The following is a transcript of a Q&A session at Spinal CSF Leak: Bridging the Gap 2024. Please note that the transcript been edited for length and clarity to center on the participant questions, whether put forward via online or in-person submission.
Question: From a physician perspective: what can patients do communication-wise to give themselves the best chance of getting the care they need?
Answer:
Dr. Mark Mamlouk: I think it’s important, as mentioned in this conference, to be a good patient advocate. You are your best advocate and relay all you can to your provider. I think it’s important to look at as a team approach. This is a journey, I like to mention to my patients, and this is not just going to be a one-and-done type of thing. So, I think if you have that mindset and have that approach, you can look at it as a journey, and I think that will hopefully yield a fruitful relationship and a good outcome.
Question: What in a patient history or symptoms would clinically indicate that a workup for a spinal CSF leak is warranted? What is relevant for patients to bring up? When do you think an additional workup is indicated for a diagnosis of CSF leak? What is relevant for the patient to bring up to their physicians?
Answer:
Dr. Ian Carroll: I would say bringing up all of the symptoms that are impairing their quality of life and then having the physician try and assess whether there is a good or poor match with what we understand about CSF leaks. But any symptom that is impairing their quality of life is relevant. Clearly the main ones are what Dr. Rau covered – headache, more compelling when it is severe, when it is daily, when it is second half of the day, when it is orthostatic, neck pain, neck stiffness, tinnitus.
I think of vestibular cochlear symptoms in the setting of headache as really important, and I think there’s three classes: there’s symptoms of balance related to vestibular function, there’s symptoms of altered hearing, and then adventitious sounds like ringing that are related to vestibular function. There’s a sensory domain of feeling like your ears are pressurized or you need to pop.
So that constellation, and then daily nausea, difficulty with memory, thinking, concentration, and fatigue – all of those together, they are the constellation that tells you there is a good chance you are dealing with a CSF leak and should at least be evaluated.
Kristen Friese (patient speaker): The one thing I would add is because I was leaking for so long, I didn’t realize pain was an abnormal state of being. So, bring up any relevant pain and also bring up any relevant fatigue because any pain is abnormal. Any profound fatigue that has you falling asleep throughout the day or after 10 hours of sleep at night you’re still exhausted – if that’s happening on a regular basis that is atypical and you should bring that up to your provider.
Question: Primarily for Dr. Carroll: my wife has yet to be officially diagnosed with a CSF leak, but as an effort to try to relieve the pain and stuff, they did try a couple of patches – two non-targeted patches. And the doctor that did it said, “Well, we tried two, it didn’t work, not likely three or four is going to work.” Although I’ve seen today a lot of people testify about or mention multiple patches. What is your guidance? Should I even try to pursue getting another patch before we get referred here or wherever we go in that regard?
Answer:
Dr. Ian Carroll: So, I think it is important to have a sense of at what point are you going to stop? I think if you’ve done a non-directed patch two times and it has done nothing, the likelihood of a third patch dramatically changing things when done in the same way is low. Some of the patients you’ve heard from have had multiple patches – if it wasn’t working in one place, we would try something else. I think that if you’ve tried two non-directed patches and you have symptoms that were convincing enough to your physician to do that, then getting referred to an expert center where you could get some advanced imaging would be very appropriate.
As Dr. Callen said earlier, 50% of the leaks that we’re finding now at least are CSF-venous fistulas. We don’t think most of those are fixed by non-directed lumbar epidural blood patches. They might respond better to transforaminal fibrin patches or occlusions. We’re going to hear more about that. But I think two lumbar epidural blood patches is a good thing to try and then say okay, it didn’t work, let’s go do something else.
Question: The question I had is about the imbalance issue, which has been touched on bit briefly. That seems to be one of [my wife’s] probably second biggest symptom, but it hasn’t been mentioned by anybody we’ve been to thus far, and I was curious to see if people that have had recovery or been sealed had imbalance problems and were they resolved? I mean really, I either have a gate belt on her or she’s on a walker.
Dr. Ian Carroll: We see significant balance problems in both the image positive cohort of patients and the patients who have the symptoms of a leak but whose imaging is negative. When they respond to patching, those symptoms often get better. The tinnitus we see get better less reliably, but the other symptoms we see more reliably get better.
Kristen Friese (patient speaker): I can also say personally I was in a wheelchair for part of my time while I was leaking from balance issues, and I also was regularly using a cane intermittently. And when I was sealed, those symptoms completely went away after I think even just a month being sealed for me personally.
Moderator: Thank you very much. We’ll try to ask two questions which I think are important from the audience. Dr. Carroll is asked a question but I think he answered that – how long do you recommend a patient to lie flat after a blood patch? He has three days but sometimes 24 hours.
Another question is, “what evidence-based best practices do you recommend that a physician who infrequently performs epidural blood patches follow”?
Answer:
Dr. Mark Mamlouk: I would just suggest on – if you’re performing an epidural blood patch and you’re not very familiar with it, I would say to get familiar with it because there can be harms in itself of performing an epidural blood patch. You could be advancing the needle too far, injecting into the wrong space, and it could potentially be doing more harm than good. So, while it is a safe procedure, there are risks of the procedure, and you want to ensure that you get the adequate training before you end up doing the procedure.
Moderator: Yes, we need more training for a lot of people. Another question: Some leaks seem to resolve immediately after an epidural blood patch, but others require several interventions. In your opinion, what factors seem to influence whether a patch is successful in resolving a patient leak?
Answer:
Dr. Mark Mamlouk: I will say that – I can add to this and Ian you can add to this – first you have to know what you’re treating. Not all leaks are the same and there is not one tool for every type of leak. So, if there is a ventral dural collection that may be treated different than a CSF-venous fistula. Also, the chronicity – Dr. Callen and I have done some research on that has shown that if you’re leaking for a longer time maybe patching may be less effective and surgery may be needed. So, you have to look at it holistically and look at the different types of leaks that you’re approaching or evaluating before approaching it with a patch.
Dr. Ian Carroll: Yeah, I would say the best thing is when you can find the leak and you can localize it, and then you can put fibrin glue right over the site. And if that doesn’t work you can get surgery, and if you can’t find the leak, surgery is really not an option. And then you’re basically hunting for it saying you know, do I see a bone spur here, do I see a perineural cyst here, is there some better kind of imaging I can do?
I think the recent papers that have come out about surgical outcomes when the leak can be found – that you see coming out of the group from the Bern group or the Freiberg group really show [that] if you have a chronic leak, the best thing is to find it and close it surgically. And I think that is number one, and number two is: if you can’t find it, then you’re in the world of trying patches to see if you can get some improvements in quality of life – even though it’s not as good as finding and fixing the leak. And the patches probably work much better when they’re done early in the course of the disease, but our data suggests that they’re helpful even when done eight years into the disease for reasons I don’t really understand, but that’s where we are.
Question: A question for Dr. Carroll or Dr. Callen – is there any experimentation with any new types of glue besides fibrin?
Answer:
Dr. Ian Carroll: I’ve seen one report of using DuraSeal. I think there’s recently another report with a different polymer, I’m trying to remember what it was, and Andrew may be aware of something that another company is exploring, but I don’t know of any anything that has actually been used other than DuraSeal. There was one other sealant that was just a case report but I can’t remember what they were using.
Dr. Andrew Callen: Yeah, I’m not aware of other sealant being used in a routine clinical basis, but we have a collaboration here with some of engineers at CU Boulder looking at sort of hydrogel synthetic alternatives that may have better adhesive properties.
Question: If a sealant is approved, let’s say for you know other procedures, let’s say they’re multiple sealants approved, is it – and I know the epidural space is a dangerous one to deal with for intractable cases – is it something that one could do off label and is that some – is there an innovative branch of our field that’s trying these modalities?
Answer:
Dr. Andrew Callen: The off-label thing is an interesting question. I mean, the fibrin glue being used as patching is off label – it is not a specific clinical indication. When we inject for CSF-venous fistulas, there’s even a black box warning saying don’t do this intravascularly and that’s precisely what we’re trying to do.
We are kind of working in no man’s land, so to speak, trying to do the best thing for our patients. But you have to balance working on the forefront of something where the knowledge is uncertain, with not making our patients guinea pigs and doing things that are inappropriate or that could hurt them. So, using your judgment in the scope of medical ethics and what’s appropriate to find that space.
The fibrin glue had been used so extensively in the surgical literature that we knew that it was safe in that space. And so similarly for something novel, we would want some sort of basis whether it’s animal studies or a parallel use in another medical domain where we felt like it was appropriate to use.
Moderator: Is there any study that contains data on spinal CSF leak misdiagnosis? For example, the percentage of spinal CSF leak patients who are initially misdiagnosed with something else and what the most common misdiagnoses are? In terms of what most common misdiagnoses are, Dr. Friedman and earlier Dr. Ruhoy were discussing about that, and most recently Dr. Reiman was talking about misdiagnosis. If I can mention just a paper from 20 years ago, Dr. Schievink saying if I remember correctly 90% of the cases were misdiagnosed. That was 20 years ago – I’m sure it’s much better now, but if any of you wants to comment on that, or add some data?
Answer:
Dr. Ian Carroll: I think Schievink’s paper is the only one that’s addressed it as the main focus of the paper unless I’m not remembering one.
Dr. Mark Mamlouk: I’m not sure of any specific recent data, but I will say that I think in the discovery of the CSF-venous fistula that those gaps in care have largely improved. And I think it’s a good thing that we’re able to have identified a new type of spinal CSF leak and patients are able to get better treatments than before. But I think there’s still more data, more research that needs to be done on that.
Dr. Deborah Friedman: Median time to diagnosis was 2 years but highly variable. 75.5% were initially misdiagnosed. Some of the most common misdiagnoses were migraine in 65%, a psychiatric issue 23%, vestibular issue 14%, or IIH in almost 10%. And then about 60% of respondents felt that their healthcare provider had dismissed their symptoms.
Moderator: Thank you everybody.