Progress

[GasconAlex]

After a fair bit of telephone work I have at least had some progress. The latest update is I get to have yet another cranial MRI (this makes 11 on the current problems) followed by consultation and surgery a couple of weeks later. (Early summer) The MRI is for a roadmap, they are not expecting the expected CSF leak to be visible.

It would appear that a large portion of my skull base is defective with confirmed leaks (confirmed by surgery) and my brain dropping slightly out of its cavity. They currently believe I have a leak into the sinus area, the previous leak was into the ear.

Not sure if this will change the dysautonomic problems, really hoping it solves the headaches.

[bombsh3ll]

Hi, I am so glad you are getting somewhere and that it may lead to some relief. 

Can I ask what surgery is proposed specifically if you know that yet?

I recently had a supine MRI with contrast to look for CSF leak due to my orthostatic headaches, which showed no sign of a leak. 

I have had a consultation with a neurosurgeon (Dr Gilete in Barcelona) who felt I had cranial settling based on upright scans and discussed C0-2 fusion. It is very expensive surgery and not without risks, so I am sending my scans for a second opinion from a neurosurgeon in the US. 

There is a very encouraging account by a guy called Jeff at MEchanical basis - he had severe POTS, bedridden, and completely recovered after C0-2 fusion, so it is very exciting. 

He actually inspired me to pursue that direction, although I am as yet not totally convinced it is my issue. 

I wish you the best of luck with your upcoming procedures, please keep us updated. 

B xxx

[Pistol]

@bombsh3ll - what is CO-2fusion? If you don't mind - could you explain it? Thanks … 

[GasconAlex]

Not entirely sure exactly what they are proposing as this gets explained just before the operation at the same time as I  have the anesthetic consultation (about a week or so before the op.) The only information I have is that they need the MRI for the surgery to act as guidance. 

This specialist is a 'nose' guy who based on symptoms, and a cone beam (type of CT scan), and the fact that a couple of years ago the MRI said no leak and intact dura a couple of days before the surgeon had to stop because the dura was leaking and had collapsed into my ear, is convinced the last MRI (January) is not definitive and there is another, different leak from the nose.

Several of the specialists have said that the MRI can miss 20-30% of cranial leaks so they cannot be taken as definitive. I'm 'lucky' in that I have several areas of 'defects' in my skull base visible on the CT scans, so they think this could explain some of the symptoms, but with a family history of eds it may well be that the leak just pushed me over the edge from coping with symptoms to being unable to fight through all the issues.

The only thing that really helped my headaches was a blood patch which  worked perfectly for 36 hours - no pain meds at all and about 5 hours out of hospital feeling human (still unstable and unable to walk unsupported but able to stand without headaches). They only tried the blood patch because of several lumber punctures I had with the meningitis. Shortly afterwards I had a consultation with the regional headache specialist neurologist who, unfortunately, is of the opinion if a leak isn't visible on the MRI then there isn't a leak. After that consultation they were unwilling to retry another blood patch. 

Cranial CSF leaks are notorious for being difficult to detect in those with spontaneous leaks so it might well be worth pushing down that road as at least there is a chance things might get better for you if this is your case.

[bombsh3ll]

13 hours ago, Pistol said:

what is CO-2fusion? If you don't mind - could you explain it? Thanks … 

The neurosurgeon lifts the skull up and fixes it in its proper place above the first 2 cervical vertebrae. In the case of cranial settling, this relieves the pressure on the brainstem where the centres controlling fluid balance and autonomic function are located. 

This procedure has cured a number of people of POTS. It doesn't help everybody though and is obviously only indicated if you have cranial settling, which tends to occur in EDS and possibly other connective tissue disorders. 

13 hours ago, GasconAlex said:

Several of the specialists have said that the MRI can miss 20-30% of cranial leaks so they cannot be taken as definitive.

The neurologist I saw feels the scan I had picks up 80%, and definitely any big ones. I have no signs of cranial fluid loss, the concern for me was spinal. As the next investigative step would involve a lumbar puncture I don't think I would pursue this as these can be notoriously difficult to heal in EDS and can actually leave you with a(nother) leak. 

I am glad I had this scan though. It is very possible for orthostatic headaches to occur in POTS in the absence of a leak, as a result of cerebral hypoperfusion. They can also be a symptom of cranial settling but again my scan reports for this are a bit ambiguous. There are also usually additional motor symptoms such as weakness, numbess and choking with cranial settling.

B xxx