vhl alliance

Active Surveillance

Active surveillance is important for anyone living with a chronic disease. However, it is even more important for people living with VHL disease, given the unpredictability of the condition. Early detection, active surveillance and appropriate treatment can greatly reduce the most harmful consequences of this gene mutation.


The VHL Alliance and its Clinical Advisory Council recommend that children who are at risk for VHL should have genetic testing as early as age 1. A person is considered “at risk” if they have a biological parent with VHL. If someone has a positive test for VHL, they should follow the Active Surveillance Guidelines.

Early screening includes an eye/retina examination, a hearing test, blood pressure measurement and a neurological assessment. Some manifestations, like kidney tumors, tend to show up later than other manifestations, like retina tumors.

Note: the Active Surveillance Guidelines state that abdominal scans should start at age 16 while eye exams begin much earlier. Although it may seem like a lot to do, these guidelines are VERY important in helping keep the worst consequences of VHL at bay.

For more information about specific considerations for imaging VHL tumors in each area of the body, please refer to Section 2 of the VHL Handbook: Possible VHL Manifestations.

For references, click here