HELP! Chiari symptoms in toddlers?

General Parents and Caretakers of Children with Chiari
1

I was operated on for Chiari malformation last year, as was my eldest daughter (now 18 years old).

I also have a just-turned 3 year old son. He is the "classic" tough guy - falls, runs, smacks into stuff and it never phases him.

Please bear with me....I have a point.....

Well, as of September (4 months ago), he was still 2 years old and wearing a size 24 months - although they were getting pretty small. I bought him some 2T's....and boom! by November, I had to start buying him 3T's....and now he is in a solid 3T and can wear some 4T's.

A few weeks ago, maybe a month ago, I was playing with him and he turned his head suddenly to the right, his face crinkled up in pain and he yelped "ouch!" crouched down and grabbed the back of his head at the base of his skull with both hands, while screaming "ow! ow! ow!"

Of course, I asked if he was okay. He waited a second or two, raised his head, gave it a little, slight shake and said, "yes. that just happens sometimes."

Me (keeping my voice conversational), "What happens sometimes?"

Him, "THAT". [me: what?] "When I turn my head sometimes it just hurts real bad. But it stops."

Me," So it's happened before?"

Him, "Yes. I don't want to talk about it."

That has happened about 15 times since then (that I have witnessed).

Questions:

1. Has anyone else experienced this (pain in the back the head when turning the head) - personally as a symptom of Chiari or in their child?

2. Any recommendations re: pediatric doctor? I live in MD. You can message me privately with that info if you'd like.

I am scared sick. Any information or experience you can share about young children with Chiari would be greatly appreciated.

2

We are waiting for confirmation of my daughter’s Chiari. (I saw the diagnosis on her MRI report, but we have not followed up with her neuro yet).

The symptoms that my daughter (4) seems to have are chronic (but not severe) headaches. She points to her forehead and cries that her “brain hurts”. She also has chronic pain/discomfort in her legs.

As far as other symptoms, she also has a host of other diagnoses due to a chromosome abnormality, so I’m not sure which are from the CM or her disorder. But she has been failure to thrive since infancy (currently 23lbs @ 4yrs), constipation, reflux, temperature regulation issues, dyspraxia, hypotonia, sensory issues, among others.

I can say that my daughter has always been very accurate in describing her symptoms. Like you, I try not to feed into complaints or put thoughts in her head. It sounds like you have more than enough reason to be concerned and at least ask for a neuro consult.

Best of luck!

3

Megan,

Thank you. My son has always had digestive problems - so much so that he drank breast milk for 2 + years. Allergies to soy and dairy; he is often constipated. Complains of tummy hurts almost every night. I also have EDS, so I was suspecting he may have that, also.

I will definitely take him to the doctor re: this issue (even though we are in the midst of changing pediatricians b/c we just moved so how to approach the topic with a new ped dr is another issue.....).

I just wasn't sure if it's possible to have some sort of growing pain at the base of the skull....For me, the back of my head/base of my skull always felt like it had been hit with a baseball bat. It was a constant pain. Can Chiari pain be sporadic or occur when turning the head?

Also, Megan, if you don't mind...how do they do a MRI on a child so young? Do they have to medicate him to stay still? I had to have an upright MRI to diagnose Chiari....how could he have an upright MRI if he is medicated?

The whole thing is just scaring me sick.

Again, thanks for your time. Good luck with your daughter.


Megan said:

We are waiting for confirmation of my daughter's Chiari. (I saw the diagnosis on her MRI report, but we have not followed up with her neuro yet).

The symptoms that my daughter (4) seems to have are chronic (but not severe) headaches. She points to her forehead and cries that her "brain hurts". She also has chronic pain/discomfort in her legs.

As far as other symptoms, she also has a host of other diagnoses due to a chromosome abnormality, so I'm not sure which are from the CM or her disorder. But she has been failure to thrive since infancy (currently 23lbs @ 4yrs), constipation, reflux, temperature regulation issues, dyspraxia, hypotonia, sensory issues, among others.

I can say that my daughter has always been very accurate in describing her symptoms. Like you, I try not to feed into complaints or put thoughts in her head. It sounds like you have more than enough reason to be concerned and at least ask for a neuro consult.

Best of luck!
4

I've never seen an upright MRI done on a toddler. They would need to hold still for the entire MRI(hard enough for an adult).

Here's the question you want to answer. How severe are the symptoms? If he has occasional pain but it doesn't prevent him from leading a normal life, many parents choose to follow their child clinically(not see a specialist or get an MRI).

With your family history, there is an increased risk of Chiari. But remember, 80% of patients with Chiari never require surgical intervention.

If the symptoms are bad enough, I would suggest asking your pediatrician to order a head MRI without contrast(with anesthesia) to rule out Chiari. If you find a Chiari, get a referral to a pediatric neurosurgeon. A list of board-certified pediatric neurosurgeons can be found at http://www.abpns.org If the MRI is normal, it could give you comfort.

5

Dr. Trumble,

First, thank you for the information and for taking the time to respond. It is very, very much appreciated.

Since I and my daughter have Ehlers Danlos Syndrome, we needed an upright MRI in order to "see" the CM. Laying down, I did not have CM; upright, it was 7+ mm. I couldn't imagine how one could be done on a toddler; I hope that if he does have CM, this is not the case with him.

While I understand that Chiari may be asymptomatic in some patients, the "wait and see" approach scares me -- and this is why:

1. The damage from (or symptoms of) Chiari may be severe. For example, in my family (myself and my teen), symptoms included: problems swallowing; walking "drunk;" sudden onset of narcolepsy; loss of bladder/bowel control; memory and speech problems; and nerve damage/pain/numbness on the face and various areas of the body, just to name a few.

2. From what I understand, surgery does not necessarily or normally reverse the symptoms or damage. I was lucky in that most of my symptoms are gone. However, I understand that this is the exception rather than the rule. Since my son started complaining of head pain almost daily.... right after a serious growth spurt....my concern is that if I wait and he has another grown spurt, the damage could be severe (i.e., losing the ability to walk, talk, go to the bathroom?) and possibly irreversible. Maybe EDS patients are more likely to have symptomatic Chiari malformation??? I don't know.

Again, thank you for your input and your involvement on this board. It is invaluable.

6

Hi!

My daughter was diagnosed with Chiari at age 3. She is now 5 and has not had surgery. She woke up one day with a stiff neck, and would not turn it or look up, and a headache in the back of her head. After several months of headaches (every other day, at the back of her head, increased with activity, coughing, sneezing...as one neurosurgeon told us...a classic presentation for a child her age), and our pediatrician blowing us off, we made an appointment ourselves with a Neurologist. After much pressure from me, they ordered an MRI showing a 13mm Chiari Malformation. I pursued genetics due to her hypermobile joints, and both geneticist also believe she has EDS type 3.

We are in wait and see mode. BUT we closely monitor with routine MRI's, visits with our Neurosurgeons, genetics, ophthamologists, sleep studies etc. Due to some mild instability found by one surgeon we saw, she also has an Aspen collar that she uses when she is actively playing, to avoid damaging it if she were to get bumped or fall hard. I think wait and see can be a good place to be if correctly monitored. Toddlers cannot have upright MRI's, as it requires too much sitting still for most. We have been told that our daughter could possibly have one at age 6 or 7 if we wanted to try.

In regards to doctors, near you Dr. Tye comes well recommended by friends of mine who's children have both EDS and Chiari and Dr. Keating also. We have a local neurosurgeon (an hour away) who works a lot with Chiari, who orders our MRI's and follows our daughter, but we would travel to Iowa for Dr. Menezes should she need surgery.

7

My daughter was sedated (general anesthetia) for her MRI. I don’t believe it would have been possible for her to have an MRI without sedation.

Hope that helps!

8

Giovanna,

Thank you so much! This info was very helpful. I will travel for a doctor, if necessary...this is such a critical procedure!

One more thing - not to scare you, really, truly -- but I think I am so worried because 3 close relatives (my brother, father, and my father's sister) all died in their sleep with no warning within an 18 month time period. They all had a history of headaches, EDS symptoms (plus fibromyaligia in the case my aunt) and are all from the side that I strongly suspect the EDS & Chiari came from. This may be an unrealistic fear on my part...but it is there.

So, for me, it is good to hear that your daughter is doing okay with monitoring.

I wish you and your daughter the best of luck.



Giovanna said:

Hi!

My daughter was diagnosed with Chiari at age 3. She is now 5 and has not had surgery. She woke up one day with a stiff neck, and would not turn it or look up, and a headache in the back of her head. After several months of headaches (every other day, at the back of her head, increased with activity, coughing, sneezing...as one neurosurgeon told us...a classic presentation for a child her age), and our pediatrician blowing us off, we made an appointment ourselves with a Neurologist. After much pressure from me, they ordered an MRI showing a 13mm Chiari Malformation. I pursued genetics due to her hypermobile joints, and both geneticist also believe she has EDS type 3.

We are in wait and see mode. BUT we closely monitor with routine MRI's, visits with our Neurosurgeons, genetics, ophthamologists, sleep studies etc. Due to some mild instability found by one surgeon we saw, she also has an Aspen collar that she uses when she is actively playing, to avoid damaging it if she were to get bumped or fall hard. I think wait and see can be a good place to be if correctly monitored. Toddlers cannot have upright MRI's, as it requires too much sitting still for most. We have been told that our daughter could possibly have one at age 6 or 7 if we wanted to try.

In regards to doctors, near you Dr. Tye comes well recommended by friends of mine who's children have both EDS and Chiari and Dr. Keating also. We have a local neurosurgeon (an hour away) who works a lot with Chiari, who orders our MRI's and follows our daughter, but we would travel to Iowa for Dr. Menezes should she need surgery.

9

Thanks, Megan. It does help. There are so many opinions and doctors do things so many different ways.

I wish you and your daughter the best of luck.

Megan said:

My daughter was sedated (general anesthetia) for her MRI. I don't believe it would have been possible for her to have an MRI without sedation.

Hope that helps!
10

MomMD,

Wow, I think you’ve had your fair share already! I’m not a mom, but to me it seems that an MRI will either give you some piece of mind (no syrinx) or let you know it’s time to be more vigilant- an opportunity to enact a plan. The not knowing would be very hard for me, and I get he can’t have an upright, but it would give some answers, at least, and a direction.
Jenn

11

Jenn,

Thanks for your feedback. I am definitely going to have him checked out, even though he cannot have an upright MRI. I agree -- not knowing is the worst!

I have to change pediatricians (we moved; I am waiting for a recommendation from a friend for a name) and at his checkup, I will bring it up, along with our family history. I hope everything is okay. But I have a "feeling" that it's not.

Since I posted this, he has mentioned his head hurting several times (at the base of skull) "when he turns it."

Again, thanks.

12

I just read MissKriss’s post and she brought up the sleep study test - might be a good idea.