The following article is a part of conference coverage from the American Academy of Ophthalmology 2020, being held virtually from November 13 to 15, 2020. The team at Ophthalmology Advisor will be reporting on the latest news and research conducted by leading experts in ophthalmology. Check back for more from the AAO 2020.
In 2018, the von-Hippel Lindau (VHL) surveillance guidelines consortium launched a project to create new guidelines specifically geared toward treating the retinal aspect of this multi-organ condition, Anthony B. Daniels, MD, explained at the American Academy of Ophthalmology 2020 virtual meeting. The slideshow he presented focused on the top 3 primary recommendations and 5 additional points.
The recommendations, Dr. Daniels said, were “fairly straightforward.” First, individuals with known or suspected VHL should be screened with dilated ophthalmoscopy. Second, patients with retinal hemangioblastoma — even only a single hemangioblastoma — or who are at risk should undergo genetic testing as soon as possible to identify disease early. Finally, the guidelines recommend that patients are managed by physicians who have obtained subspecialty training and have experience with VHL and retinal hemangioblastoma.
The consortium also addressed a number of specific questions to help establish a universal approach to diagnosing and managing VHL patients. The team concluded that clinicians can begin screenings as early as 6 months and continue them at least every 6 to 12 months afterward until age 30.
“There’s clearly a peak in retinal hemangioblastoma incidence in ages 10 to 30,” Dr. Daniels explained. Patients should also be screened prior to a planned pregnancy. “Most tumors don’t grow faster during pregnancy, but a small subset can have very profound growth, particularly in associated exudation,” he explained.
The new recommendations also advocate for the use of widefield photography to monitor retinal hemangioblastomas in addition to a dilated exam. Finally, the researchers recommend early treatment for lesions, unless there is concern because the lesion is near the nerve or macula.
Prior guidelines, he reported, were lacking in that they were often created with only 1, or sometimes 0, ophthalmologists weighing in. The new consortium put together subcommittees of professionals to address the care for each organ affected. The ophthalmology subcommittee included Dr. Daniels as well as Emannuel Chang, MD; Emily Chew, MD; Dan Gombos, MD; Michael Gorin, MD; Carol Shields, MD; and Henry Wiley, MD.
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Daniels A, Chang E, Chew E, et al. The new 2020 guidelines for retinal surveillance of patients with von-Hipple Lindau (VHL) Disease: a new approach. Presented at: American Academy of Ophthalmology 2020 Annual Meeting; November 13-15, 2020. Abstract PO478