Question: I recently visited my family doctor concerning strange bruising or rash on my feet and legs. I am wondering if there is anything in VHL that causes this, or not. My family doctor is referring me to a dermatologist to see if there is some other reason, or it may be nothing. I have had these marks for almost a year now. One thing my doctor did say that is a possibility is that it could be blood vessels growing close to the top layer of skin.

Answer: I have not seen bruising or rash on the feet and legs in any of the hundreds of patients affected by VHL whom I have screened. Other causes should be pursued in the differential diagnoses, including localized vascular insufficiency or peripheral neuropathies. If bruising is generalized (occurring randomly on various part os the body) see a Hematologist who will order blood tests that may diagnose a treatable problem.

Regarding VHL, I would also check for an elevated red blood cell (RBC) count and elevated erythropoietin levels, causing a reddened or flushed appearance, although this does not tend to be localized as described in this case. There have been a few individuals with VHL who have had secondary polycythemia (abnormally high RBCs). If the RBCs are abnormally high, a hematologist will advise whether the level is high enough to require therapeutic phlebotomy (drawing off of some blood) to relieve vascular congestion.

-- Gladys Glenn, M.D., Ph.D., Medical Officer, Genetic Epidemiology Branch, National Cancer Institute, Bethesda, Maryland

Standing MRI?

Question: What can you tell me about the open standing MRI? Are the pictures as good as with the tunnel kind? So many people are claustrophobic that this would be an interesting option as long as the definition is good. Are there a couple of key questions that people could ask to determine whether it would be a wise decision to use the open MRI or not?

Answer: Experience with this type of magnet is still limited. The images are not the same quality as with the enclosed magnets which are also higher in field strength. Moreover, the field of view is smaller, so each image covers a smaller area of the body and more pictures are required.

My first choice for claustrophobic patients who need an MRI would be some light sedation and scanning in a good enclosed high field magnet. Failing that, some of the newer open horizontal magnets are getting pretty good. A Standing is my last choice because of the lack of experience with it and the problem of motion -- imagine standing perfectly still for 5-10 minutes! However, I agree completely that the manufacturers should come up with better designs for their magnets, and there are some under development. But meanwhile we shouldn’t compromise on image quality, since missing those little tumors can be a big problem.

Here are the question I would ask myself when considering an MRI:

1. Can I tolerate (even with some mild sedation) a closed MRI scan? If yes, go for a 1.5 Tesla magnet, (preferably one of the major manufacturers like Siemens, Philips or GE). (Tesla is a measure of the strength of the magnet).

2. If no, what are the available open MR scans in my area? Pick the newest one in the area in a radiology group with a good reputation, (latest models of Toshiba, GE, and Hitachi are good). The early ones were barely satisfactory. The standing open is another option but is not superior to the others for VHL screening. We recently saw a case in which a large mass was missed on an open magnet but was picked up on the closed one. They are not yet equal.

3. Make sure the radiology group has a knowledgeable neuroradiologist familiar with VHL! People are as important as machines! If this is not available locally, ask another radiologist with greater VHL experience for a second opinion on the scans.

-- Peter Choyke, M.D., Chief of MRI, Diagnostic Radiology Branch, Clinical Center, National Cancer Institute, Bethesda, Maryland

As printed in the VHL Family Forum 10:1, March 2002. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org.