My soon to be 7 year old son had decompression surgery December 5 of 2011. He had remarkable results and all symptoms stopped almost immediately after surgery. He did wonderfully until May of 2013. He started having headaches again and hands going numb. He was due for a follow up this coming December but they went ahead and got us in July 29, 2013. After he MRI the found he now has neck instability. With tissue build up also on the top vertebrae. They said he could have possibly 2 more surgery to remove the tissue build "like arthritis" I can not remember the name, and to stabilize the neck. We are still waiting to see the neurosurgeon because the one who did his initial surgery is retiring. Has anyone been through this. We just have no idea what to expect.
A lot of Chiari patients have ehlers danlos syndrome which can cause instability in the neck. Not sure about the tissue build-up. Is it scar tissue? Some patients build scar tissue around the dural graft which can cause problems.
I'm so sorry to hear about your son. Our son is 8. He also did very well after his first surgery and in a way, it makes things more tough because before that, for all the years of his life, he didn't knwo anything else but the pain, you know? Anyway, our son has somethign they called basilar invagination and also brain stem compression. I'm not sure if this is the same thign as instability. The chiari institute wants to do a cranial spinal fusion, fusing the head to the spine, 6-12 month recovery and we've heard if it doens't go perfectly, he'd be in much worse shape than he is now. Two other NYC neurosurgeons, including the one who did our son's surgery, would refrain from any more surgery and just manage the pain. I pray for a cure or another answer for this suffering, it's just not a normal existence for any child or adult. Let us know
my.daugjter had decomp
surgery.im.2009 and.now needs.a.craniocervical.fusion, c1.and.c2.fuswd.to.skull.wirh her.rib. TCI dnt.tell.us.recovery would be.6 to.12 months, but.we.are not.gg.thr.for.suegery. she.jad.beem.diagmosed.with.cm1, tc,.sciolosis,.basilar impression, retroflexed.odomtoid,.etc,.oh.and.most.recently, EDS. New.surgeon, new diagmosis
funny how that always.seems.to.work. also.has.cranial.settling, instability, same.thimg. sad thing is.pain associated.with.chiari.dsnt.seem to be manageable. thats.what.is.goimg.on in my.daugjters world. good.luck
Hi hun. After I read your post on Aidan, it was almost like a flash-back for me. I haven't followed your son's full story, but- I hope you get some other opinions AND to find out HOW they will go about doing his next surgery..... (I'm not sure, but I think we may have talked before) I had my acm decompression and about the same time length apart as Aidan, I was having issues,... but I do have arthritis in my neck.. also my bones are VERY unstable and I was dxd with "Basilar Invagination" (bent odontoid) and had to have major surgery due to BOTH issues... I ended up being fused from "skull to C-5" and was in a halo brace for 6 1\2 months,,BUT,, due to the instability in neck (ddd=degenerative disc disorder) The 1st surgery, they did bone graft from one hip to fuse in the neck AFTER,,, the odontoid procedure,then I was placed in the halo brace and rechecked and found that the fusion wasn't even taking place (holding) in my neck, almost like my body was rejecting it due to the severe instability in my neck.... So I had to go in and have another surgery and they took another graft from my other hip and a harvest of bone from part of 7th rib and split it ... now the hip bone was used on my neck was used with suture and some type of liquid protein (what the surgeon called liquid bone mineral???) my rib was split length-wise and placed on the bottom sides of my skull right and left side down to C-5 which left me "stuck" ..still in the halo brace for another 3 1/2 months...... I got the halo off and wish I would have done more research on other surgeries for my condition..... <3 Thats why when here about those with these issues I share SOME of my story and suggest they research other types of surgeries for unstable neck issues with chiari it is VERY IMPORTANT <3 and I say this from my heart..
Angyln- Do people with EDS have any other symptoms because I have not noticed any issues other than occasional knee pain which is described the same as my oldest son when he was having “growing pains”. They have not mentioned EDS but everyone that seems to have neck instabilit has EDS also. your thoughts?
There are several different types of EDS. While there are some symptoms that are the same, each type of EDS is different. For all types of EDS, diagnosis is based on medical and family histories and a physical exam.
If you think that you or someone you know may have any type of EDS, see a doctor who treats patients with connective tissue disorders.
The three most common types of EDS are:
- Hypermobile
- Classic
- Vascular
Hypermobile EDS
People with EDS hypermobile type (EDS-HT, previously EDS III) will have very loose joints and soft, velvety skin without unusual stretchiness or wounding / tearing. EDS-HT is the most common type of EDS. Common medical problems include:
- Joint dislocates or separates (called subluxation) with mild trauma
- Pain, which first appears as “growing pains” in young children, but can become more widespread and chronic in teens and older individuals. Pain is caused by the overall joint instability. Physical therapy and nonsteroidal anti-inflammatory medications are the main treatment for this pain. Surgery may give only temporary relief since the joints often become unstable again.
- Skin wounds may take more time to heal, and sometimes sutures from surgery will not hold a wound closed. Scars may stretch and widen. Stretch marks often occur.
- Bruising easily and nosebleeds and heavy periods may be common.
- May have dental issues, like high palate with dental crowding, poor enamel, multiple cavities, receding gums, bleeding gums, periodontal disease
- Jaw pain and dislocation are common and can lead to temporomandibular joint disorders (TMJ).
- Feeling dizzy, faint or “blacking out” when you stand up is common. This happens because the blood vessels do not react quickly enough to the shift in blood pressure when going from sitting to standing.
- Frequent headaches
- GI issues, such as acid reflux, irritable bowel syndrome (IBS) and gastroparesis
There is no genetic test for EDS-HT. The diagnosis is based on physical exam and medical and family histories. It is important that you find a doctor who has experience treating EDS-HT.
Classic EDS
The second most common type of EDS is the classic type (previously known as EDS type I and II). Classic EDS is similar to EDS-HT and patients will have:
- Loose joints
- Skin that is stretchy and has a soft velvety texture
- Fragile skin that bruises easily
- Poor wound healing and paper thin scars
Joint hypermobility can lead to early osteoarthritis and chronic pain. Some patients with classic EDS have heart issues that may include mitral valve prolapse and enlargement of the aortic root.
Diagnosis of classic EDS can be made based on clinical findings. Genetic testing is available for classic EDS (generally performed on a blood sample). There are two collagen genes that are known to cause classic EDS,COL5A1and COL5A2. You need only to have a change in one copy of one of those genes to have classic EDS.
Vascular EDS
Vascular EDS is quite rare and is the most serious type of EDS. This type of EDS is very different from EDS-HT and Classic EDS. A doctor who handles patients with EDS can tell if a person has vascular EDS.
Individuals with vascular EDS have:
- Distinct facial appearance with a thin nose, thin lips and hollow-appearing cheeks
- Thin “see-through” skin – can see blood vessels just beneath the skin in areas that are not typical
- Very loose small joints (fingers), but usually not the large joints (knees, elbows, hips, shoulders)
- Normal scar formation
- Skin that bruises easily. It can lead to brown iron deposits in the skin.
- High risk for life-threatening rupture of the intestine, uterus, or medium-sized arteries. Stroke and uterine rupture following delivery can occur.
Diagnosis for vascular EDS is made based on clinical exam, family history, and the presence of a change in one copy of the COL3A1 gene.
I hope this helps! Here is a link with more info!
Thanks Abby how do you find out if you have ehlers danios he’s not stretchy or flexible but he has more than “just chiari” plus it’s hard to find a doctor who understands chiari and treats children, we met one guy but he won’t take him on because he is a euro surgeon and our son is not his neurosurgical patient so he refers us back to him. Our ns wants him to see a neurologist though, not him, unless another surgery is warranted. Plus he views chiari Asa headache, pins and needles and that’s just about it so
try.dr henderson in md. hes an.EDS.specialjst. diagnosed.my.daugjter.right.away.and.after.4.years.with.her.neuro,.we switched over to him
Hi all I don’t think he has eds but no knows, he is definitely afflicted, could be “just chiari” and the results of the two surgical interventions. I can’t tell whether dr Henderson is pediatric but if you love him ill check him out thanks!