Chari Type 1 surgery with Syrinx and asymptomatic.

Brain and nerves
1

Hey there. We have a different situation to most in that our Son (15 yo) was born with a Type 1 Chari, and Syrnix which we have been monitoring for a few years now by annual MRI. We are (or were...) blessed to say he is Asymptomatic. He also has fused C1 and C2, that were all part some abnormalities at birth. Essentially, he is just a normal 15 yo boy with limited neck movement, and some slight asymmetry. But arent well all a bit imperfect? [Smile]

 

However, that was a double edged sword in that this year the Syrinx grew, and our Neurosurgeon said it was now time to look at Surgery before it became a problem.  We were told that the Sryinx would shrink over time, and esssentially disappear.

 

The 3 of us discussed the matter for a short time, and decided surgery from a, preventative measure, would be the best option. My wife and I also went looking on the internet for answers, but it was littered with horror stories, which to some extent made as feel grateful that our son didnt have symptoms, but fearful that those symptoms may develop after surgery, or there would be “something” there from surgery that wasnt there before. Our Dr basically brushed over this point, and said there would be no problems. We were lucky to also find a video on Youtube where 3 doctors talk about this very issue in a Webinar......whether or not to operate on a Chari patient who has no symptoms. The general answer was “not necessarily, but it was case by case”......but if there was a Syrinx also present, then it was an overwhelming “yes”.   

 

Surgery took place on the 20th June. They did a decompression and Dura patch, and cauterized the tonsils.

 

The DR said surgery went great. He has a 15cm scar at the back of his head, and spent just short of 2 days in ICU. Then he was moved to the ward for 4 days, where we were all amazed at his recovery. We were sent home on day 4. Without any meds for Nausea or Pain. Big mistake!

 

Anyway, over the weekend, it became a downhill spiral of him not being able to keep anything down, and that meant being sick with having just had the the back of your head open!!! I cannot imagine the pain of dry reaching and straining.

 

Fantastically, our DR is now on holidays! Go figure. To add to that,  5 Neurosurgeons on staff, and 4 of them are on holidays all at the same time. Genius! So we were left to deal with the Condescending and belittling admin staff at the private practice. And I dont mean to mince words there. I am a professional person, and cannot say that I have ever seen a culture like this of keeping a “client” away from the “boss”. Anyway, that is all another argument for another day.

 

So our son went back to Hospital yesterday in an ambulance because he couldn't be driven in a car due to vomiting. Just getting into the Ambulance made him throw up again. The sole remaining Neurosurgeon has been in, ordered blood test, and they have stabilised him. Drugs have him happy at the moment.

 

But now its like WTF have we done.

 

This site is also full of horro stories and some of hope and “success”, but most of those also seem to have a side note of regression or never ending problems.

 

Has anyone had a similar situation to ours where someone has not had symptoms beforehand, had the operation, and then had a “new reality” afterwards?

 

Im not sure why I am on here, not sure what I want to be told,, or hear..........its a confusing time. I am in a dark place, and if I am in a dark place, my wife is in a pitch black one......

 

2

Well I'm very sorry to hear about your son I don't know how much you really want to know what could happen or has happened or or what the future holds I mean I really can't tell you why I'm one of the few that had very little beforehand symptoms besides headaches but when I came out of surgery I came out with seizures pretty bad I'm on two medicines for that it's pretty scary not have them before and now I do but like I say everybody's different I scratch off my own skin it's they're saying it's it's just something of my anxiety or depression that I have from the Arnold Chiari of which made no sense to me but they say that it's all connected and while I was at the doctor today my vision is slowly going where are the blurriness is coming pretty bad and the double vision so that's not too good I also have body tremors and this is all after a surgery of decompression when my doctor said I had to have it and I was told I would be perfectly fine afterwards I came out of the surgery having the seizures and the next day my neurosurgeon when on vacation so I had nobody around me that could answer any of my questions either are you can do is pray and Hope that he gets better I'm much older I'm 40 years old I heard it's much easier for younger people than it is for older I really hope he gets better and I send all my luck is way

3

Oh I do feel for you all especially your son. I am older but simular to what your son is experiencing. I vomited every morning for weeks even when it did stop I reached for antisickness pills a soon as I opened my eyes, surgery was to stop symptoms getting worse. I am 20 months since decompression and still have some symptoms, but can get on with most things. I am due to see consultant . They told me I was one of the I unlucky ones to suffer the sickness and dizziness for so long. I do hope your son sees this easing off over the coming months. I thought there would be no end, but I'm back to working part time.

Keep us posted as to how he does. It's awful at 1st and a worry as parents. I needed help with walking and bathing for a while too, your helpless at 1st but does slowly get better. Can I ask are you in UK?

4

No were are in Australia. There seem to be few awesome success stories, and a huge amount of "unlucky ones".

Talking of unlucky ones.... surgeon not to be on holidays has said that his reaction right now is typical of someone that has just has the surgery, and before it may have been masked by steroids that were provided after surgery

However, we cant dismiss that he had NO symptoms beforehand.....nothing!!...and if he comes out of this with anything other than what he was before.....well, lets just say this is now at the forefront of our darkest fears. Lets face it, without surgery, in October the Syrinx could have gotten smaller again! Or of course it could have contiinued to grow and he may have started getting symptoms. We were advised that waiting could cause irreversible damage if they start

We were advised he would be able to go back to school 4 to 5 weeks after surgery, and that is expected to be a full recovery.

Good luck Helen. God speed to you.

5

thanks Melanie. I have read it all, and watched countless videos....I know all the sh*t things that are out there. Its a horrible thing. I am hoping to hear someone say they are now fantastic....that started out also with no symptoms before surgery...good luck in your recovery. love to you.

6

I was also told I would be back on my feet in 6wks. Yes I know where your coming from sometimes I think i was better before operation. But if it turned out he did get worse before operation and was irreversible !! You would be thinking same sort of thing should I have done it earlier ! It was appalling sending him home like they did though. People who recover well don't really post on here so there probably are more successful operations than not, you sort of come on here for reassurance and talk to people who are in or have been through what your son has. It must be torture for you and your wife watching your son with this. I have a daughter and son and I'm glad it's me and not them. But you have to be strong and get him through it. There are a few young people on here with this condition, again once they are better they don't need this sight. And I'm praying same for your son. X

7

So sorry to hear of the problems you've had ,I have chiari 1 and syrinx and had my op in 2005 my syrinx as shrunk and I'm left with the damage it had already done , I see what you say about no symptoms your son had not had up till then and should you have treatment ,mine didn't start giving me trouble until later life 40 I think half the trouble with hospital / doctors they haven't always come across it.as for coming home then having these problems I'm not surprised it's home now and no follow up only to a doctor that may not know what to look for I think the first week or so should be regular checks , I hope your son is on the mend and that it's nothing that can't be sorted out easily . Best wishes to you all

8

Dear  Squeegee,

I could not believe when reading your post, it must be terrible you both as parent seeing your child suffering. I just have my decompression 6 weeks ago, trust me, I did not want to have it done when my neurologist diagnosed me with CM1 2 years ago, as I was so scare, but as the time went on, I became worse and housebound, I was not able to deliver my normal human being role (bathing, bending, turning my head right or left) in the end even coughing/sneezing/moving position from sitting down to standing up I would be passing out for a minutes or two, So in the end I agreed to have the decompression, but I went to 2 neurosurgeons finally I was lucky that I was referred to the no 1 UK CM specialist, he is very knowledgeable and he has done more than 350 also majority of the people being operated by him had a succesful result and thanks GOD that is to include me.

The day after my operation, all the symptoms went, a part of pressure on my occipital - from the 1st - 5th day I was find, but the 6th day I was in agony, just like your son, I was vomitting, I could not take anything down, I have to be in canulla (drips) for two weeks, I just being discharged 2 weeks ago, I was at the hospital for over 4 weeks, but I am on my recovery now, as I experiences the tough CM symptoms prior to my operation, I really listen to what my neurosurgeon : ''don't do too much'' - ''the first 3 months just take it easy'' ''try to let others do do the house work'' after 2 months start mobilise, but take it easy'', he warned me, recovery period is between 5 -7 months, he also said, patients who listening to his advice have a tendency of doing excellently well and have their quality of life back (just like me), so what I am trying to say to you is, please BE POSITIVE, pray that your son will get better, get the neurologist to scan/mri again, but each individual are different, one of the nurse told me, there are few patients (perhaps like your son), have a terrible time during recovery period, but each day is getting better and better- keep praying. If you can but please check with your doctor, try to get vitamin/supplement thta have high antioxydant and omega 3 (fish oil) - make sure he drinks plenty of fluid...just reassure him that everything will be OK..make sure he does not doing any movement that bring the symptom (I have to make each of my movement carefully) I bought supplements and check with my neurologist - he said its good (I bought mangoesteen extract and grapeseed extract plus omega oil) they will have no side effect, I am now drug free (not even Ibuprofen or paracetemol).

I have a success story, but my recovery period up to 3 days ago was tough indeed, I am one of the lucky oneI suppose and I thanked God for that, you too should ask your heavenly father to help your son (I am unsure if you believe God or not) but wish to whoever (the highest being) that your SON will be OK - I Am SO SURE THAT HE IS GOING TO BE FINE!!!!

9

You came to the right place.. you need support..we are here for you. Vent..Ask questions..we are here

10

Thanks for your reply Waffle. All the best to you also....

11

Thanks b2wc. Its great to hear you are on the road to recovery, I hope if continues that way for you. Thankyou for your heart felt prayers

12

Thanks Gabby. I have googled looking for success stories, or stories from people that had no Symptoms before hand. I found 2 sites with some success stories, but the level of success still mostly seems to be based on comparison to before and after.....and mostly it is that things are "better". Those are the success stories! My kid had no symptoms. Success to us is that we get the same kid back.

It is often discussed this is a rare condition and surgery. For something so rare, there seems an amazing amount of negativity, reoperations, endless pain. I have only read one account anywhere that said they were 100% back to normal. One! 

Yes I have read stats on Medcial websites, and they are also all comparisons between "pain before" and "pain afterwards"...but to me stats are kind of like being on Trip Advisor and looking at the Management photos of a resort, and the looking at the visitor photos.

13

Thought Id update here.

Our son had developed Hydrocephalus, and this is why he was so sick when we took him back. He spent a few days on Steroids to give his body some time to adjust, but whilst his demeanour changed to positive, the next MRI 2 days later showed a different story and it had gotten worse.

The feelings of "what have we done" were overwhelming, as this was simply preventative surgery, for a Syrinx that next MRI may have gotten smaller....it may not have too! Remember, he was 100% assymptomatic before surgery.

As a side note, the Syrinx had actually gotten smaller already.

Anyway, our Neurosurgeon suggested he would like to try a Endoscopic third ventriculostomy, and it was 50/50 chance of success.

So my beautiful 14 yo went in for a second brain surgery. I am not religious, but found myself praying. The surgery was short (about 40mins). He came out of the surgery fine, and was great withing hours.

We spent another 3 days in hospital, and had another MRI. This MRI was inconclusive in regards to the ETV in that we couldnt see a black stripe indicating it was working, HOWEVER the fluid behind his sinuses had improved, and the top view also showed a small amount of improvement. We thought "well take that for now!"

We went home and were due for a new MRI a week later. The Neuro office called everyday to see how he was.

Our son had good days, and average days. He showed no real symptoms of the Hydro, but did vomit a couple of times rather randomly. Now the main thing was neck pain. We read that neck pain in the main complaint after Chari Surgery as they pull the muscles aside to access the area. We gave him some Valium, and it worked to comfort him and he was fine. So we just kept the valium up to him.

2 days before our scheduled MRI we noticed a fluid bubble at the site of the ETV surgery. When he laid down, it got bigger (about the size of 1/2 egg) and when he stood up it receeded. We were beside ourselves, and contacted the surgery. The Neuro rang us and advised us if there was no fluid leak (there wasnt) to come in the next day for an MRI, and that it sounded like the ETV may not have worked. That night we prepared ourselves and our son that we were likely going in to have a shunt (for life) put in. Our son said "well if I have to have that, lets get it done, and move on with a beautiful life".

I often read about people going thru things, and often thought that regardless how brave you are, you simply have no choice. But I have learnt its the attitude that makes a person brave.

Anyway, we went in and were met by the Neurosurgeons Registrar as he was away for alot of the day. He actually said he had seen this many times, and it may not be as bad as indciated. So we had the MRI. Our son wasnt well during all this (ONLY THE NECK PAIN), and was on valium....

Anyway, to our utter amazement ( and the Neurosurgeons), the MRI actually showed the Hydro was much better. He said the lump should correct over a period of time....but we were told if it doesnt go down in 30mins, or becomes hard, we need to go back in.

Our son has just had 3 amazing days since we left the hospital again. No neck pain at all, and has been up and about for every minute of every day. Its amazing what some positive news can bring! 

We arent out of the woods yet....not by a long way, but we are a long way from where we started.

Next MRI is in 2 weeks.......

 

14

Hi There, its so relieve to read your post also encouraging...!!! its the prayer I think you have done had helped you ..I personally had decompression 6 weeks ago, and so far I am free drug..I thanked our heavenly father who give my surgeon a gift to treat people like your son and I..they are the vessel to carry on to keep helping people, I do believe with positive attitude and asking to our creator both combine will have such a successful result - GOod luck

15

First, I'm so sorry to hear about all the difficulties you're son has had! I know this was posted a long time ago but I am wondering how your son is doing now and what are your thoughts of having the surgery done?

I just learned that my son has chiari malformation type 1 and a large syrinx as well. He is also asymptomatic and it was discovered during an MRI he had done for a developmental research study. We are going in the a second opinion but they are recommending surgery. I'm thankful we discovered it before he had symptoms but I'm also nervous about having surgery and then having symptoms start because of it. I'm looking for stories of others who had preventive surgery and their thoughts on afterwards.

I hope your son is doing much better!

16

hi there Maren. Sorry to hear of your similar predicament. The question ultimately is would we do it again if we knew what would have occured? The answer would be "yes".....but after much debate in our house.

Had he needed a shunt......Im not so sure. We didn't understand when we first talked about surgery that a shunt was for life, and it CAN change your perspective as they can and often need to be "revised"....ie replaced. II guess even if we were armed with that knowledge that there was a small chance still, we would have rolled the dice.

His Syrinx has shrunk, and that was the point of the surgery to start with. We just went a REALLY long way to go around it.

Zach today is happy and "normal"....he is exactly the same as he was before the Operation.

Our SURGEON was in Brisbane. Dr Martin Wood. I couldnt recommend him highly enough.

Good luck!!