Question: I have some elective abdominal surgery coming up, and the anesthesiologist is recommending an epidural. Doesn't that involve sticking a needle into my spinal cord? Should I be concerned about possible VHL tumors in the way? -- Tania D., Ottawa, Canada

Answer: With epidural anesthesia the catheter is entirely outside the dura (the strong, fibrous covering of the spinal cord) and outside the cerebrospinal fluid (CSF) that is contained by the dura. However, occasionally during placement of the catheter for epidural anesthesia the needle enters the dura and the CSF space. It would be quite a rare occurrence for it to go deep enough to reach the spinal cord or a tumor in the spinal cord. Therefore epidural anesthesia is not contraindicated in patients with VHL, it just needs to be selected and planned with knowledge of the current state of the central nervous system involvement with VHL. That is also so for any major surgical procedure. In addition to the potential spinal involvement with large hemangioblastomas, the cerebellar tumors can cause pressure-related changes that can be abruptly exacerbated by withdrawing CSF from the spinal area. -- Edward H. Oldfield, M.D., Chief, Surgery Branch, National Institute of Neurological Disorders and Stroke, Bethesda, Maryland

Hiccups

Question: My partner often mentions the significance of hiccups as an indication of VHL. Her MRI's and CAT scans have all been negative, but she does hiccup more than the average person. I don't think I hiccup more than 2 or 3 times a year, but she does pretty regularly. It makes me very nervous. Are hiccups really an indication of VHL problems? -- Concerned, New York

Answer #1: This might be something that relatives talked about in her family. Brain tumors can sometimes occur near the hiccup center in the brain and can cause hiccups. There is one family in particular that swears that increased hiccupping is their first sign of a brain tumor. So if she has heard this in her family, it could be contributing to her anxiety.

However there are lots of things (including anxiety) that can set off hiccups. One swallow of a carbonated beverage will do it for me. I know that as a young woman I got hiccups quite regularly and very intensely. I still get them intensely, but less often and I have learned ways to control them. I do not have VHL, it's just the way I am.

So can hiccups be related to VHL? -- Yes. Are they a sure sign of VHL? -- No.

If her scans are clear, then they are most likely garden-variety hiccups.

Things that work for me:

1) Hold your breath and take 10 sips of water swallowing each one in turn before you take another breath. It's the sip-swallow action and buildup of carbon dioxide that helps.

2) Swallow a whole teaspoonful of sugar, all grainy, without chewing it. -- really!

3) At the first sign of hiccups, gently press against your breastbone. Find the spot that gives you the most relief, and press as firmly as required. With this method I can usually stop them before they get established, so this is my preferred method. My spot is low, right between my breasts.

I am also forwarding your question to Dr. Oldfield for an expert medical reply.

-- Joyce Wilcox Graff, Editor, VHL Family Forum

Answer #2: We have cared for a couple of patients at the NIH in whom hiccoughs were the initial sign of a brainstem hemangioblastoma. From our studies it is clear that there is a particular site in the brainstem from which almost all brainstem hemangioblastomas arise and that site is in an area posteriorly just below the fourth ventricle in an area known as the "area postrema". Involvement of that region can be associated with hiccoughs. Therefore, if the hiccoughs clearly persist much longer than one might expect they normally should, it might be a good idea to get an MRI scan.

We should keep in mind that this has occurred in only a couple of patients among several hundred VHL patients that we have evaluated, indicating that everyday hiccoughs are the most likely explanation in most persons with VHL, just as in persons without VHL.

-- Edward H. Oldfield, M.D., Chief, Surgery Branch, NINDS, Bethesda, Maryland

As printed in the VHL Family Forum 9:2, June 2001. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.