Grisel's Syndrome

Going back to the beginning of Lucy's neck problem I remember her waking with a sore throat and neck one morning, it became better throughout the day. Then a day later it was back again. I took her to the GP who diagnosed tonsilitis.

I wasn't fully happy with the diagnosis at the time because it was obvious that her neck was also bent, I just felt it was more. So I took her to A&E. They said it was torticollis and it would get better in a couple of days. It didn't.

Two weeks and a few more visits to A&E later and she was finally admitted after a CT scan had shown a rotary subluxation of the atlanto occipital joint. 

The consultant was adamant that she had been in an accident and that the subluxation was traumatic. I knew, however, that she hadn't and insisted that it was non-traumatic. Much later I had heard about Grizel's Syndrome and had a conversation by e-mail with an American Neurosurgeon who had suggested Grizel's Syndrome when I'd told him Lucy's story. 

Grizel's syndrome is a rare condition which usually affects children. It occurs more in children who already have lax ligaments, such as those with hypermobility syndrome. It is the subluxation of the atlanto axial joint after an infection such as tonsilitis, Group B streptococuss or similar ear, nose and throat infections. It can also occur after sugery for ENT. The symptoms are torticollis, cervical pain and symptoms related to infection. 

Lucy started with a subluxation of the atlanto occipital joint and was treated with muscle relaxants and soft collar, followed by physio therapy. Three months down the line there was no improvement and in fact the C1 and C2 had subluxed as well. Evidence shows that early treatment and intervention can prevent the situation from getting worse. 

Lucy was eventually given a manipulation and a traction collar, halo, was applied for three months (13 weeks). This did not work. As soon as the halo was removed the subluxation returned and it was discovered that a little piece of the C1 was missing. 

Ten months since the first signs of torticollis Lucy was was given a further manipulation followed by a fixation by Harm's method and the halo re-applied. 

It's been almost fourteen months now and Lucy is free from the halo and soft collar and appears relatively straight again. We will find out next week if her treatment has finally worked.

Her consultant still believes that her original subluxation was traumatic. 

From: Case Reports in Otolaryngology

Volume 2014 (2014), Article ID 703021

In conclusion, diagnosis of the Grisel’s syndrome is largely based on suspicion of the patient who has recently underwent surgery or history of infection in head and neck region. Early diagnosis of the atlantoaxial subluxation is required for careful clinical and radiological evaluation and consultation with relevant branches. Early intervention is critical for prognosis; conversely, delay in diagnosis can be dramatic. Therefore, clinicians should be aware of acute nontraumatic torticollis after recently applying the head and neck surgery 

Almost one week later

It's Lucy's 9th Birthday :)
There have been times when I've worried that she wouldn't make it :(
But she has and is now out of danger and hopefully will recover well. She's already doing really great, last night she slept without a collar for the first time in over a year!

This time she had stitches in her pin wounds, she didn't last time, which I don't really understand, but then the halo was fitted by different consultants each time, maybe they have their own methods?

She only had four pins second time where she had eight the last. So now we have just four wounds to heal and they are in the same place as four of the last ones which is good (otherwise we'd be dealing with six scars)

Pin wounds on the forehead

I can see the scars from the old pin wounds alongside the new ones, but I'm not sure how noticeable they are to others.

Here is a photo of her scar as it is now. I think it may be time to try out some lotions to approve it's appearance.

It seems she can look further to the right than the left, but look carefully and you can see her shoulder are more twisted, so in fact the movement is about the same. So far, it's as the Dr's expected, her movement is 50 per cent that of a normal child. There may be further improvement in time, but it's unlikely.

Lucy still walks, plays and moves as if she is still wearing the halo. I suppose this is normal.

We still have so much to be thankful for, our little girl is still with us and no longer in danger. We are so lucky.

Free

Today Lucy had her halo removed (again)
We arrived at the hospital at 8am, she went to theatre at around 9.15am and was back on the ward by 10.15am

She was drowsy for a while as the anaesthetic wore off. She'd been waking a little and asking if the halo had gone, then falling back to sleep. The she'd wake again and ask if the halo had gone. Finally when she woke up properly she expressed her joy at not having the halo anymore and said.

"At last, I'm FREE!"

Then she started crying, which made me cry.

She had a collar on but the consultant had told us that she needed to take it off as much as possible so she could strengthen her neck muscle which hadn't been used for two months. Two months! It's been over 12 months that she's been in a collar or halo, the consultant can't see past the current situation which was removing the halo after two months.

We took the collar off and Lucy exclaimed

"I'm normal again" 

Then she started crying again, which made me cry again.

Truly an emotional day.

One Down, One to Go

The last three days have been a complete emotional roller coaster. First, Lucy's operations were moved to different dates, then they were postponed altogether, then yesterday afternoon we had a call saying it was all back on and could we bring her in immediately.

So we arrived on the ward and had a nice little cubicle, Bed no1. We had a fairly good night despite it being a very noisy ward. So many crying children, it's difficult to ignore them.

This morning Lucy was a little panicked. We had told her that she was having the halo back and she'd been ok with it. She was ok with it last time, she dealt with it much better than us! However, this morning she decided she didn't want it back and was distressed.

By the time we took her down to be anaethetised for theatre she was really upset. She screamed when given the canula, and gagged and sobbed as the anaesthetic was administered. It was very distressing.

The manipulation and halo application went really well and took nearly an hour shorter than last time. We could see why when she got back...four screws instead of eight? Also, her neck is not very straight. Still it's just a temporary measure until the next big operation.

On Wednesday she will go back to the theatre, hopefully she'll be keeping her canula in until then so no big needles. The will open her neck and attempt to screw her bones in place along with a metal plate. To create a better fusion they will also had some bone graft, taken from her thigh or her skull. She is going to be hurting afterwards for sure.

Once they have completed the fusion they will decide whether or not to keep the halo on for a while. She could be coming home in five to ten days after the operation depending on her recovery.

During the time leading up to operations you feel so anxious, so worried that something may go wrong and that you will be leaving the hospital without your child. You don't want to think that way, but you know it's a possibility and it hurts to even think about it. Then while your child is recovering on the ward another little patient loses their battle and your heart starts pounding and the pain feels so real even though you know it's not your child. Then shortly afterwards, yet another patient loses their battle. And you cry, you don't even know these children, but you know it could so easily have been your child and you feel the pain of the parents an family, you feel their loss.

Having a sick child is so emotionally draining.

Almost Time

I have just received a call from the hospital asking me for Lucy's measurements for the halo.
She had a shower yesterday and complained she didn't like showers....just  a few more days and she won't be able to shower for weeks, maybe months.
I put on her t-shirt for her this morning, removing her cervical collar and pulling it gently over her head. Just a few more days and she won't be able to wear t-shirts.
Then I gave her a gentle cuddle and kiss. Just a few more days.......

I can't really explain how I'm really feeling right now. I was talking with Lucy's dad last night and we both feel the same. The main feeling is fear, we are both really scared that something is going to go wrong, we can't even mention the worst case scenario...over-reacting...maybe, but always a possibility. We are worried about how she will cope with it all next week, it's definitely going to be one of the worst weeks of her life and we don't want to see her suffer. We are worried that it may not work and all her suffering will be for nothing. Another over-reaction? Can you blame us, we thought the physio therapy would work but it didn't, we thought the manipulation would work but it didn't, we thought the halo would work, but it didn't, of course we are not going to have complete faith.

There are so many things that can go wrong. There is no promise of a full and complete recovery, she won't be able to move her neck very much at all afterwards. There is no way we can't go through with it, it's not just the twisting of her neck, or the pain, she can cope with that. It is getting worse and we can't allow that, the more it slips the closer it becomes to being fatal. Quite simply, if she doesn't get it fixed she could die.

I'm trying to keep myself busy with planning. We are all hoping to stay at the hospital with her until after her operations. Dad will stay by her side and I will stay in a family room with the other two little ones. They will have to have a few days off school, sorry Mr Gove I know you won't approve. If they can't accomodate us at the hospital then I will stay by Lucy's side and Dad will stay with the other two at his mum's. Simply so there is someone to look after them while he is at the hospital all day with me.

I have to leave things in order at home so the older two can look after themselves, so a weekend shop is in order and I'll have to make sure all the laundry is sorted.

So it's going to be a tense week coming up. Please keep Lucy in your thoughts and hopefully I'll be bringing her smiling face back home again as soon as possible.

Dear Mr S.

I am writing this letter to my daughter's paedatric neurosurgeon. I felt the need to write it down and share. Whether I actually send the letter to him I don't know yet.


Dear Mr S.

I called your office today to ask your secretary to give you a little reminder that you promised to call me last week. I know you are an incredibly busy man, but my daughter is eight years old and has been suffering for more than eight months and is very much in need of your care and attention. Also, I am a very stressed out mother who has barely slept for the last week.

Constantly on my mind is how my daughter's condition has become so bad. I really can't help thinking that if she had been treated properly and promptly in the beginning we would not now be facing surgery. For two weeks I brought her into the Accident and Emergency Department and was constantly told it was nothing to worry about and it would fix it self. It didn't and she was offered a CT scan of her neck. The scan showed a subluxation of the atlanto occipital joint and she was admitted to your ward.

For a week she was treated with muscle relaxants while we waited for a traction bed for her. The bed arrived but the decision was made not to go ahead with traction and to try physio therapy instead.

I was happy to go along with this. Of course, the less distress to my daughter the better and if it could be put right by physio therapy then I was happy to bring her to the hospital twice a week.

It soon became apparent, however, that the physio therapy was not working. Her nurse was really concerned but had to push for another CT scan. After the scan the physio therapy was stopped, we were not told why, but we were then left in limbo. For weeks I waited for a call to come and see you and find out what was going on, what the next step was going to be. I was told that you were busy, that there were no appointments in your clinic, that I had to wait. 

I became impatient and called the Patient Liason team at the hospital who finally managed to get me a clinic appointment. First my girl had to have another CT scan. At the appointment we were told she was going to be admitted for a manipulation as the subluxation had become worse and it needing correcting. So my daughter was taken to theatre and the manipulation performed and a halo traction brace fitted.

She wore the halo for thirteen weeks and on removal we believed that she would now be fixed. This was not the case, her neck began to slip back the very same day. At her halo removal check it was obvious that something was wrong so I was told she would need another CT scan. After the scan we were left in limbo again, not knowing what was going to happen next. I called your secretary who then managed to fit me in for an appointment.
The scan showed that a piece of my daughter's bone had deteriorated and it was unlikely that it would hold her neck up without further intervention. 

Then, well, we are still waiting to hear from you. 

The questions going around in my mind are, how, when and why has her bone deteriorated? Was it in the beginning when her neck was so twisted the bones were grinding against each other? Did it happen while she was having physio therapy trying to make the bones go back into place? Maybe it happened during the manipulation? Why has it only just been detected, did it happen very recently? My daughter has had many scans and xrays (I have the dates and times of every one written down) why wasn't it noticed sooner? 

More importantly, what are you going to do for her now, and how much longer will we be waiting.

Yours Sincerely,

A Distressed Mum of a Beautiful, Brave Little Girl who deserves to be treated better.

This post will be duplicated on my other blog Raisiebay.

Not The Best News

Today we went to the hospital to get the results from Lucy's CT scan.
It wasn't good news. The bones have definitely slipped again, but much worse is that the bone has deteriorated quite a bit and no longer fits in with the rest of the spine so it will keep on slipping.

We have been told that Lucy will require an operation and possibly the halo will be returned.

Lucy's consultant will be discussing the CT scan with neurosurgeons at GOSH tomorrow and then will decide what the next step will be.

I don't have a picture of Lucy but I found this photo of scan which looks very similar. You can see the two bones at the top of the spine. One is square, the other is triangular. They should both be square. The one on the left of the picture has deteriorated changing the shape. This means the skull does not stay in position and tilts to the side.

photo credit: http://neuroradiologyonthenet.blogspot.co.uk/

A possible cause could be Rheumatoid Arthritis. Something I've suspected before.

I will be asking lots of questions, and pressing for our appointment with the rhuematologist.

It could be that the bone deteriorated because it was rubbing against the adjoining bones when they slipped out of place the first time. Will we ever know?

Cock Robin

We had a hospital appointment today to see how Lucy was getting on after having her halo removed.
The Doctor straight away noticed that her neck was not straight. He asked several times when the halo was removed, I'm not sure why, it was two weeks ago, it seemed like he didn't believe me. He said that she had a xray on 3rd March and her neck was straight. I said 'yes, that was two weeks ago, when she had her halo removed.'

He took off her collar and asked her to look to the left, which she did (and grimaced). The he asked her to look to the right, which she couldn't do (and grimaced even more). He asked her if she was in pain, she replied 'a little bit' She hates admitting to being in pain for some reason.

Then he said he would need to speak to her consultant, but was certain that she needed a scan. So we waited around for a while. Then he got back to us and said that they couldn't arrange a scan until tomorrow so could we come back. On the way home I had a call to say we had to come in Thursday instead.

So, still no answers. We really don't know what is going on.

I have two theories.

1. The muscles are too week to hold her head up straight and that's what is making her lean. Perhaps if they can strengthen her muscles then she will be recovered.
I worry that maybe her muscles were lax in the first time and that's why the bones subluxed. In which case there may not be a way to strengthen them. I'm not sure what the answer would be if that was the case.

2. Her joints are starting to sublux again. In which case then they will need to find another way to fix her, if that's possible. It also means the last three months of enduring the halo have all been for nothing!
It also brings up the question, why are her joints subluxing all the time?

One thing that the consultant has mentioned in the past is fusion. The joints in her neck would be screwed into place to stop them from moving.

I hope it doesn't come to that, but I do want her fixed.

Rotary Subluxation of the Atlanto Occipital Joint

Lucy was first admitted to hospital in August. She had been diagnosed as having a rotary subluxation of the atlanto occipital joint. Now, I had no idea what this meant at first, other than it was a bone in her neck that had moved.

The atlanto-occipital joint is the top join of the cervical spine which connects to the skull. It is very rare for this joint to move, it is slightly more common for the atlanto axial joint to move, especially when involved in an accident. My little girl did not have an accident, the bone moved by itself, probably due to lax muscle control holding it in place. I'm still not completely sure why it moved, neither are the doctors, but I do hope to find out.

First the doctors talked about manipulation, then they talked about traction. They spent five days locating a traction bed for Lucy, then changed their minds. In the end we were sent home with a Miami J collar and regular physio appointments. The only treatment she had been given in hospital was muscle relaxants in the hope that her neck would just spring back.

We came home and really nothing had changed except that with the collar on the pain was not so bad and Lucy was able to eat and drink again. We went for physio therapy every week. There was no improvement but we managed to keep her neck from completely stiffening up.

Thirteen weeks after we had first taken Lucy to the hospital she was given another CT scan. We were told to stop physio therapy and keep the collar on all the time. We then waited and waited to be seen by the hospital. I had to call and make a fuss before they finally found time to see us.

Lucy was given another scan and then admitted for a manipulation. We were told that she may have a Halo Collar fitted while in the theatre. It wasn't until a few weeks later that we learnt that the joint had continue to move out of position, from the first scan to the last it had moved just over 2 mm, in total it had moved 4 mm.

When I went to see Lucy after she came out of theatre she was very distressed and horrified by the halo. It really is an awful contraption, but it is something you get used to. Lucy was amazing, her initial distress soon passed and she has just got on with things. She was in a lot of pain for the first couple of days, and the anaesthetic had made her sick, but she was soon up and walking about, she has been so brave.

Day 1 Of Halo wearing

We are currently starting week four of halo wearing. Just two more weeks to go!