I have a 15yo daughter, diagnosed 12/15 with 3-4 mm cerebellar tonsillar ectopia, after having 24/7 frontal headaches beginning Thanksgiving week. NO med helped -- Naproxen 500, hydrocodone. Only Toradol pain shot gave relief, but only for about 6 hrs at a time. We are in Knoxville, TN. Neurosurgeon here -- Jennifer Savage -- does quite a bit of chiari surgeries, but said her best results come in patients with classic chiari headaches (back of head) and wanted to try conservative treatment with the neurologist first because surgery may not help the frontal headaches. No tethered cord or syrinx on spine MRI, btw. Pediatric neurologist was awful (wouldn't even look at the scans himself) -- MY personal neurologist agreed to see her. He feels ectopia may be even smaller after reviewing the scans.
She started Elavil 10 mg every night and has stabilized somewhat -- has mostly pain-free days for the last month with headaches occasionally spiking to 2-3 on the pain scale most days. Returned to school full time (was on half days) and playing softball, etc. She did have some weakness in her legs and hands right after starting Elavil, but the leg weakness resolved after a couple of weeks. Hand weakness is sporadic, but only when she's taking notes in class. The biggest side effect is fatigue for her.
The neurologist feels she may have had an undiagnosed concussion (she hit her chin diving for a ball in a softball tournament one month before the constant headaches started) and is now in a chronic daily headache cycle and should be doing better in a few months with medical management. I know a lot of neurologists say this. He is a great neuro (literally saved me a few years ago), BUT he said he has lots of patients who have ectopias on MRI who don't have chiari and it's just the way they're made. He said he has only a few who are true chiari patients. He is very serious and listens to every thing we tell him, takes it all in and explains everything thoroughly, so he's not blowing us off, but I'm concerned that he isn't taking the ectopia seriously enough. I would love for him to be right, but the internet and FB groups scare me about all neurologists being ignorant about chiari. Oh, and she does do headache PT and it does help her -- electro acupuncture takes all the pain away for hours after.
My question is: Does Dr. Trumble allow you to send records, symptom history, scans, etc and give an opinion via email or snail mail? I hear wonderful things about him and, while, my daughter is doing OKAY, she's still not 100%. If she needs a decompression, then, of course, we want to do that. You never want your kid to have to have surgery like this, but I also don't want her to constantly suffer. She is a very athletic, social child and every day I worry how she's going to feel and I see her working hard to be normal. We are so far away from Dr. Trumble, but in the day and age of Skype and email, I wondered if he does review things for patients in other states. (I wasn't sure if he sees kids or not)
Sorry so long!
Why don't you ring his office and ask?
Yes, we are always happy to help and we usually recommend sending a CD-ROM of the images, with a 1 page summary of treatment and symptoms(your above note is perfect).
I am a fellowship trained pediatric neurosurgeon so I see many more kids than adults.
What Dr. Savage stated is born out by the literature. Last year, there was a Chiari Severity Index published:
Neurosurgery. 2015 Mar;76(3):279-85; discussion 285. doi: 10.1227/NEU.0000000000000608.
The Chiari Severity Index: a preoperative grading system for Chiari malformation type 1.
Abstract
BACKGROUND:
To develop evidence-based treatment guidelines for Chiari malformation type 1 (CM-1), preoperative prognostic indices capable of stratifying patients for comparative trials are needed.
OBJECTIVE:
To develop a preoperative Chiari Severity Index (CSI) integrating the clinical and neuroimaging features most predictive of long-term patient-defined improvement in quality of life (QOL) after CM-1 surgery.
METHODS:
We recorded preoperative clinical (eg, headaches, myelopathic symptoms) and neuroimaging (eg, syrinx size, tonsillar descent) characteristics. Brief follow-up surveys were administered to assess overall patient-defined improvement in QOL. We used sequential sequestration to develop clinical and neuroimaging grading systems and conjunctive consolidation to integrate these indices to form the CSI. We evaluated statistical significance using the Cochran-Armitage test and discrimination using the C statistic.
RESULTS:
Our sample included 158 patients. Sequential sequestration identified headache characteristics and myelopathic symptoms as the most impactful clinical parameters, producing a clinical grading system with improvement rates ranging from 81% (grade 1) to 58% (grade 3) (P = .01). Based on sequential sequestration, the neuroimaging grading system included only the presence (55% improvement) or absence (74% improvement) of a syrinx ≥6 mm (P = .049). Integrating the clinical and neuroimaging indices, improvement rates for the CSI ranged from 83% (grade 1) to 45% (grade 3) (P = .002). The combined CSI had moderately better discrimination (c = 0.66) than the clinical (c = 0.62) or neuroimaging (c = 0.58) systems alone.
CONCLUSION:
Integrating clinical and neuroimaging characteristics, the CSI is a novel tool that predicts patient-defined improvement after CM-1 surgery. The CSI may aid preoperative counseling and stratify patients in comparative effectiveness trials.
In that paper, there was a >80% chance of improvement for sub-occipital head-aches("classic Chiari"), about 70% with frontal head-aches, and <60% with myelopathy(possible permanent damage to the brain stem).
For the Chiari work-up, you want to make sure you have achieved maximal medical management(it is a lot easier to take medicine than have an operation). I always try to get an entire spine MRI(20% of patients will have a syrinx or a tethered cord, some other anatomical issue that may push you in the direction of surgery or change the recommended surgery). Once all that is done, and if the symptoms are severe enough to consider surgical decompression, that is when you want to sit down with your surgeon and have a specific talk. How bad are your symptoms? Are they continuing to get worse? Has anything helped? If symptoms are progressive, you have achieved maximal medical management, and you have a documented Chiari, I then leave the decision up to the patient. Continue down the current pathway(which has not been fruitful) or consider changing the trajectory with surgery(knowing that there is no study to guarantee a person will improve with surgery and, even under the best circumstances, 15-20% will fail to improve).
Hope these thoughts have been of some assistance. I don't want to treat this forum as an advertisement but my contact information is on the web. Just google me(you can actually e-mail me directly from my web site as well).
Dr. Trumble,
Can I say how refreshing it is for a doctor to interact openly with parents and patients seeking answers? I truly appreciate your response and explanation of the literature. I keep copies of her scans, so I can send them any time and I will be in touch next week. Again, thank you so much for your time.