Toddler with Chiari

General
1

Hello. My daughter was diagnosed in Nov at 16 months old. 8mm herniation. No syptoms. Only had 1 brain MRI. Now almost 6 months later, I think she is displaying symptoms, but her neurosurgeon disagrees and is not concerned.

She has been grabbing either side of her head saying "ow" atleast once a day this week. I dont know if this is pain or pressure -NS says that because its the side of the head not the back of the head she is grabbing, its not Chiari. And since she was not crying/sneezing/coughing beforehand its not Chiari

She has been complaining about her feet on different occasions. The other night she had woken up screaming grabbing her legs. Took 45 minutes to calm her down and get back to sleep. I dont know if they hurt/tingle/are numb - NS says her neck and arms would bother her before her feet would so its not Chiari.

She has been pulling at her ears/sticking her finger in her ears. I dont know if they are ringing/pressure/pain, but there is something going on. -NS says not related

She is a terrible sleeper. Doesnt sleep for more than an hour uninterrupted.-NS didnt even address this

The NS has no plans on any upcoming MRI's. I then brought up to her that a friend well versed in Chiari, though not a doctor, had mentioned that CHloe's MRI clearly shows a retroflexed odontoid/CCI. The NS laughed at me. I understand my friend is not a doctor but she knows her stuff and I just wanted to hear what the NS had to say...after all, she never gave me as much as a flyer.

Attached is an MRI for anyone who is interested. Im not freaking out, and im not even thinking surgery, but these are not normal actions by CHloe and are a cause for concern. I have no other way to explain them

I am looking into a second opinion at The Cleveland Clinic, but I almost dont want to and look like the crazy hypochondriac mom...

2

I am so sorry that Chloe is not feeling well. You are correct that what she is doing is not normal. You are her mom and that being said you know her better than anyone. I tell my patients this all the time. If something is wrong you will know it. If a certain dr doesn’t listen then find someone who will. Listen to your instincts. If I had then my daughter would not have been sick for so long. I know that it is hard and that you want nothing more than your child to be healthy so you start to think that it’s your imagination. Many here have been told by several doctors that there symptoms are not related to chiari. Find an expert. There are several links here that can help you find someone. I will add Chloe to my prayer list. Hang in there momma!

3

Sorry to hear of Chloe's issues.

It never hurts to get additional input. In neurosurgery, anyone call call themself an expert in any sub-section of neurosurgery. However, there is a separate board of pediatric neurosurgery. I always recommend checking to make sure your child is being treated by someone who has that additional certification. Here is the web site of those who have passed the test http://www.abpns.org/diplomates.html

You will drive yourself crazy with all these concerns.

How can you tell for sure whether the Chiari is causing Chloe's problems?

You can't. It is a frustrating but true answer in pediatrics. She can't come up and say she has a head-ache. She is already incontinent of bowel and bladder. She is not yet walking well. Her handwriting can't be checked.

So, you want to ask yourself. Do you think anything is going on that is bad enough you would want Chloe to undergo removal of the back of her skull, knowing that, at 6 months of age, >80% will need another operation in the future(we can't make a big enough opening on an infant usually)?

Almost certainly, from what you've described, the answer is no.

So you are left with looking for someone who will follow her along and make sure she has as few symptoms as possible. How bad is her sleeping? Consider a sleep study(central sleep apnea is a common indication for Chiari decompression in infants).

Most of us would repeat an MRI when we don't know for sure which direction things are going. Expect her next MRI to have further herniation numerically. In growing chidlren, everything should be growing. Measure her fingers and her toes over time. they will grow. Measure her cerebellar tonsils over time. They should grow. You are looking for proportionate growth. With the next MRI(just ask for it, most MDs will do it, although they may ask that you follow up with neurology until to have confirmation of a neurosurgical issue(remember, a neurosurgeon treats anatomy with a knife. If that's not the treatment you are looking for, look elsewhere-usually neurology)), consider a head and entire spine MRI(and, if you really need additional data to push you toward surgery, consider a cine/flow MRI). Those studies will add to the time of the MRI but, at that age, Chloe already needs anesthesia.

There are several good lists of questions for treating MDs on this web site. Look at them and customize them to your needs.

If it would set your mind at ease, specifically ask for a repeat MRI(and ask them for their recommended timing). I often repeat the MRI in 6 months. Ask they think any other studies would be helpful(spine MRI, cine MRI, sleep studies). Ask if they think this would be better handled by a neurologist or a neurosurgeon.

You are doing the right things now. It doesn't sound like anyone would jump into treatment at this time. Getting additional data is always helpful(and it is always unsettling when you don't get a definite answer, which is often the case with children and Chiaris).

In terms of the retroflexed odontoid, get the MRI reading. If no one comments on it, it may simply be positional. For most children who get MRIs, because they are under anesthesia, they are further flexed than is normal. Looking at the entire MRI would give the treating MD a better idea(and again, that is an appropriate question for the Cleveland Clinic).

4

Thanks for your support. I don't want to jump to surgery at all, but I would like for her to have a Dr that takes my concerns seriously. After all being said, her neurosurgeon does not plan on doing another MRI anytime soon. The last one was Oct. (something about Chloe having heart surgery in June, laryngoposcy in September, and a neck then brain MRI in Oct?) She has had ENT issues immediately following her heart surgery, although her neursurgeon says its not related to Chiari and the ENT says it was. She has stridor, bilateral vocal cord peresis, choking while eating/drinking and sometimes on saliva, and some voice hoarseness all following her heart surgery. Could her neck have been hyperextended during intubation to onset the symptoms? There are a few cases out there with the same issues. I just want her to be as healthy as she can be.

5

Sounds like it's definitely time to get another opinion. Many of the symptoms you describe could be from a Chiari(and the midline issues she has had are classic-all the midline structures are being created at the same time, so most children with anatomical heart issues need to have their brain cleared as well).

6

Her heart surgery was for closing her PDA. She also has a PFO, although they say it is common and not an issue. Can you please clarify about the midline issue/structures?

Also to add, she was diagnosed as failure to thrive last spring/summer. AT 12 months she still had not doubled her birth weight (8lbs 6oz). The heart surgery helped her gain some, but she still is tiny. She is slowly growing on her own curve. At 21 months she is finally 20 lbs.

7

I understand the midline issues now. Thanks for clarifying. I did catch the age mix up. She is 21 months old now. Diagnosed at 16 months.

8

Yes. I'm not sure anyone has published their data but Boston Children's presented their data a few years ago at a Spina Bifida conference(Chiari II decompressions are more common in children, hence the spina bifida bent, even though the operation is the same). In their hands, >50% of patients under 5 years of age required a second operation due to bony regrowth. I reviewed my data and had about 80% of the under 2yo patients that required a second surgery(Boston Children's did not have large enough numbers to break down into smaller groups). Barring a complication, e.g. CSF leak, the adult re-operation rate is <5%.

9

So Dr. Trumble, In your honest opinion, am I overreacting? Should I just chill out and just keep an eye on her? Go ahead and get a second opinion? Im at a loss here. I honestly do not know what to do. Not to mention the second opinion at The Cleveland Clinc is a 3 hour drive each way.

10

You are Chloe's mom and I'm not. However, I do have7 kids and I would do what you are doing. She's having enough funky symptoms to have another neurosurgical evaluation. If she were my biological child, I'd get another opinion(it will probably be the same but perhaps with greater follow-up or review). Nothing wrong with chilling out, keeping an eye on her, and getting peace of mind with another opinion.

11

Thank you for listening and helping. I will definitely get a second opinion,and even if the results are the same, I may have a better chance with them checking on her and listening to my concerns. Its nice to know I am not crazy when it comes to this!

12

Just wanted to tell you that my 5 yr. old granddaughter had her decompression surgery last Nov. for her Chiari. She had it at the Cleveland Clinic with Dr Violette Recinos and she was more than wonderful. She was trained at Johns Hopkin and did her pediatric neurosurgery residency there. She is very knowledgeable about Chiari and is very kind and compassionate. I hope that she is who you are seeing. God bless.

13

Pdrum- Thank you. I have had such a hard time finding a good neurosurgeon and getting real feedback. I am considering Dr Recinos and/ Dr Lucciano. I have heard and read great things about them both. I will go over everything with the pediatrician on the 7th and have her get moving on the referral!

14

Just wanted to let you know that you can see lectures by Dr. Luciano at the ConquerChiari site under the Education link under the conference videos. You can also view Dr. Recinos lecture under the cfsinfo.org. site under lectures also. They both were of great comfort to me during our preoperative waiting period. Thinking of you and your daughter. My prayers are with you.