Retinal Capillary Hemangiomas Indicating von Hippel–Lindau Disease

By Sjakon Tahija, MD
 

An 18-year-old man from an isolated village was brought to our hospital complaining of decreased vision in his right eye. He reported that his left eye had experienced no vision for more than 2 years. On examination, his BCVA was 0.4 (Snellen decimal) in the right eye and hand motion in the left eye. Indirect ophthalmoscopy with a 20-D lens and peripheral indentation revealed a tractional retinal detachment in the left eye. The right eye had large areas of exudates and retinal capillary hemangiomas of various sizes (main image, inset image). With these clinical findings, a diagnosis of von Hippel–Lindau disease was made, as retinal capillary hemangiomas are the most frequent and earliest indications of the disease.1

Management of capillary hemangiomas is difficult, and treatment options are varied. Options include low-power, long-duration laser photocoagulation, photodynamic therapy, cryotherapy, and excision.

We initiated treatment with laser photocoagulation, which caused an exudative response that we treated with intravitreal bevacizumab (Avastin, Genentech). Following this treatment, the capillary hemangiomas temporarily shrank, but they later increased in size again. We then performed photodynamic therapy with intravitreal bevacizumab, which was also only temporarily effective.

The patient later developed a tractional retinal detachment in the right eye, which we treated with vitrectomy, scleral buckle, and silicone oil tamponade. Following surgery, the retina settled down and, after cataract surgery, the patient achieved 0.1 VA in the right eye.

1. Thanos A, Young LH, Weng CY, Karth PA, Shah VA. Retinal Capillary Hemangioblastoma and von Hippel-Lindau Disease. Eyewiki.AAO.org. December 11, 2018. https://eyewiki.aao.org/Retinal_Capillary_Hemangioblastoma_and_von_Hippel-Lindau_Disease#Von-Hippel_Lindau_Disease. Accessed April 24, 2019.

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Section Editor Manish Nagpal, MS, DO, FRCS(Edin)
• Senior Consultant, Retina and Vitreous Services, Retina Foundation, Ahmedabad, India
drmanishnagpal@yahoo.com

Sjakon Tahija, MD
• Vitreoretinal Surgeon, KMN EyeCare, Jakarta, Indonesia
• Editorial Advisory Board Member, Retina Today
sjakon.tahija@gmail.com
• Financial disclosure: None

 

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Retina Today is a publication that delivers the latest research and clinical developments from areas such as medical retina, retinal surgery, vitreous, diabetes, retinal imaging, posterior segment oncology and ocular trauma. Each issue provides insight from well-respected specialists on cutting-edge therapies and surgical techniques that are currently in use and on the horizon.