International Conference on Ophthalmic Photography

Organized by
Australian Institute of Medical and Biological Illustration
Japanese Ophthalmic Photographers' Society
Ophthalmic Imaging Association
Ophthalmic Photographers' Society

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Saturday, 20 May 2006
Plenary Session 6
Clinical Case Presentations
1.50 OPS Continuing Education Credits
08:15 Introduction By Moderator
Colin Clements
King's College Hospital
London, England, UK
08:20 A Case Of Vogt-Koyanagi-Harada Syndrome
Tay Yilmaz, C. Stephen Foster, MD
Massachusetts Eye Research and Surgery Institute
Boston, Massachusetts, USA
Presentation: A 53 y/o wf of Inuit descent was referred in 12/05 for a complaint of redness and pain for one month and decreased vision OU for the past four days. The morning after receiving a flu shot, one month ago, her eyes were swollen and she experienced flashes (OD>OS) with an accompanying migraine and scalp tenderness to touch. Her past medical history was unremarkable for diabetes, hypertension, arthritis, and/or skin lesions. She did not have a past history of ocular trauma. Prior to coming to our clinic, she was treated for iritis and potential angle closure glaucoma in her right eye. She presented to our clinic with VA (without correction) of CF at 2’ OU.
On the patient’s first visit an FA was performed, which included peripheral sweeps demonstrating the areas of multiple hyperfluorescent pinpoint dots at the level of the retinal pigment epithelium. Images captured during fundus photography demonstrated the numerous pinpoint spots of hyperpigmentation. The montage feature was utilized in an effort to demonstrate how widespread these choroidal infiltrates are in the fundus and to fully emphasize the diffuse nature of this disease. Given the fact that the patient presented with macular detachments OU, all three angles were used to view the area of subretinal fluid.
Conclusions: The FA revealed the characteristic findings of a “starry sky” appearance, multiple hyperfluorescent dots during the AV transit phase, pooling of sodium fluorescein in the subretinal space in areas of exudative RD, and optic nerve head leakage. Photos obtained using 20, 35, and 50 degree angles allowed for better delineation of affected ocular vasculature. Furthermore, features such as the zoom, enhance contrast, and montage successfully illustrated pathology associated with this condition.

08:35 A Case Presentation On The Radiologic And Ophthalmic Images Of A Visually Asymptomatic Patient With Systemic Lupus Erythematosus And Retinal Vascular Disease
Denise Cunningham
National Eye Institute
Bethesda, Maryland, USA
Introduction: It is not unusual for the ophthalmologist to be the first to diagnose a systemic disease or disorder, such as diabetes or hypertension, with an eye examination. In this case, a visually asymptomatic patient with the autoimmune disease, Systemic Lupus Erythematosus, was referred for an eye examination by a radiologist because of “vitreous enhancement” of the right eye seen on Magnetic Resonance Imaging (MRI).
Presentation: A 35 year old female without ocular symptoms was seen in consultation by a comprehensive ophthalmologist. With correction, her visual acuity measured 20/25 OU. In both eyes, her anterior segments appeared normal as did her vitreous and optic nerves. Fundus examination after dilation revealed the presence of RPE changes, evidence of “old vasculitis” with collateral vessels and a sea-fan configuration in the temporal periphery of the right eye. Fluorescein angiography (FA) demonstrated hyperfluorescence of the sea-fan with non-perfusion of the capillary bed just temporal to the vascular abnormalities. Images demonstrating the fluorescein leakage, which coordinates well with the enhancement seen on MRI, will be shown along with frames from the radiologic study.
Conclusion: Ophthalmic problems can present in a variety of ways. Having an MRI that was read astutely by a radiologist who properly referred the patient to an ophthalmologist allowed this patient to be diagnosed and treated promptly - before suffering any visual consequences.

08:50 A Case Of "Doughnut Rings"
John V. Arnold
Hammersmith Hospital
London, England, UK
A young female was referred with bilateral blindness. Funduscopically, circular lesions of unknown aetiology were observed. A fluorescein angiogram was requested to determine possible retinal or choroidal vascular involvement.
The patient was hyperactive and not particularly co-operative. The angiogram had to be obtained with the patient standing up because she found it difficult to sit down.
The result of the angiogram suggested lesions at the level of the pigment epithelium. The cause of these lesions is still under investigation.
This presentation will describe the nature of these lesions and the way in which an angiogram was obtained in particularly difficult circumstances.

09:05 A Case Of Systemic Lupus Erythematosus- Associated Choroidopathy
Tay Yilmaz, C. Stephen Foster, MD
Massachusetts Eye Research and Surgery Institute
Boston, Massachusetts, USA
Methods: A 36 y/o wf was referred in 5/05 for a complaint of blurriness OD>OS. Her past medical history was remarkable for systemic lupus erythematosus idiopathic thrombocytopenia purpura. She presented with VA of 20/40(OD) and 20/20 (OS). Two months later her VA was HM (OD) and 20/20 (OS) as a result of recurrent subretinal fluid. This led to the suspicion of possible multifocal central serous retinopathy (CSR).
Her treatment regimen included prednisone, mycophenolate mofetil, and cyclophosphamide at various points during the course of seven months.
Results: Multiple FAs were performed, which included photos taken at a 50 degree angle to demonstrate the areas of multiple leaks at the level of the retinal pigment epithelium. This was done in order to show that the retinal pigment epithelial detachments were not only localized in the macula, but also inferior and superior to the macula. The patient also experienced a bullous RD near the inferior arcade with two persistent “blow-out” areas of leakage in the superotemporal juxtapapillary region in the right eye. The left eye had a total of approximately ten areas of focal leakage. The patient was referred to Dr. Lawrence A. Yanuzzi in which focal laser treatment was performed on these unrelenting leaks affecting both eyes. On the patient's most recent exam, the VA with correction was 20/25 (OD) and 20/20 (OS).
Conclusions: The FA revealed the characteristic findings of multiple areas of leakage through the RPE with pooling and delayed choroidal filling. Photos obtained using 20, 35, and 50 degree angles allowed for better delineation of affected ocular vasculature.
Furthermore, features such as the zoom, enhance contrast, and montage successfully illustrated pathology associated with this condition.

09:20 A Case Presentation Of Acute Exudative Polymorphous Vitelliform Maculopathy In A 13 Year Old Male
Kirsten G Locke¹, Petra Kozma¹, Albert O. Edwards², David G. Birch¹
Retina Foundation of the Southwest¹
Institute for Retina Research²
Dallas, Texas, USA
History: Acute Exudative Polymorphous Vitelliform Maculopathy (AEPVM) was first reported by Dr. Gass in 1988, where he described two young male adults (ages: 24 & 30) with acute vision loss associated with headaches. The fundus showed multiple, round, yellow-white subretinal lesions at the level of the RPE with serous detachments in both eyes. Since 1988, another four adult patients (female: age 43-62) have been identified. We present here the first case of AEPVM in a male teenager and document the evolution of the fundus appearance and retinal function.
Case Report: A 13-year old Caucasian male presented with a 6-week history of photophobia and bilateral vision loss immediately following a flu-like illness and headache. The best-corrected visual acuity (VA) was 20/63 OD and 20/50 OS. The retinas of both eyes had confluent oval, yellow-white subretinal lesions associated with serous retinal detachments and absent foveal reflex. The lesions were hyperfluorescent by the mid-phase of the fluorescein angiography (FA). The visual field (VF) had reduced sensitivity throughout the central field. The multifocal-electroretinogram (mfERG) showed decreased amplitudes across the central 50° of the retina with delayed implicit times.
At a follow-up visit, 8 months later, the VA had improved to 20/20 OD and 20/25 OS. The subretinal lesions had evolved to large, confluent, meniscus-like yellow subretinal deposits near the inferior vascular arcades and parafoveal regions with discrete pigmentary changes in the foveal region. Both VF and the mfERG had improved, but remained abnormal. The mfERG implicit time had returned to normal in the macular area, but remained delayed in the fovea.
At 25 months after the initial visit, VA was 20/32 OD and 20/20 OS with continued complaints of photophobia. The large confluent yellow deposits along the inferior arcade had dispersed into smaller dots and decreased in density. Deposits were also apparent in the central macula. The FA showed window defects and staining associated with areas of pigment disturbance and subretinal lesions. The VF and mfERG were normal, although the mfERG had a few focal areas of borderline reduced amplitudes. The foveal amplitude was normal with borderline delayed implicit time.
Conclusion: Although the cause of AEPVM remains unknown, the speculation of a viral etiology or post-viral inflammatory disorder is consistent with the history of previously reported cases as well as ours. The visual function and imaging studies demonstrate that residual retinal dysfunction persists up to 25 months after visual acuity has returned to normal.

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Saturday, 20 May 2006 Plenary Session 6 Clinical Case Presentations 1.50 OPS Continuing Education Credits
08:15	Introduction By Moderator Colin Clements King's College Hospital London, England, UK
08:20	A Case Of Vogt-Koyanagi-Harada Syndrome Tay Yilmaz, C. Stephen Foster, MD Massachusetts Eye Research and Surgery Institute Boston, Massachusetts, USA Presentation: A 53 y/o wf of Inuit descent was referred in 12/05 for a complaint of redness and pain for one month and decreased vision OU for the past four days. The morning after receiving a flu shot, one month ago, her eyes were swollen and she experienced flashes (OD>OS) with an accompanying migraine and scalp tenderness to touch. Her past medical history was unremarkable for diabetes, hypertension, arthritis, and/or skin lesions. She did not have a past history of ocular trauma. Prior to coming to our clinic, she was treated for iritis and potential angle closure glaucoma in her right eye. She presented to our clinic with VA (without correction) of CF at 2’ OU. On the patient’s first visit an FA was performed, which included peripheral sweeps demonstrating the areas of multiple hyperfluorescent pinpoint dots at the level of the retinal pigment epithelium. Images captured during fundus photography demonstrated the numerous pinpoint spots of hyperpigmentation. The montage feature was utilized in an effort to demonstrate how widespread these choroidal infiltrates are in the fundus and to fully emphasize the diffuse nature of this disease. Given the fact that the patient presented with macular detachments OU, all three angles were used to view the area of subretinal fluid. Conclusions: The FA revealed the characteristic findings of a “starry sky” appearance, multiple hyperfluorescent dots during the AV transit phase, pooling of sodium fluorescein in the subretinal space in areas of exudative RD, and optic nerve head leakage. Photos obtained using 20, 35, and 50 degree angles allowed for better delineation of affected ocular vasculature. Furthermore, features such as the zoom, enhance contrast, and montage successfully illustrated pathology associated with this condition.
08:35	A Case Presentation On The Radiologic And Ophthalmic Images Of A Visually Asymptomatic Patient With Systemic Lupus Erythematosus And Retinal Vascular Disease Denise Cunningham National Eye Institute Bethesda, Maryland, USA Introduction: It is not unusual for the ophthalmologist to be the first to diagnose a systemic disease or disorder, such as diabetes or hypertension, with an eye examination. In this case, a visually asymptomatic patient with the autoimmune disease, Systemic Lupus Erythematosus, was referred for an eye examination by a radiologist because of “vitreous enhancement” of the right eye seen on Magnetic Resonance Imaging (MRI). Presentation: A 35 year old female without ocular symptoms was seen in consultation by a comprehensive ophthalmologist. With correction, her visual acuity measured 20/25 OU. In both eyes, her anterior segments appeared normal as did her vitreous and optic nerves. Fundus examination after dilation revealed the presence of RPE changes, evidence of “old vasculitis” with collateral vessels and a sea-fan configuration in the temporal periphery of the right eye. Fluorescein angiography (FA) demonstrated hyperfluorescence of the sea-fan with non-perfusion of the capillary bed just temporal to the vascular abnormalities. Images demonstrating the fluorescein leakage, which coordinates well with the enhancement seen on MRI, will be shown along with frames from the radiologic study. Conclusion: Ophthalmic problems can present in a variety of ways. Having an MRI that was read astutely by a radiologist who properly referred the patient to an ophthalmologist allowed this patient to be diagnosed and treated promptly - before suffering any visual consequences.
08:50	A Case Of "Doughnut Rings" John V. Arnold Hammersmith Hospital London, England, UK A young female was referred with bilateral blindness. Funduscopically, circular lesions of unknown aetiology were observed. A fluorescein angiogram was requested to determine possible retinal or choroidal vascular involvement. The patient was hyperactive and not particularly co-operative. The angiogram had to be obtained with the patient standing up because she found it difficult to sit down. The result of the angiogram suggested lesions at the level of the pigment epithelium. The cause of these lesions is still under investigation. This presentation will describe the nature of these lesions and the way in which an angiogram was obtained in particularly difficult circumstances.
09:05	A Case Of Systemic Lupus Erythematosus- Associated Choroidopathy Tay Yilmaz, C. Stephen Foster, MD Massachusetts Eye Research and Surgery Institute Boston, Massachusetts, USA Methods: A 36 y/o wf was referred in 5/05 for a complaint of blurriness OD>OS. Her past medical history was remarkable for systemic lupus erythematosus idiopathic thrombocytopenia purpura. She presented with VA of 20/40(OD) and 20/20 (OS). Two months later her VA was HM (OD) and 20/20 (OS) as a result of recurrent subretinal fluid. This led to the suspicion of possible multifocal central serous retinopathy (CSR). Her treatment regimen included prednisone, mycophenolate mofetil, and cyclophosphamide at various points during the course of seven months. Results: Multiple FAs were performed, which included photos taken at a 50 degree angle to demonstrate the areas of multiple leaks at the level of the retinal pigment epithelium. This was done in order to show that the retinal pigment epithelial detachments were not only localized in the macula, but also inferior and superior to the macula. The patient also experienced a bullous RD near the inferior arcade with two persistent “blow-out” areas of leakage in the superotemporal juxtapapillary region in the right eye. The left eye had a total of approximately ten areas of focal leakage. The patient was referred to Dr. Lawrence A. Yanuzzi in which focal laser treatment was performed on these unrelenting leaks affecting both eyes. On the patient's most recent exam, the VA with correction was 20/25 (OD) and 20/20 (OS). Conclusions: The FA revealed the characteristic findings of multiple areas of leakage through the RPE with pooling and delayed choroidal filling. Photos obtained using 20, 35, and 50 degree angles allowed for better delineation of affected ocular vasculature. Furthermore, features such as the zoom, enhance contrast, and montage successfully illustrated pathology associated with this condition.
09:20	A Case Presentation Of Acute Exudative Polymorphous Vitelliform Maculopathy In A 13 Year Old Male Kirsten G Locke¹, Petra Kozma¹, Albert O. Edwards², David G. Birch¹ Retina Foundation of the Southwest¹ Institute for Retina Research² Dallas, Texas, USA History: Acute Exudative Polymorphous Vitelliform Maculopathy (AEPVM) was first reported by Dr. Gass in 1988, where he described two young male adults (ages: 24 & 30) with acute vision loss associated with headaches. The fundus showed multiple, round, yellow-white subretinal lesions at the level of the RPE with serous detachments in both eyes. Since 1988, another four adult patients (female: age 43-62) have been identified. We present here the first case of AEPVM in a male teenager and document the evolution of the fundus appearance and retinal function. Case Report: A 13-year old Caucasian male presented with a 6-week history of photophobia and bilateral vision loss immediately following a flu-like illness and headache. The best-corrected visual acuity (VA) was 20/63 OD and 20/50 OS. The retinas of both eyes had confluent oval, yellow-white subretinal lesions associated with serous retinal detachments and absent foveal reflex. The lesions were hyperfluorescent by the mid-phase of the fluorescein angiography (FA). The visual field (VF) had reduced sensitivity throughout the central field. The multifocal-electroretinogram (mfERG) showed decreased amplitudes across the central 50° of the retina with delayed implicit times. At a follow-up visit, 8 months later, the VA had improved to 20/20 OD and 20/25 OS. The subretinal lesions had evolved to large, confluent, meniscus-like yellow subretinal deposits near the inferior vascular arcades and parafoveal regions with discrete pigmentary changes in the foveal region. Both VF and the mfERG had improved, but remained abnormal. The mfERG implicit time had returned to normal in the macular area, but remained delayed in the fovea. At 25 months after the initial visit, VA was 20/32 OD and 20/20 OS with continued complaints of photophobia. The large confluent yellow deposits along the inferior arcade had dispersed into smaller dots and decreased in density. Deposits were also apparent in the central macula. The FA showed window defects and staining associated with areas of pigment disturbance and subretinal lesions. The VF and mfERG were normal, although the mfERG had a few focal areas of borderline reduced amplitudes. The foveal amplitude was normal with borderline delayed implicit time. Conclusion: Although the cause of AEPVM remains unknown, the speculation of a viral etiology or post-viral inflammatory disorder is consistent with the history of previously reported cases as well as ours. The visual function and imaging studies demonstrate that residual retinal dysfunction persists up to 25 months after visual acuity has returned to normal.