Question: What is the best way to deal with a hemangioblastoma with a cystic part? My doctor keeps draining it, but it keeps coming back. I've had eight brain surgeries to drain cysts and it looks like I'll need another. Is there an end to this? -- T.P., Ohio

Answer: Draining the cyst only relieves the immediate symptoms; it does not get to the basis of the problem. The cystic fluid is generated by the tumor itself. As long as the tumor is in place, the cyst will continue to re-fill. Thus, the tumor should be removed. With advances in surgical techniques, a neurosurgeon up-to-date in microsurgical techniques and familiar with hemangioblastomas should be able to remove this vascular tumor. They can be tedious and it requires certain surgical techniques that are not needed for most other tumors of the brain or spinal cord, so not all neurosurgeons deal with them.

In the rare cases where the patient is pregnant, or if the patient has already undergone so many surgeries that an additional surgery is not advisable at the time, then draining, shunting, or otherwise managing only the cyst may be the right short-term strategy, but it is not a long-term solution and is rarely advisable, or needed. For the same operative stress on the patient, the tumor could be removed and a longer-term solution reached.

The NIH will be happy to consult with patients and physicians facing this difficult question. -- Edward H. Oldfield, M.D., Chief, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, Maryland

Concerned about Eyestrain and VHL

Question: I have multiple retinal angiomas in my left eye. Despite several laser treatments, I have lost central vision in that left eye. I am concerned about what might happen to my right eye. Will the strain of using my right eye to do the job of two eyes make my condition worse? -- Brenda R., South Africa

Answer: In response to your question, you will not do any harm to your good eye by performing any visual task. You should feel free to read, write, use computers, etc.

Several considerations:

1) We recommend that you use "protective eye wear" (such as polycarbonate lenses, goggles when playing sports such as tennis or when performing tasks that might injure your eyes) to protect your right eye physically

2) Be cautious, especially when driving or in unfamiliar surroundings, since loss of significant vision in one eye can result in loss of depth perception and stereoscopic vision

3) Be cautious when skiing, playing tennis, etc. since you will have loss of depth perception and may have difficulty judging distances.

Please be assured that you should feel free to perform any visual task you desire. -- Jerry Cavallerano, OD, PhD for Lloyd M. Aiello, MD, Beetham Eye Institute, Joslin Diabetes Center, Boston, Massachusetts

Tuning Out Tinnitus

Question: I found an advertisement for a device that "tunes out" tinnitus. Is there any reason why someone with VHL should not use one of these?

Answer: In general, I don't see any harm in these devices as long as the volume or intensity of the masking is not loud enough to cause further damage to the hair cells of the cochlea. They work about as well as any of the other techniques for tinnitus. Biofeedback, hearing aides, etc. .... all have about the same efficacy. The causes of tinnitus are too numerous to count. So it makes sense that not all patients would respond to the same treatment. I wouldn't recommend someone just using something for tinnitus without figuring out why they have the tinnitus in the first place. It could range from anything like a tumor, to too high a medication, low thyroid, high thyroid, infection, aspirin, Motrin, kidney failure, aneurysm, arteriovenous malformation, post-trauma, etc...just to name a few. Too commonly though, a real cause for tinnitus cannot be pinpointed. But its important to make sure its not a treatable, identifiable cause. As a caveat, most tinnitus devices claim amazingly high success rates. Unfortunately, I have not seen a single one that lives up to its claims. -- Daniel Choo, M.D., Neuro-otology Branch, NIDCD, National Institutes of Health, Bethesda, Maryland

Ophthalmic Ultrasound

Question: I heard that they can now detect retinal involvement early with ultrasound. They can see the blood vessels curl before they turn into angiomas. Is this true? -- Emily S., New Jersey

Answer: Ophthalmic Ultrasound is extremely useful and suggested in the evaluation of retinal angiomas. This is a technique that is highly dependent upon having the most sophisticated equipment and a highly experienced examiner. It is a method best used to follow existing angiomas to note change. The Joslin Vision Network and digital imaging is terrific for the initial evaluation of retinal vessels and adjunct to ophthalmic ultrasound. Should you need additional information, feel free to call me. -- Dr. Richard M. Calderon, Associate Chief, Clinical Practice, Beetham Eye Institute, Joslin Diabetes Center, Boston, Massachusetts

As printed in the VHL Family Forum 6:3, September 1998. For permission to reprint, please contact VHL Family Alliance, info@vhl.org.