This document discusses the case of a 57-year-old female patient with von Hippel-Lindau (VHL) disease who presented with decreased vision in her left eye due to retinal hemangioblastoma. She underwent two unsuccessful laser treatments but photodynamic therapy (PDT) resulted in significant resolution of the serous retinal detachment and cystoid macular edema. PDT was concluded to be an effective treatment for exudative retinal detachment caused by retinal hemangioblastomas in patients with VHL disease.

1. Doheny Days 1/5/2008 Irina Bykhovskaya, MD PGY-3

2. HPI 57 year old Caucasian female with VHL disease. VHL gene confirmed. PMH: 25 surgeries for posterior fossa, adrenal and kidney hemangioblastomas Family Hx: VHL in maternal grandmother, mother, and two brothers. In 2000: no retina findings at Doheny. Then followed by her comprehensive ophthalmologist on an annual basis. Since 2/2002: Followed at DEI with c/o decreased VA OS for several months and suspected retinal hemangioblastoma OS

3. Clinical Examination VA 20/20 OD, 20/200 OS Tonopen IOP - normal SLE: normal DFE: OD: entirely normal fundus OS: extensive inferior neurosensory retinal detachment two focal areas of obvious von Hippel Lindau type retinal hemangioblastomas cystoid macular edema epiretinal membrane

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11. Treatment 2/22/2007: laser photocoagulation #1 was performed 3/1/2007: laser treatment #2 was performed 3/15/2007: VA was still 20/400 and fluid persisted Diagnosis: Exudative retinal detachment due to inferior large hemangioblastoma remained recalcitrant after two laser treatments

12. Next Step in Management: PDT 6/27/2007: PDT treatment was delayed due to patient breaking her ankle and performed three months later (6/27/07) at which time her vision declined to 3/200 and surrounding serous retinal detachment and extensive lipid exudation have persisted Standard PDT treatment 6 mg/m2 body radiant exposure of 100 J/cm2 at an irradiance of 600 mW/ cm2 166 seconds using Volk Transequator wide-field lens was used to visualize the lesion during treatment Unable to cover the entire lesion. The treatment was performed without limitations. 9/13/07 s/p PDT: VA 20/200 OS, clinical exam revealed significant resolution of serous exudative neurosensory detachment.

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18. Conclusion PDT was effective for the treatment of serous retinal detachment and cystoid macular edema a patient with retinal hemangioblastoma in a setting of VHL disease

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