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PDT VHL

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Information about PDT VHL

Published on July 2, 2008

Author: ophthalmologyweb

Source: slideshare.net

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

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

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

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

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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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

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

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.

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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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

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

References 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. 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. 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. 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. 5. Grossniklaus HE, Thomas JW, Vigneswaran N, Jarret WHI. Retinal hemangioblastoma: a histologic, immunohistochemical, and ultrastructural evaluation. Ophthalmology. 1992;99:140–145. 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. 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 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. 9. Singh AD, Nouri M, Shields CL, et al. Treatment of retinal capillary hemangioma. Ophthalmology. 2002;109:1799-1806. 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. 11. Lindsay Harris and Tom Aaberg Sr. “Therapeutic Options for Retinal and Optic Nerve Angiomas.” Vail Vitrectomy 2007

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.

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.

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.

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.

5. Grossniklaus HE, Thomas JW, Vigneswaran N, Jarret WHI. Retinal hemangioblastoma: a histologic, immunohistochemical, and ultrastructural evaluation. Ophthalmology. 1992;99:140–145.

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.

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

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.

9. Singh AD, Nouri M, Shields CL, et al. Treatment of retinal capillary hemangioma. Ophthalmology. 2002;109:1799-1806.

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.

11. Lindsay Harris and Tom Aaberg Sr. “Therapeutic Options for Retinal and Optic Nerve Angiomas.” Vail Vitrectomy 2007

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