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  1. 1. Doheny Days 1/5/2008 Irina Bykhovskaya, MD PGY-3
  2. 2. HPI <ul><li>57 year old Caucasian female with VHL disease. VHL gene confirmed. </li></ul><ul><li>PMH: 25 surgeries for posterior fossa, adrenal and kidney hemangioblastomas </li></ul><ul><li>Family Hx: VHL in maternal grandmother, mother, and two brothers. </li></ul><ul><li>In 2000: no retina findings at Doheny. </li></ul><ul><li>Then followed by her comprehensive ophthalmologist on an annual basis. </li></ul><ul><li>Since 2/2002: Followed at DEI with c/o decreased VA OS for several months and suspected retinal hemangioblastoma OS </li></ul>
  3. 3. Clinical Examination <ul><li>VA 20/20 OD, 20/200 OS </li></ul><ul><li>Tonopen IOP - normal </li></ul><ul><li>SLE: normal </li></ul><ul><li>DFE: </li></ul><ul><ul><li>OD: entirely normal fundus </li></ul></ul><ul><ul><li>OS: </li></ul></ul><ul><ul><ul><li>extensive inferior neurosensory retinal detachment  </li></ul></ul></ul><ul><ul><ul><li>two focal areas of obvious von Hippel Lindau type retinal hemangioblastomas </li></ul></ul></ul><ul><ul><ul><li>cystoid macular edema </li></ul></ul></ul><ul><ul><ul><li>epiretinal membrane </li></ul></ul></ul>
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  10. 11. Treatment <ul><li>2/22/2007: laser photocoagulation #1 was performed </li></ul><ul><li>3/1/2007: laser treatment #2 was performed </li></ul><ul><li>3/15/2007: VA was still 20/400 and fluid persisted </li></ul><ul><li>Diagnosis: </li></ul><ul><li>Exudative retinal detachment due to inferior large hemangioblastoma remained recalcitrant after two laser treatments </li></ul>
  11. 12. Next Step in Management: PDT <ul><li>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 </li></ul><ul><li>Standard PDT treatment </li></ul><ul><ul><li>6 mg/m2 body </li></ul></ul><ul><ul><li>radiant exposure of 100 J/cm2 at an irradiance of 600 mW/ cm2 </li></ul></ul><ul><ul><li>166 seconds using Volk Transequator wide-field lens was used to visualize the lesion during treatment </li></ul></ul><ul><li>Unable to cover the entire lesion. </li></ul><ul><li>The treatment was performed without limitations.  </li></ul><ul><li>9/13/07 s/p PDT: VA 20/200 OS, clinical exam revealed significant resolution of serous exudative neurosensory detachment. </li></ul>
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  17. 18. Conclusion <ul><li>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 </li></ul>
  18. 19. References <ul><li>1. Singh A, Shields JA, Shields CL. Solitary retinal capillary hemangioma: hereditary (von Hippel-Lindau disease) or non-hereditary? Arch Ophthalmol. 2001;119(2):232-234. </li></ul><ul><li>2. Welch RB. von Hippel-Lindau disease: the recognition and treatment of early angiomatosis retinae and the use of cryosurgery as an adjunct to therapy. Trans Am Ophthalmol Soc. 1970;68:367-424. </li></ul><ul><li>3. Raja D, Benz MS, Murray TG, Escalona-Benz EM, Markoe A. Salvage external beam radiotherapy of retinal capillary hemangiomas secondary to von Hippel-Lindau disease: visual and anatomic outcomes. Ophthalmology. 2004;111(1):150-153. </li></ul><ul><li>4. Aaberg TM Jr, Aaberg TM Sr, Martin DF, Gilman JP, Myles R. Three cases of large retinal capillary hemangiomas treated with verteporfin and photodynamic therapy. Arch Ophthalmol. 2005;123(3):328-332. </li></ul><ul><li>5. Grossniklaus HE, Thomas JW, Vigneswaran N, Jarret WHI. Retinal hemangioblastoma: a histologic, immunohistochemical, and ultrastructural evaluation. Ophthalmology. 1992;99:140–145. </li></ul><ul><li>6. Vortmeyer AO, Chan CC, Chew EY, et al. Morphologic and genetic analysis of retinal angioma associated with massive gliosis in a patient with von Hippel-Lindau disease. Graefes Arch Clin Exp Ophthalmol. 1999;237:513–517. </li></ul><ul><li>7. Bressler NM, Treatment of Age-Related Macular Degeneration With Photodynamic Therapy (TAP) Study Group. Photodynamic therapy of subfoveal choroidal neovascularization in age-related macular degeneration with verteporfin: two-year results of 2 randomized clinical trials-tap report 2. Arch Ophthalmol. 2001;119:198-207 </li></ul><ul><li>8. Allison BA, Waterfield E, Richter AM, Levy JG. The effects of plasma lipoproteins on in vitro tumor cell killing and in vivo tumor photosensitization with benzoporphyrin derivative. Photochem Photobiol. 1991;54:709-715. </li></ul><ul><li>9. Singh AD, Nouri M, Shields CL, et al. Treatment of retinal capillary hemangioma. Ophthalmology. 2002;109:1799-1806. </li></ul><ul><li>10. Chan CC, Collins AB, Chew EY. Molecular pathology of eyes with von Hippel-Lindau (VHL) Disease: a review. Retina. 2007 Jan;27(1):1-7. </li></ul><ul><li>11. Lindsay Harris and Tom Aaberg Sr. “Therapeutic Options for Retinal and Optic Nerve Angiomas.” Vail Vitrectomy 2007 </li></ul>