nasal obstruction, and clear nasal or ear discharge. CSF leaks can affect the sinuses, nasal cavities, middle ear, mastoid bone, and ear canals. The symptoms may change depending on the location of the leak.

Additional symptoms of cerebrospinal fluid leaks are:

• Headaches that improve while lying down
• Vision changes
• Hearing changes, such as ringing
• Sensitivity to light or sound
• Balance problems
• Drainage down the back of the throat

It is possible to have an encephalocele without CSF leak, and it is possible to have CSF leak without an encephalocele.

Diagnosing CSF Leaks

To confirm a diagnosis of encephalocele and/or CSF leak, images of the skull will be taken with magnetic resonance imaging (MRI), computed tomography (CT) scan, or both. An otolaryngologist will also ask about your symptoms and health history and perform a physical examination. They will examine the area of reported drainage and may ask you to place your head in a certain position, such as leaning forward. If fluid can be collected, it may be sent to a lab for testing to confirm that it is cerebrospinal fluid.

How to Treat Encephalocele

At AOO/ENT specialists of the Rockies, we treat encephaloceles in different ways depending on its cause and the severity of symptoms. Surgery is recommended to reduce the risk of meningitis and prevent additional loss of CSF. The initial non-surgical treatment for CSF leaks includes hydration, bed rest, and saline infusions. High flow leaks may be temporarily stopped with a lumbar drain, or permanently diverted with a shunt placed by a neurosurgeon. However, for most persistent CSF leaks of the skull base, surgical repair is recommended. The goal of surgery is to re-establish the separation between the brain and the ears or nose.

Surgery to repair an encephalocele/CSF leak is done in a variety of ways, which primarily depends on its location. For encephaloceles within the nasal cavity and sinuses, the procedure is usually done with an endoscope, a very thin tube with a camera on the end to help visualize internal tissue, inserted through the nose. Encephaloceles within the middle ear or mastoid bone may require an incision behind the ear and microscopic surgery to repair the defect and stop the CSF leak.

Some encephaloceles within the ear require a craniotomy, in which the skull is opened above the ear in order to access and repair the skull base defect. The specific technique will vary depending on the size and location of the encephalocele or CSF leak. Your surgeon may use a synthetic graft, surgical bone cement, or your own tissue to repair the encephalocele.

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If you have symptoms suggestive of a CSF leak or encephalocele, make an appointment with our trusted otolaryngologists sooner rather than later. The majority of cases require surgical repair to reduce the risk of meningitis and loss of CSF. With a few tests, we can help identify the cause of your symptoms and offer targeted treatment to help you feel back to normal. Call a location that’s convenient for you or fill out a contact form online to get in touch with our diverse team of otolaryngologists and audiologists today.

Jeffrey L. Cutler, M.D.

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Dr. Jeffrey Cutler is board-certified with the American Board of Otolaryngology and received his medical degree at Tulane University Medical School in New Orleans. He performed his residency at Vanderbilt University Medical Center in Nashville. In addition, he completed a 1 year fellowship in nasal and endoscopic sinus surgery.  His area of expertise includes the medical and surgical treatment of complicated sinus and skull base diseases.  Dr. Cutler has published many articles on office based nasal and sinus procedures - particularly on balloon sinus and Eustachian tube dilation  When not at the office, he enjoys spending time outdoors with his family, skiing and cycling.

• Jeffrey L. Cutler, M.D.

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