Chiari Malformation

We found out that my 12 year old daughter has scoliosis in May of 2009. She had taken the the Scoliscore test and scored 65 out of 200, which is on the low end of what her progression should be. We took her back to the scoli doctor in September, her curve progressed another 10 degrees in 4 months time. Her doctor was pretty shocked the she progressed so much in that short of time considering what her score was. He suggested that we put her in Lyon brace for 18 hours a day and he ordered an MRI. She had her MRI in beginning of November and we saw him at the end of November. Her curve increased again and the MRI results said she has Chiari 1 Malformation. She no Chiari symptoms, not even one and that surprised him also. He said we needed to see a pediatric neurologist. So off to the neurologist we went. He ordered another MRI and we waited for his phone call. Once he received the test results he contacted her scoli doctor and they came to the decision that she needed to have surgery soon. Doctor said she has minimal spinal fluid in her spine because of this and this is why her curves are progressing so much. We are going there on Monday to discuss the surgery and to set things up. I have been reading what I can find about Chairi but I was hoping someone on here would be able to tell me what to expect. What was the surgery like, how long was it, what is the recovery like, are there any questions you wanted to ask but had forgotten or thought about later? Any advice would be great appreciated.

Hi there,

I am sorry to hear of your daughter's recent diagnosis. I am sure this is a blow on top of rapidly-progressing scoliosis. I have no personal experience with Chiari, but I know there are some parents here whose kids had Chiari surgery. You might be able to find some of their old posts by searching for Chiari. Also, Carmell is an absoutely fantastic resource on this, if she pops in and sees your post. If you search her posts she has commented on Chiari before, I think.

Good luck. I am sure your head is spinning now, but I am sure you will learn a lot more on Monday. Please let us know how the appointment goes.

wigglesgiggles,

Taking Gayle's suggestion, here is what a search on "Carmell chiari" returns...

http://www.scoliosis.org/forum/searc...archid=2310348

Good luck.

Thank you both for your replies. I did look to the posts you suggested and did learn a couple more things. Boy, do I have a ton of questions for the surgeon tomorrow.

Have you had surgery yet? I found this website on Chiari Malformation and Syringomyelia helpful www.asap.org . I Private Messaged you my email as this times out quickly. Also I am not on this site much anymore. If you want to email or PM me that is easiest for me. My daughter was diagnosed with scoliosis and wore a brace and still had rapid progression. Nine months later, after a third opinion, the 3rd pediatric orthopedic surgeon had an MRI done with results of Chiari I. He referred us to a top pediatric neurosurgeon who recommended surgery due to multiple large syrinx, spinal fluid pockets in her spinal cord due to the restriction of spinal fluid flow from the Chiari. She had no Chiari symptoms either. She had decompression surgery within months of diagnosis as we waited maximum time doctor allowed to fall over winter break. She had 2 weeks off from school so she only missed 2 weeks of school after the surgery because we were not sure how long recovery would take (was about a month). She went back after break. She was able to return to swimming within a couple months. She still had rapid progression on her curve and had spinal fusion surgery 9 months later (as also maximum doctor wanted to wait and wanted to get in summer and earliest summer could get). She went back to school in minimal time, believe it was exactly 4 weeks, with restrictions, as she didn't want to miss first day of school, and the doctor gave the okay. First surgery (Chiari) nausea due to anesthesia and area of surgery the worst, second surgery the stiffness and pain was worse, but was fine after a week and a half to 2 weeks as far as pain. She had quick recovery within 9 months fully active doing all she did before.

Hi, my daughter was diagnosed with scoliosis in January 2008 and her orthopaedic surgeon sent her for an MRI which revealed a chiari 1 malformation and whole of cord syrinx. She had no other symptoms. Within 1 week we saw the neurosurgeon and within 5 weeks she had neurosurgery to repair the chiari. I understand the anxiety of this time, I spent all my waking hours thinking about it. The surgery went well and my daughter was returned to the ward in 3 1/2 hours. Her main problems post op were severe nausea and vomiting, especially when she moved and severe pain mostly 7and 8 on a pain score of 1-10. After 5 days her narcotic analgesics were ceased and she was off all oral medication within 2 weeks. She was in hospital for 8 days. My daughter would get neck spasms and of course she was reluctant to turn her head, She was back at school after 3 1/2 weeks. We were concerned that she may have some neurological defecit from the surgery but have not been able to notice any differences.

We had been told that the surgery may stop the progress of the scoliosis or in rare cases even return to a normal spine if done before the age of 10 years. My daughter had the surgery 3 weeks before turning 10 and of course we were very hopeful she would make a full recovery.

Unfortunately this was not to be. Her curve continued to increase at almost 1 degree a month and in June last year she had a T3-L2 spinal fusion for a 73 degree curve. We were able to see her after about 4 hours and she again had a good recovery. Nausea and vomiting were not a problem (they used different anaesthetic agents but did say the vomiting was much more common with neurosurgery) and her pain was much easier to manage mostly 3-6 on the pain scale. She was discharged on day 6, ceased oral analgesics by 2 1/2 weeks and had 4 weeks off school, 1 week of half days then full days. She did complain of ongoing hip pain at night which seemed to be relieved by hot packs. We are coming up to 9 months when all restrictions of activities are removed and my daughter is looking forward to resuming sport.

We wish you and your family all the best through this difficult time and a rapid recovery for your daughter. Please feel free to private message me if you wish. I would have loved to have spoken with other parents before my daughters surgery because despite being a registered nurse for nearly 30 years had never previously heard of the condition.

My now 15 year old son was diagnosed with Scoliosis and Kyphosis in 2008 and was monitored for a year. He was dx with Chiari Malformation and Syringomelia in January 2009. He had his Chiari decompression a month later. He had no symptoms that we recognized - it was discovered on MRI. (They checked for tethered cord, and renal issues too, because he was born with imperforate anus, they wanted to make sure he didn't have this as part of the VATERL syndrome)

His Chiari surgery was particularly painful because as a defensive football player, he had a very muscular neck. The surgeon warned us that it would be painful recovery for him. I talked to several nurses and his pain was not typical at all of what they see there - so anyone reading this, don't worry, ours was not a typical case!

Anyway, he had migraine like headache pain and was on narcotic pain meds around the clock for 3 weeks. He wanted to not move, and keep the room dark. It became much easier when he added the valium to his narcotic. He also ended up with impacted bowels because 1) he was so immobile, 2) he couldn't strain, 3) all the pain meds and 4) he was born with imperforate anus (all repaired during his first year), but still has some issues from time to time. So we had to go to the ER for that (ugh)

When the tide turned, and the headaches went away, he was like a new kid and went pretty quickly to normal activities... within weeks it seemed. At last check, his "flow rate" is all good and he was gaining strength in his right side (he didn't even realize how weak he was on that side)

We homeschool so we were able to ease into school, and/or do school laying down, at his own pace, so we did not have any stress over that aspect.

He started playing baseball and got in a season this spring, playing catcher (which I am not convinced is a "non-contact" position, and is a source of contention in my home LOL).

For almost 9 months, his scoliosis quit progressing and we thought for a while we were one of the lucky few. But, it started progressing again, about 2 degrees a month, and it had reached a 50 degree curve. So, we had to get the fusion and we just had that done 3 weeks ago. They did something else, that involved cutting an area of bone that I've forgotten the name of.

Oh my goodness, compared to the Chiari surgery, the fusion is a piece of cake! He has never put his pain higher than a 3. He was off ALL pain meds at the end of the first week, even OTC meds.

He can walk for quite a while. He wants to run, swim, play ball. I can tell you the difficult thing this time around will be keeping him down - he feels like he can do more than he can do.

He had some blood drainage. It was profuse at one point, and we went to the ER, but there was no infection or spinal fluid so they had him lay back down last week (that was hard on him) and keep compression bandage on it. They put him on antibiotics just in case. That seems to have cleared up.

He did have to receive his own blood back during the surgery (they used cell capture). For a while he looked very pale, and kept feeling dizzy upon standing, so I have been giving him iron supplements and children's multivitamins and that seems to have helped.

His incision is about 16", I don't know which vertebre were fused (I was told but I was in the pre-surgery fog and forgot) but will ask this Friday for our follow up. He has internal dissolving stitches and surgical glue externally. They chose to use titanium rods and screws because he will need future MRIs to monitor his Chiari. His neurosurgeon was on standby during the surgery, and they also used a brainwave monitor.

The surgeon said he had to use a torque wrench to pull him straight (but he might have been kidding LOL)

He is SO straight - it is unreal. He gained 2 inches of height. I will find out next visit what his degree is now.

All in all we are pleased with how things are going. We absolutely love his surgeons - Dr. Barun Brahma (neurosurgeon) and Dr. Michael Schmitz (orthosurgeon) at Children's Healthcare of Atlanta at Scottish Rite

My son was diagnosed at age 4 with Chiari,holocord syringomyelia, scoliosis..he had chiari decompression in 6/05 & revision in 5/06, His syrinx never disolved in 6/09 he had a syringopleural shunt placed and syrinx is almost totally resolved. My son is 11 years old now. His scoliosis is C curve and over the years he has been boston braced off and on, it has slowly increased until recently he had growth spurt and the curve is around 50 :(, they told me years back that with the chiari/syrinx diagnosis that his curve was going to increase no matter what. Within the last 2 weeks we saw 2 ortho doctors who both said its time for a posterior fusion... I dont know all the details yet but it will be below where his shunt was placed.