Attempt to explain new diagnoses....

Since Chiari is a rare disorder and not very well heard of, many people have asked what Chiari (kee-ar-ee) Malformation and Syrinx is, which is what Meghan has recently been diagnosed with.  I will do my best to try to explain these two conditions.

There are 3 different types of Chiari Malformation.  Type 1 is the most common, and is what Meghan has.  Type 2 is more severe and is accompanied by Spina Bifida.  This type can not be operated on.  Type 3 is a very serious and very rare type and also can not be operated on.  Luckily, Meghan's type can be operated on and the hope is that her symptoms will go away after surgery.

Chiari malformation is a brain abnormality that causes the cerebellum, the part of the brain that controls coordination and muscle movement, to protrude into the space normally occupied by the spinal cord. Some children are born with the condition, and others develop it as they grow (Meghan's neurosurgeon believes Meghan was born with Chiari, along with Hydrocephalus).  Chiari malformation sometimes happens because the space at the back of the skull, where the cerebellum sits above the spine, is too small or is unusually shaped. These "cramped quarters" squeeze the cerebellum and even part of the brain stem, which controls the nerves in the face and neck, down through the foramen magnum (a funnel-like hole below the skull through which only the spinal cord usually passes).  The pressure caused by Chiari malformation on the cerebellum, brain stem, and spinal cord can keep them from working correctly. It can also block the movement of cerebral spinal fluid (CSF), the liquid that surrounds the brain and spinal cord and protects them from injury. When CSF doesn't flow properly, it can build up in the brain and spinal cord and block communication of the brain's messages to the body (In Meghan's case, she has total blockage of CSF flow.  This alone warrants surgery).   If a Chiari malformation is severe enough to block the flow of CSF or put significant pressure on the brain or spinal cord, a child's nervous system can be affected. Symptoms can include dizziness and problems with balance and coordination. This means a child might fall down a lot, walk unusually, have trouble grasping items, or have poor hand-eye coordination.
Other symptoms can include:

• neck or chest pain
• headaches that are brought on by coughing, sneezing, or laughing
• difficulty swallowing, which may cause gagging, choking, or vomiting
• difficulty speaking
• rapid eye movements or vision problems like light sensitivity or blurred vision
• hearing problems like a tinnitus (ear ringing) or hearing loss
• weakness, numbness, tingling, or other abnormal feelings in the arms and legs
• insomnia
• depression

Signs of Chiari malformation in babies include:

• irritability when being fed
• excessive drooling
• weak cry
• trouble gaining weight
• arm weakness
• developmental delays

Children with Chiari malformations might have other conditions that are associated with the abnormality, such as:

• Hydrocephalus: An excessive buildup of CSF in the brain that causes an enlarged skull and, if left untreated, can lead to brain damage, a loss in mental and physical abilities, and even death.  Meghan was diagnosed with this in April 2012 and had surgery to place a shunt in her head.
• Syringomyelia: A disorder in which a cavity called a syrinx develops in the spinal cord. This cavity usually fills with fluid and stretches the spinal cord, which can cause permanent nerve damage and paralysis.  Meghan also has this on her lower spinal cord.  The hope is that after her decompression surgery, that her syrinx will diminish.  If it does not, she will need another surgery a few years down the road to remove the syrinx.
• Scoliosis: A condition that causes a curvature of the spine. Scoliosis can be a complication of syringomyelia, which causes an imbalance in the strength of the muscles that hold the spine straight and upright.  Meghan does not have this........thank goodness!

Unfortunately, there is no cure for Chiari malformation. Most children who don't have symptoms won't require treatment.  When symptoms do occur, and are serious enough to warrant surgery, your neurosurgeon will decide if surgery is necessary.  If surgery is necessary, it is called decompression surgery.  The surgery will help to relieve pressure on the brain and spinal cord, restore the flow of CSF, and prevent additional damage. This surgery usually takes several hours and involves removing part of the skull and a portion of the spine's top vertebra.  Surgery isn't a cure but it can help to greatly improve the problems caused by a Chiari malformation. It does have drawbacks, though. Surgery can be painful and can put a child at risk for infection and other complications.  Many people believe once the decompression surgery is done, that they are cured.  That is not the case.  The hope is that the decompression surgery will improve Chiari symptoms.  However, it is a possibility of a person that has been decompressed to have the Chiari come back.  This is common in kids whose brains are still growing, or if a person is involved in a whip-lash  type accident.  The whip lash motion can trigger the Chiari and a person can experience the Chiari symtpoms again.  30-40% of people who have been decompressed report being symptom free after surgery.  Another 30-40% report their symptoms improved slightly and another 10-20% report no relief of symptoms after surgery.  We ofcourse are hoping for a completely successful surgery for Meghan and that NO other surgeries will be needed for her down the road for Chiari, or her syrinx.