Retinal hemangioblastoma occurring at or immediately adjacent to the optic disc — referred to as juxtapapillary or epipapillary — represents a distinct clinical subset with specific therapeutic considerations related to its location.

Hemangioblastoma arising in association with the optic disc is classified as juxtapapillary or epipapillary, in contrast to extrapapillary lesions found elsewhere in the retina. This location influences both the risk of exudation threatening central vision and the feasibility of available interventions.

Clinical approach & goals

The primary objective is to decrease exudation, with a treatment goal of at least modest improvement in intraretinal edema or subretinal fluid. When intervention is undertaken, certain photodynamic-based and intravitreal approaches may be considered — alone or in combination — to address vision-threatening exudation. Treatment benefit in this location is often limited, and procedural risks must be carefully weighed. The complete structured regimen is available in the full protocol.

References

DOI: 10.1097/IAE.0000000000002572

RH can arise in association with the optic disc (designated as juxtapapillary or epipapillary RH), or within retina elsewhere (designated as extrapapillary RH).

Photodynamic therapy, proton beam therapy, or use of intravitreal VEGF antagonists or corticosteroids can be used individually or in combination to decrease exudation in some cases, but risks of treatment must be weighed, and benefit is often limited.

We occasionally use intravitreal VEGF antagonists in an attempt to control RH-associated exudation in cases where ablative therapy is not feasible (as mentioned above for juxtapapillary lesions) or has failed, and some but not all eyes can exhibit at least modest improvement in intraretinal edema or subretinal fluid, as suggested by case reports.

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Treatment of Retinal Hemangioblastoma Arising Near the Optic Disc (Juxtapapillary / Epipapillary)

References