VHL Eye Care Survey

The American Optometric Association asked us to revisit our recommendation in the VHL Handbook that people at risk for VHL should be seen regularly by an ophthalmologist. They argue that more people in the United States see an optometrist than any other eye care professional. In many places there are not enough ophthalmologists to go around and it would be more convenient and less expensive to go to an optometrist. Is it really necessary to recommend an ophthalmologist? We decided to collect some feedback from the membership.

While we were at it, we asked the members whether in their opinion an ophthalmologist is enough? Is there a case to be made for going straight to a retinal specialist?

A questionnaire was put up on the VHL website in March, and kept there for six weeks. A total of 46 people replied to the questionnaire: 37 people with VHL, 3 people at risk for VHL, 4 other VHL family members, one ophthalmologist, and one other concerned citizen. 43 people (93%) see professionals regularly for eye care.

Of the people with VHL who responded, 25 (68%) have diagnosed lesions. At least once a year, 35% of them see an optometrist, 87% see an ophthalmologist, and 65% regularly see a retinal specialist. All three of the people at risk for VHL see an ophthalmologist annually.

Diagnoses were made 11% (5) by optometrists, 26% (12) by retinal specialists, and 63% (29) by ophthalmologists. At the time of the diagnosis, each had seen another eye care professional an average of 3.5 months prior to the diagnosis.

63% of all respondents recommended that people at risk for VHL be followed by an ophthalmologist. 12 respondents (26%) recommended going directly to a retinal specialist. Only 11% (5) felt that an optometrist would be sufficient to diagnose VHL. Among people with VHL, 65% (24) recommended an ophthalmologist, 30% (11) recommended a retinal specialist, and only two people (5%) felt that an optometrist would be sufficient to diagnose early VHL lesions in people at risk. Both of these respondents noted that their diagnosis was made by an optometrist, but neither of them has an eye lesion, so diagnosis was not made based on finding lesions.

Comments

This data is consistent with the experience of the hotline team in speaking with families over the past five years. The comments left with the questionnaires explain the issue very well.

"If there is a hereditary risk of VHL, I would recommend going directly to an ophthalmologist for all check ups and if there are eye lesions then a retinal specialist. The retinal specialist I see recommends an optometrist for visual acuity exams and necessary corrective eyewear."

"Because of good medical care and numerous laser procedures, I have 20/20 vision."

"Seeing an optometrist is when a new pair of glasses is needed. Angiomas in my eye are being looked after by an ophthalmologist."

"I have had a history of angiomas in both eyes. Presently I have 3 stable ones in right eye. I have had cryo in right eye and laser in left. I have found that sometimes these lesions are difficult to spot unless by an retina specialist or ophthalmologist. I would not trust my boys to be tested by a optometrist since these are not the usual areas an optometrist would study and because they are so hard to spot."

"An Optometrist referred me to an Ophthalmologist who specializes in the retina. He did the diagnosing. My Optometrist described her capabilities as ‘looking into a room through the keyhole’ — she could only see so much. To see all the room you need to go to an Ophthalmologist. I feel anyone at risk for VHL should see an Ophthalmologist."

Delay = Visual Impairment

All too often there were delays in spotting that first angioma, and delayed treatment can mean loss of vision.

"The Optometrist only referred me to a retinal specialist when I complained of seeing "cobwebs" and floaters in my vision. I only see the Optometrist for a new prescription for my glasses."

"We had an evaluation for our daughter with an optometrist who said her retinas were clear when she actually had a large hemangioblastoma in the right eye that was diagnosed at the NIH approximately 12 months later."

"I was seen by an optometrist one month before I went to NIH. He said he did a complete check and there were absolutely no retinal angiomas in either eye. I saw the retina specialist at NIH and I had one in each eye. After that experience, I have gone to a retinal specialist only."

"My Optometrist missed my lesions for five years and I paid because of it. I would avoid them and go right to Ophthalmologists every time"

"My tumors were almost missed by an ophthalmologist. It was only after I asked him to look carefully for these tumors that he found one. (There were two.) They were treated by a retina specialist and I see him for yearly checkups."

Challenges of Managed Care

"Managed health care coverages generally will not allow a person to see a specialist without a referral, which means a problem has to be found first by the Primary care physician or optometrist before a ophthalmologist or retinal specialist can be seen. This will certainly delay effective treatment."

"Without being seen by an ophthalmologist it would have been worse. However it does put a strain on the pocket book."

We realize that there are cheaper options. We discussed this issue at length prior to publication of the first Handbook, and again prior to the publication of the second edition. However because of the number of stories like these that exist in our community, we felt that we could not stop with the cheap solution, we needed to recommend that an ophthalmologist be used. Degrees do not make the difference. There are very talented and conscientious optometrists who will do at least as well as most ophthalmologists, and optometrists do often make diagnoses of VHL (27% of the time in this group alone). But as a general rule, it is statistically more likely that an ophthalmologist will find lesions at an earlier, more treatable stage than an optometrist.

Conclusions

The people who said they did not see an optometrist, and saw an ophthalmologist or retinal specialist instead, would surely correct themselves to say that when they go to the ophthalmologist or retinal specialist, that check-up almost invariably includes a session with an optometrist who takes care of the refraction, pressure testing, and other first-line eye screening. While many diagnoses of VHL are made by optometrists, ophthalmologists are more likely to find lesions early enough to successfully conserve vision.

The experience of our members has led us to the belief that most optometrists cannot be relied upon to find retinal angiomas early enough to conserve vision. Even many ophthalmologists may not find very small angiomas.

Retinal specialists are even more likely to find early tumors, but because of the additional expense and the availability of services, referral to a retinal specialist is usually not warranted until at least one lesion has been identified, or at least until a clinical diagnosis of VHL has been made.

We therefore confirm our recommendation that all people at risk for VHL visit at least once a year an ophthalmologist familiar with VHL who will do a thorough check of the retina and its equator.

This is not to diminish the essential role of the optometrist in eye care. Every person (with or without VHL) should see an optometrist regularly for basic eye care (refraction, pressure testing, first-line screening). People under the care of an ophthalmologist or retinal specialist will generally see an optometrist as part of that care.