Macular hole secondary to capillary hemangioblastoma

Full-thickness macular holes affect 0.3% of the population, worldwide. This condition has a predilection for females in their sixth to seventh decade. Patients can present with severe impairment of their central vision or can be relatively asymptomatic. We believe that an optic disc hemangioblastoma adhered to the vitreous, causing an anterior displacement of the hyaloid membrane that resulted in a form of vitreomacular traction that pulled the macular hole nasally, toward the optic nerve.

A 66-year-old white male presented to our clinic with decreased vision. He had a previously stable, longstanding optic disc hemangioblastoma. On presentation, an early developing full-thickness macular hole was noted. The outcome was a full-thickness macular hole that was disproportionately more detached on the nasal side of the macula. Treatment regimen included repeated intravitreal off-label injections with bevacizumab, followed by external beam radiation therapy, ultimately followed by surgery.

Treatment in this case was especially complicated because of the presence of the macular hole in addition to the hemangioblastoma. The plan was to decrease vascular activity of the hemangioblastoma before proceeding with macular hole repair. First, off-label bevacizumab injections were administered, followed by external beam radiation therapy, and finally combined surgery. Surgery was successful, and to date, the macula is flat and intact.