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 Jane S. - Chiari Malformation


Chiari Malformation

Problem

The patient, Jane S., had symptoms that were getting much worse over an eighteen-month period.  She had severe dizziness, vertigo and difficultly swallowing.  If she bent over, coughed or laughed there was an increase in pressure causing immediate headaches and vision problems.

Initially she went to a primary care physician. She had a 6-month-old baby and was sleep deprived, so he thought it might be a migraine.  She started getting more sleep and the symptoms got worse.

Jane went to a new primary care physician who referred her to Dr. McLaughlin who correctly diagnosed the problem as Chiari malformation.

Solution

"When I met him, it was so obvious he knew what he was doing and I felt completely confident that he could do the surgery.  I have only met a few surgeons, but my experience has been that they are often arrogant and abrupt with no bedside manner.  He wasn’t that way at all.  The way he described everything was very clear."  Dr. McLaughlin gave her the names of two doctors, one at Columbia Presbyterian and one at U Penn.  Jane went to the Web to review the bio for both the doctors; it was clear that Dr. McLaughlin had more experience than either of the other two. 

It turned out that her cerebellum, the back part of the brain that controls coordination, was elongated and herniated out of the bottom of her skull into her spinal canal. This was putting pressure on her spinal cord and causing a build up of cerebrospinal fluid.  Cerebrospinal fluid was not draining properly out from her head into her spinal canal causing her headaches.  Dr. McLaughlin shaved off part of her skull, and the back parts of the C1 and C2 vertebrae and opened the fluid pathway.  The operation took 4 hours and she was discharged 3 days after surgery on Thanksgiving Day of 2002.

Dr. McLaughlin notes:

Chiari Malformation is an abnormality of the cerebellum, the back portion of the brain, where the brain funnels down to the spinal cord.  The tips of the cerebellum, called the tonsils, sag down into the opening where the brain attaches to the spinal cord.  These bottom tips obstruct of the normal flow of Cerebral Spinal Fluid (CSF).  The result is that the cerebellum clogs the spine like the neck of a bottle.  Therefore every time you cough or strain, it causes terrible headaches and neck pain.  These tonsils can slide down deep into the spinal column.  There is no medicine, no exercise, and no physical therapy to treat it.  It is a mechanical obstruction; the only thing that can be done is to create a larger space where the brain meets the spinal cord.  In Jane’s case, I created a slightly larger hole in the bottom of the skull and enlarged the spinal canal to make more space for her brain and spinal cord to alleviate the compression and remove the pressure. 

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