Hi-
My 15-year old son has been recently diagnosed with chiari I and syringomyelia. He is not having any neurological symptoms other than bad headaches periodically (which could be totally unrelated). His syringomyelia is however pretty large from what the doctors are telling us.....9 mm in width and he has developed scoliosis, which actually led to the chiari diagnosis. Doctors are suggesting surgery (without duraplasty) because of the size of the spinal cyst. I have done a lot of reading on the subject and I actually feel okay with the surgery itself, however, I have been a little disturbed by many things that I have read about people suffering from things such as occipital neuralgia and memory loss after having the surgery. I do not want to put off surgery if the cyst is putting him in danger for future neurological issues, but do not want to expose him to these dreadful post-operative possibilities either.....especially since he is pretty much asymptomatic right now (excluding the scoliosis). We will be seeing another neurosurgeon this week for a second opinion and I have jotted down many questions for him, but I would really like to get opinions from people who have had the surgery. Thanks so much. I appreciate any feedback I can get.
Surgery is usually recommended when a syrinx is present due to the risk of paralysis. I did not have a syrinx but still opted for surgery and I am very pleased with the results! Also keep in mind the younger patients seem to have a lot better success as they have had less time for permanent nerve damage to take hold.
I would question him on why he isn't doing duraplasty however. While it does have a slightly higher risk of post-op complications like csf leaks and such it also makes it more likely that a second surgery will be needed especially when a syrinx is present. When csf flow is blocked it can cause scarring and adhesions to form under the dura and make a decompression without duraplasty unsuccessful.
Huge hugs I am so sorry you and your son are having to consider any of these options ,as a Mum I can empathise with you , I am also sorry I have no words of wisdom to share just want to acknowledge your post and hope that some of the lovely people here can help you with information again huge hugs and sending you positive thoughts :)
Please do some research and come to your own conclusions. In my practice, Chiari + >5mm syrinx = surgery. Your son also has scoliosis and head-aches. I think every neurosurgeon would recommend surgery. So, then you get down to the exact surgery recommendation.
Here's the most recent meta-analysis(not level 1 data, just a review of previously published reports).
Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis.
Abstract
OBJECT:
Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children.
METHODS:
The authors searched Medline-Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000-2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years.
RESULTS:
Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08-0.69) and a higher rate of cerebrospinal fluid-related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53-23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95-1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91-2.25) were noted between PFDD and PFD.
CONCLUSIONS:
Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid-related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.
Many neurosurgeons started using that paper as a reason to back away from duraplasty because there was no statistical significance in outcome between those with duraplasty and those without.
The reality(at least to me) is that there were inadequate patient volumes to get a final answer.
Look at the syrinx outcome specifically. 56% improved without duraplasty. 87% improved with duraplasty. Reverse those numbers for failure rates. By entering the dura, your failure rate drops from 44% to 13%!!
However, even though the failure rate was 3x higher in the bony decompression alone patient population, because the patient volumes weren't high enough, there was no statistical significance to that finding. Does that make it wrong? In my opinion, it means we have inadequate data and need further study. But given my choice, I'd choose the treatment that has 1/3 the failure rate for patients with a syrinx.
I hope the logic is understood(this is a conversation I have several times a week). But that is the reason I recommend opening the dura in all my non-syndromic patients and feel it is inappropriate to even offer anything else in patients with syrinxes.
Quoted for accuracy:
Anglyn said:
Surgery is usually recommended when a syrinx is present due to the risk of paralysis. I did not have a syrinx but still opted for surgery and I am very pleased with the results! Also keep in mind the younger patients seem to have a lot better success as they have had less time for permanent nerve damage to take hold.
I would question him on why he isn't doing duraplasty however. While it does have a slightly higher risk of post-op complications like csf leaks and such it also makes it more likely that a second surgery will be needed especially when a syrinx is present. When csf flow is blocked it can cause scarring and adhesions to form under the dura and make a decompression without duraplasty unsuccessful.
Mom, the below advice is great advice. Your son has a sizable syrinx which, along with the Chiari, could cause the memory loss and neuralgia. Many of us acquire these symptoms before surgery. I did. Since my decompression both of these have improved for me. I also had the adhesions that Anglyn mentioned, and had I not had them cut away after my dura was opened I feel that I would have a lot more on my plate, symptom wise, post op. every surgeon has their own rate of infection and CSF leaks, some are higher than others- so if the surgeon doesn’t like performing the duraplasty because of his rate of complication with these- it’s more telling of the surgeon, not the procedure. Keep going, researching, and meeting surgeons until you and your son feel comfortable with your choices. It has to feel right.
Also, it it very wise to look into Ehlers Danlos Syndrome. MANY chiarians have EDS, which causes ligaments to be lax and unstable and ultimately causes cranio cervical instabilities, including basilar invagination. The cranio cervical instabilities require further surgical treatment. Many of us are dealing with this. This should be ruled out.
A great video that ties it together can viewed at
CSFinfo.org
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Look for EDS and cranio cervical instability, or the complex Chiari patient
Other diagnosis / issues that like to tag along with Chiari are:
Tethered cord
Sleep apnea
Dysautonomia (dinet.org)
Vitamin D deficiency
Magnesium deficiency
B deficiencies
I know this is a lot, and not trying to overwhelm you. If your son’s case/situation allows you time to fully investigate everything that may come up then that is a true positive. How are you holding up? How is your son feeling about this?
Jenn