Chiari Malformation

Dear Lisa,

My 8 yr old daughter had brain surgery three yrs ago for a Chiari Malformation. She is now having the same symptoms but they have become more severe and persistent. My question is, can a Chiari malformation return? If not what could mimic the exact same symptoms of a Chiari?(severe headaches, fatigue, pain in neck radiating down arm, shock like feeling going down arm, depression, she can't sleep, VERY agitated, blurred vision, ringing in ears, constant blinking. The list goes on and on, but I know you are busy, I can't get her neurosurgeon or neurologist to listen. I really need help, and I don't know where to get it.

“Worried Sick”

Dear “Worried Sick”,

It sounds like you and your daughter have been through a lot over the last 8 years. As you already know, Chiari Malformation (also referred to as Arnold-Chiari Malformation) occurs when the part of a child’s brain called the posterior fossa is downwardly displaced towards the spinal cord. This downward displacement causes compression and elongation of the tissues and the cranial nerves which leads to the resulting signs and symptoms known to the disorder. Although children with Chiari Malformation are born with the condition, only about 1/3 of these children develop symptoms in infancy. (1)

The common symptoms include neck stiffness, neck pain, headache, nystagmus (involuntary periodic movement of the eyes from side to side or from up to down), difficulty swallowing, drooling, vomiting, inspiratory stridor, and in some cases apnea. (1,2) A poor gag and swallow reflex can also develop due to the compression of the cranial nerves. This often leads to aspiration of food and liquids during eating and recurrent Pneumonias. (1) In older children decreased strength in the upper extremities with increased muscle tone and exaggerated deep tendon reflexes may also be present. (1)

Chiari Malformation is diagnosed through imaging studies such as x-rays and/or an MRI of the brain. In cases of milder disease the symptoms may stabilize and the child outgrows the symptoms. In more severe cases, especially when apnea or recurrent pneumonia is involved, surgery may be necessary. The surgical procedure that is typically performed to treat Chiari malformation is a posterior fossa decompression. This decompression alleviates the pressure on the fourth ventricle and the affected cranial nerves which hopefully prevents worsening of symptoms.

Since your daughter is developing the same symptoms that she experienced before her surgery it would be important to have her re-evaluated. You mentioned that you informed your daughter’s Neurosurgeon and Neurologist about her symptoms, but they did not seem to listen. You did not mention if your daughter had an evaluation, a scan of her Brain or what the specialist’s impression of the findings was.

In some cases it may seem that a Doctor is not listening, but what they may be doing is monitoring symptoms to see if they progress. In other cases the Doctor may be deciding what treatment approach to take. Sometimes a child’s complicated condition goes beyond the expertise of a certain specialist and a second opinion is necessary.

I suggest that you write down all of your concerns and questions and schedule a consultation with your daughter’s specialists. At the consultation your Doctor should be able to tell you if your daughter's symptoms are due to her Chiari Malformation or due to another cause. Don’t be afraid to ask your Doctor what his impression is, what his plan is and what pain control measures can be instituted in order to alleviate your daughter’s discomfort. Pain medications may initially be withheld because they can mask symptoms and prevent an accurate diagnosis. If your daughter’s symptoms have been persistent and the disease course has been determined the institution of pain medication for comfort should be a viable option.

When a child has a chronic or complicated medical condition it is a good idea to seek a second opinion. A second opinion can help you gain additional insight into your daughter’s condition, offer you new alternatives or reassure you that you are already on the right course. Some people are afraid to get a second opinion because they do not want their Doctor to become insulted or because they are affraid that their Doctor will think that they do not trust him. A good Doctor will not take this personally and understand that you are trying to do the best that you can for your daughter.

Just like every other profession, there are some Doctors that have a stronger background, more expertise or more experience in a particular area of medicine as compared to others. By getting a second opinion you can explore all of your options and get a different perspective on your daughter’s condition.

When seeking a second opinion, it is a good idea to search out a Neurosurgeon associated with a major Children’s Hospital or associated with a major university in a large city. Your daughter’s pediatrician is a very good resource and should be able to refer you to a specialist that can answer your questions. A Nurse at your insurance company should also be able to direct you where to find a second opinion.

You will need to get a copy of your daughter’s operative report from the hospital where she had surgery and copies of all diagnostic testing as well as films from x-rays or MRI scans performed. The specialist that you see for a second opinion will need to review this information.

Many times very practical, useful and informative suggestions come from other parents experiencing the same situation. You can meet other parents with children diagnosed with Chiari Malformation by joining a support group. In order to find a support group you can contact a children’s rehabilitation hospital, children’s hospital or the Board of Health in your area.

Chiari Malformations are very commonly associated with spinal cord defects such as Spina Bifida. Spina Bifida is more common than Chiari Malformations and it may be easier to find a Spina Bifida support group close to your home. You can find information about Spina Bifida support groups on the Spina Bifida Association Website. Within the Spina Bifida Association and support groups you should be able to find other parents with children diagnosed with Chiari Malformation.

I hope this information helped and I wish you and your daughter well.

Spina Bifida Association

Spina Bifida Support Groups

References:
(1)Betz C, Hunsberger M, Wright S. Family-Centered Nursing Care of Children. 2nd ed. Philadelphia, PA:W.B.Saunders Company. 1994:1789-1791.
(2)Behrman R, Kliegman R. Nelson Essentials of Pediatrics. Philadelphia ,PA: W.B.Saunders Company. 1990:438.650-651.


Lisa-ann Kelly R.N., P.N.P.,C.
Certified Pediatric Nurse Practitioner

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