What is Arnold Chiari Malformation and Syringomyelia?
Sat, 09/12/2009 - 12:55 | 
Chris 
What is Arnold Chiari Malformation? (Type 1)
Chiari Malformation Type I describes a condition where the cerebellar tonsils at the back of the brain descend or herniate below the base of the skull through the large opening called the Foramen Magnum into the spinal area. This results in compression of the brain stem, surrounding nerves and spinal cord, and blocks the normal flow of cerebrospinal fluid throughout the skull.
This in turn causes symptoms such as severe headaches, muscle weakness and general muscle pain and weakness amongst many other debilitating symptoms in sufferers. Fortunately, some patients can be asymptomatic for many years or indeed for their entire life span. Due to the nature and number of symptoms, this can often be mis-diagnosed, and unfortunately often for years. Symptoms can occur at any stage in life, some patients may not even notice the onset, the meaning, or even know about Chiari Malformation as the symptoms are so wide spread and present differently in every patient.
There are other types of Chiari Malformation, however, I only have personal experience of my own Type 1 variant.
Chiari is thought to affect 1 in 1000 people in the USA. No stats have been released for the UK, however if you transpose those figures tothe 6.14 million people in the UK, it suggests that there are 61400 cases in the UK currently either diagnosed or undiagnosed.
What is Syringomyelia?
Syringomyelia is a condition where a Syrinx (a fluid filled cyst) develops inside the spinal column. Patients can have more than one Syrinx, they can appear in any part of the spine and vary greatly in size. The Syrinx can enlarge over time causing the spinal cord to expand and stretch nerve tissue. The syrinx, if left can cause permanent nerve damage and maybe even lead to paralysis. They can arise as a result of Chiari malformation or can appear as a result of direct trauma.
Other links that you may find interesting:
Glossary of Terms
Chiari and Syringomyelia Symptoms
How I explained it to my young kids
Chiari Forum in the UK
Where do I start, well I will try to explain.
My sister was diagnosed with an Arnold Chiari malformation around 10 years ago; she was eventually referred to a surgeon at Oxford for surgery, when she went in for the surgery she was told she would be in hospital for around 6 weeks, in the event it turned out to be 3 months!
The surgeon said that the operation had been successful; the problem was that whilst in the hospital post surgery she developed pneumonia and eventually they transferred her from that hospital to another one in Oxford (Churchill). Eventually she recovered from the pneumonia but as she had been fairly well bed ridden for close on 3 months she couldn’t walk. Anyway the end result was that she was discharged and was supposed to have physiotherapy back at her home hospital in Northampton, this was never arranged.
Since the operation she has developed all sorts of problems over the years, chronic pain syndrome, balance problems, eyesight problems, the list goes on and on. The pain specialists at Northampton prescribed a stronger and stronger cocktail of pain killers (including a number of opiates). Eventually she ended up being so dosed up that she was barely coherent. Finally one day she collapsed and was taken in to Northampton General Hospital, whilst in there they gave her Oralmorph and unfortunately she had a bad reaction and went in to respiratory arrest and ended up in a coma in ITU. After a week in a coma she eventually came round and got discharged. Then she was sent (in a wheelchair) to a rehab centre where she stayed for three months.
Now she is back home but her symptoms seem to be getting worse, her balance is shot, her eyesight has gone haywire and she suffers from weakness in her legs and hands.
After a visit to hospital in May because of a fall at home she had a scan of her brain and neck, the consultant decided that he wasn’t happy with what he could see and referred her back to Oxford to see the original consultant who operated for the Arnold Chiari Malformation, this was in May of this year. My Sister has been waiting for an appointment to come through from Oxford since then but there has been no correspondence from Oxford, she has phoned the consultants secretary and been told that Dr Aziz the original consultant no longer carries out this procedure and she needs to see someone else. Apparently this new consultant’s secretary is away on holiday and nobody knows what is going on.
In the past week my sister has had four falls and in the early hours of Sunday she fell trying to get out of bed and was found collapsed on the floor, she was incoherent and didn’t know where she was and her eyes kept rolling back. An ambulance was called and she is now back in Northampton Hospital undergoing tests, they suspected a stroke!
The thing is I am convinced that everything that has been happening to my sister over the last ten years is all as a direct result of the Arnold Chiari malformation, many of her symptoms match up to symptoms for it. As a family we are at our wits end, no one from Oxford will make an appointment for her to see someone and Northampton Hospital don’t know anything about Chiari.
My question is can anyone offer any advice on how to get her to a specialist, the family are willing to pay if it cant be done on the NHS, she never had any follow up appointments with the specialist after the operation 10 years ago!
If anyone knows who the best consultants are in this field I would love to know, we can then start the process of trying to get her an appointment.
Sorry if I rambled on a bit here.
dr.dan heffez in milwaukee, wi. did three surgerys for my son this year. decompression, tethered cord, and vp shunt excellent doctor
Hi there, I am so sorry to hear what your sister has been throug and of course yourself and your family as well. I couldn't see any other replies so thought I'd share my experience with you.
I was diagnosed with chiari in august and a syrinx in sept following emergency admissions to hospital. I was referred to Mr Dorward who practises at the royal free and also at the wellington - both in london. I have private healthcare with work so was able to see him within a month for the decompression surgery. On Monday I will be 4 weeks post op. It's a tough op and recovery is quite slow but I can't speak highly enough of the care I received. Might be worth a call?
I hope this helps and your sister makes a full recovery.
Sarah
My name is Marlene and I am from South Africa.
For years and years I was misdiagnosed and treated for all other kinds of illnesses, before, in 2001, I was daignosed with ACM and had a decompression operation during 2001.
All went well for a few years and then the headaches, toghether with other life threatening symptoms started again. I was told that the pressure on my brain was 26.8 in stead of the normal between 9 - 11. A shunt was installed in my brain to relieve the pressure. It was better for about 3 months and then the headaches started again.
During 2009 I was informed that the pressure, even though I had a shunt in the brain, was again 28.3 and a second shunt was installed in my spine. However, this shunt now needs to be replaced, because it is not draining properly. Everytime this happens, I have a 50% chance of being paralysed. I am currently going blind as a result and escpecially my right eye is effected.
I was told by the doctors that given that ACM is an incurable illness, that there is not a lot they can do for me, but to try and relieve the symptoms as far as possible. I was told to accept my condition and try and make the best of each day, because the older I get, the worse the symptoms will be getting.
How do I feel? At first I was devastated. Why me? Howcome I have to live with the fear of being paralysed, with a sword of death over me? Now, I am thankful for every day that I wake up. I push myself to the limits, trying to find ways to overcome the pain. At this stage, my right kidney and my liver is packing up because of the pain medication that I use, but I am still alive. I have a wonderfull husband who is supporting me all the way.
Being a Christian has taught me to be thankfull for every day and to live every day as if it is my last day. I had to open my eyes and see that there are people out there that are worse off than me! That are dying!
At some stage I wished that I rather had brain cancer. I know how to fight that and what the outcome for me would be! But God chose different for me.
I would like to make contact with anyone who has ACM and become e-mail friens.
Regards,
Marlene
carel@carmagraphics.com