- Email: [email protected]
Optical coherence tomographic findings in acute exudative polymorphous vitelliform maculopathy
Optical Coherence Tomographic Findings in Acute Exudative Polymorphous Vitelliform Maculopathy
The management of RD in SW syndrome is often difficult. Surgery in these eyes is extremely dangerous because of the risk of hemorrhage from the dilated abnormal episcleral and choroidal vessels. The traditional method of therapy using external beam radiation is effective; however, there is a slow absorption of the serous RD, usually taking several months.1 Often, there is a recurrence or persistence of the fluid, necessitating additional external beam radiation therapy, which carries risk of radiation retinopathy, cataract formation, and optic neuropathy. Plaque brachytherapy has been suggested in large circumscribed hemangiomas;2 however, this modality is ineffective in an eye with a diffuse hemangioma occupying the whole choroid. Recent reports have shown success with transpupillary thermotherapy, which is effective only in small, localized tumors and may need to be repeated.3 Photodynamic therapy may be the current treatment of choice for small circumscribed choroidal hemangiomas, especially those located around the optic nerve and macular area.4,5 To our knowledge, this is the first report of successful and rapid resolution of RD in SW syndrome in an eye that had failed conventional external beam radiation therapy. The slow blood flow and capillary dilation in hemangiomas lends itself to a successful closure using a relatively noninvasive procedure. The treatment itself carries a relatively low risk, and the rapid shrinkage in the size of the hemangioma is impressive. Multiple spots of treatment were necessary to cover the majority of the lesion because of the large size of the tumor; however, we were careful to avoid overlapping spots by following anatomic landmarks. We do have some concern about the resultant pigment scarring, though it may provide a mechanical barrier to recurrent accumulation of subretinal fluid. The patient had a long-standing retinal detachment and amblyopia, which limit the recovery of central vision; however, there were very limited therapeutic options available in this scenario. Further trials are warranted to evaluate the appropriate first therapy of choice in serous RD with the SW syndrome.
Vanessa Cruz-Villegas, MD, Natalia Villate, MD, Robert W. Knighton, PhD, Patrick Rubsamen, MD, and Janet L. Davis, MD To report two cases of acute exudative polymorphous vitelliform maculopathy. DESIGN: Observational case reports. METHODS: Fluorescein angiography, optical coherence tomography, and electrophysiology were performed. RESULTS: Both patients presented with sudden onset of blurred vision and bilaterally symmetric, central, and perimacular vitelliform lesions. Perimacular lesions were faintly hyperfluorescent early in the disease, but there was no abnormal fluorescence in the macular centers. Optical coherence tomography revealed anterior displacement of the photoreceptor layer by a hypereflective subretinal layer overlying a hyporeflective space above the retinal pigment epithelium– choriocapillaris complex under all lesions and no subretinal fluid. The electrooculogram was abnormal. Near complete resolution occurred over 4 to 8 months, with no improvement in the electrooculogram. CONCLUSIONS: Transient multifocal vitelliform lesions suggest a diagnosis of acute exudative polymorphous vitelliform maculopathy. Optical coherence tomography reveals characteristic anterior displacement of the photoreceptor layer by a subretinal hypereflective deposit similar to that seen in adult-onset foveomacular vitelliform dystrophy. (Am J Ophthalmol 2003;136: 760 –763. © 2003 by Elsevier Inc. All rights reserved.) PURPOSE:
A
REFERENCES
1. Gottleib JL, Murray TG, Gass JD. Low dose external beam irradiation for choroidal hemangiomas. Arch Ophthalmol 1998;116:815–817. 2. Chao AN, Shields CL, Shields JA, Krema H. Plaque radiotherapy for choroidal hemangioma with total retinal detachment and iris neovascularization. Retina 2001;21:682–684. 3. Fuch AV, Mueller AJ, Gruetrich M, Ubig MW. Transpupillary thermotherapy (TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Ophthalmol 2002;240: 7–11. 4. Robertson DM. Photodynamic therapy for choroidal hemangioma associated with serous retinal detachment. Arch Ophthalmol 2002;120:1155–1161. 5. Schmidt-Erfruth UM, Michels S, Kusserow C, Jurkles B, Augustin AJ. Photodynamic therapy for symptomatic choroidal hemangioma: visual and anatomic results. Ophthalmology 2002;109:2284 –2294.
760
AMERICAN JOURNAL
CUTE
EXUDATIVE
POLYMORPHOUS
VITELLIFORM
maculopathy is a rare disorder characterized by the sudden onset of bilateral, yellow-gray subretinal deposits in the macular area that resemble the lesions of Best disease but rapidly subside. We describe the fourth and fifth published cases with the first report of optical coherence tomography (OCT) findings. Review was undertaken according to the guidelines of the Human Subjects Research Subcommittee.
Accepted for publication April 7, 2003. From the Department of Ophthalmology, University of Miami, School of Medicine, Bascom Palmer Eye Institute, Miami, Florida. Inquiries to Janet L. Davis, MD, Department of Ophthalmology, University of Miami, School of Medicine, Bascom Palmer Eye Institute, 900 NW 17th St., Miami, FL 33136; fax: (305) 326- 6417; e-mail: [email protected] OF
OPHTHALMOLOGY
OCTOBER 2003
FIGURE 1. Case 1. (A) Fundus photograph, right eye, illustrates multiple yellow subretinal lesions 10 weeks after onset at the time of the first optical coherence tomogram (OCT). The macula appears elevated with subretinal fluid. (Top inset) Horizontal OCT 2 scan shows loss of the foveal contour and anterior displacement of the photoreceptor layer by a hypereflective area overlying a hyporeflective space (arrow head) that appears to be above the plane of the retinal pigment epithelium. (Bottom inset) Horizontal OCT 2 scan 20 weeks after onset shows some restoration of the foveal contour, persistence of the highly reflective subretinal layer, and disappearance of the hyporeflective space. (B) Fundus photograph, right eye, shows fading of the posterior pole lesions 8 months after onset. There is a mild pigmentary change in the center of the macula with a partial ring of subretinal deposit. (Top inset) Horizontal OCT 3 scan shows marked resolution with a residual hypereflective subretinal deposit corresponding to the temporal crescent seen in the color photograph. (Bottom inset) Horizontal OCT 3 scan corresponding to the scan line in (B), through two perimacular lesions reveals nodular subretinal deposits without anterior or posterior displacement of the retinal pigment epithelium– choriocapillaris complex.
● CASE 1:
A 43-year-old healthy Caucasian woman experienced sudden onset of bilateral ring scotomas and dim vision. Seven days later, the scotomas faded, but she noticed central blurring. Medical and family histories were unremarkable. Vision was 20/50 uncorrected in both eyes with no inflammation and normal visual fields. Retinal examination revealed bilaterally symmetric, subfoveal, 1500-m circular yellow deposits. Creamy yellow subretinal deposits, 200 m to 500 m, encircled the posterior poles. Fluorescein angiography showed early and late faint
VOL. 136, NO. 4
hyperfluorescence corresponding to the lesions. The subfoveal lesion showed no abnormal fluorescence. Ten weeks later vision was 20/25 in both eyes with hyperopic correction. The centers of the maculas appeared elevated with subretinal fluid. The yellow spots surrounding each macula were more conspicuous and compact (Figure 1A). Fluorescein and indocyanine green angiography were unremarkable. Optical coherence tomography revealed anterior displacement of the photoreceptor layer by a hypereflective layer corresponding to the macular and perimacular le-
BRIEF REPORTS
761
FIGURE 2. Case 2. (A) Fundus photograph, right eye, illustrates multifocal, vitelliform yellow lesions along the vascular arcades. The macular center shows a circular, well-delineated macular elevation. (Inset) Vertical optical coherence tomogram (OCT) 2 scan reveals anterior displacement of the photoreceptor layer by a hypereflective layer overlying a hyporeflective space (arrowhead) above the retinal pigment epithelium. Attenuation of the retinal pigment epithelium reflection is seen due to shadowing by the overlying hypereflective deposit. (B) Four months after initial examination, the central macular lesion is barely visible as a yellow pigmentary disruption. The perimacular lesions have subsided. (Inset) Vertical OCT 3 scan shows marked flattening of the subretinal deposit.
sions. In addition, a hyporeflective space was noted underneath the hypereflective layer and above the retinal pigment epithelium in each macular center (Figure 1A, top inset). Reexamination 20 weeks after onset showed further compaction of the lesions. The hyporeflective space under the fovea was absent and the photoreceptor layer less elevated (Figure 1A, bottom inset). An electroretinogram was normal and an electrooculogram was abnormal with an Arden ratio of 1.54 and 1.46. Further flattening of the lesions was noted 8 months after onset (Figure 1, B, and insets), with reversion to plano correction and 20/25 visual acuity. Repeat electrooculogram showed no improvement in light peak:dark trough ratios. Genotyping of the bestrophin gene revealed no known mutations.
antiretroviral treatment; her HIV status was thought to be incidental. The lesions resolved over 4 months (Figure 2B); vision improved from 20/60 to 20/40. The electrooculogram was unchanged. In 1988, Gass reported two patients with sudden onset of submacular exudative detachments and perimacular deposits that resembled Best macular dystrophy.1 A third case of acute exudative polymorphous vitelliform maculopathy was indexed in the Atlas.2 There was no family history of eye disease, and disruption of the vitelliform lesions occurred rapidly. As in Best macular dystrophy, light:dark ratios of the electrooculogram were subnormal. The authors speculated that acute exudative polymorphous vitelliform maculopathy could either be acquired or an inherited dystrophy.1,2 When examined by OCT, the serous macular detachment described in prior cases1,2 was found to be anterior displacement of the photoreceptor layer by a hypereflective layer located over a hyporeflective space above the retinal pigment epithelium. This is similar to the OCT findings in adult-onset foveomacular vitelliform dystrophy3 and distinct from Best macular dystrophy, which is characterized by subneurosensory retina fluid.4 The hyporeflective space above the retinal pigment epithelium in the early stages of
● CASE 2:
A case with identical clinical, angiographic, and OCT features was seen in a 62-year-old HIV-positive woman. Bilaterally symmetric, multiple vitelliform lesions, 200 m to 500 m, were present within the arcades with central 1,500 m vitelliform macular lesions (Figure 2A). The electrooculogram light peak:dark trough ratios were 0.95 and 1.02. There was no chorioretinitis or HIV retinopathy, and CD4 count was 347 on highly active
762
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
OCTOBER 2003
acute exudative polymorphous vitelliform maculopathy probably indicates shadowing by the dense subretinal deposit in the initial stages of the disorder. Resolution of the hyporeflective space precedes resolution of the macular lesions and improvement in vision. Multiple perimacular lesions, lack of family history, sudden onset, and rapid resolution make adult-onset foveomacular vitelliform dystrophy unlikely, despite the similarity of OCT findings. Best macular dystrophy, adult-onset foveomacular vitelliform dystrophy, and acute exudative polymorphous vitelliform maculopathy may each share retinal pigment epithelium dysfunction initiated by different stimuli. Mutations in the VMD2 gene are associated with Best macular dystrophy,5 and novel mutations occur in maculopathies other than Best macular dystrophy, including adult-onset foveomacular vitelliform dystrophy.5,6 In Best macular dystrophy, accumulation of lipofuscin in dysfunctional retinal pigment epithelium cells is the probable cause of the abnormal electrooculogram and the accumulation of subretinal fluid. In acute exudative polymorphous vitelliform maculopathy, acquired retinal pigment epithelium dysfunction may cause deposition of subretinal material, as shown by OCT, that is similar to the pseudovitelliform material of adult-onset foveomacular vitelliform dystrophy. Interestingly, no recovery of the electrooculogram occurred in our patients despite improvement in vision, fundus, and OCT findings. Acute exudative polymorphous vitelliform maculopathy may occur in predisposed patients with abnormal retinal pigment epithelium after an unknown trigger.
REFERENCES
1. Gass JDM, Chuang EL, Granek H. Acute exudative polymorphous vitelliform maculopathy. Trans Am Ophthalmol Soc 1988;86:354 –366. 2. Gass JDM. Stereoscopic atlas of macular diseases: diagnosis and treatment, 4th ed. St Louis: CV Mosby, 1997:168 – 169,312–313,696 – 697. 3. Benhamou N, Souied EH, Zolf R, Coscas F, Coscas G, Soubrane G. Adult-onset foveomacular vitelliform dystrophy: a study by optical coherence tomography. Am J Ophthalmol 2003;135:362–367. 4. Pierro L, Tremolada G, Introini U, Calori G, Brancato R. Optical coherence tomography findings in adult-onset foveomacular vitelliform dystrophy. Am J Ophthalmol 2002;134: 675–680. 5. Kramer F, White K, Pauleikhoff D, et al. Mutations in the VMD2 gene are associated with juvenile-onset vitelliform macular dystrophy (Best disease) and adult vitelliform macular dystrophy but not age-related macular degeneration. Eur J Hum Genet 2000;8:286 –292. 6. Seddon JM, Afshari MA, Sharma S, et al. Assessment of mutations in the Best macular dystrophy (VMD2) gene in patients with adult-onset foveomacular vitelliform dystrophy, age-related maculopathy, and bull’s-eye maculopathy. Ophthalmology 2001;108:2060 –2067.
VOL. 136, NO. 4
Epiretinal Membrane Formation With Internal Limiting Membrane Wrinkling After Nd:YAG Laser Membranotomy in Valsalva Retinopathy Alvin K. H. Kwok, FRCS, Timothy Y. Y. Lai, MRCS, and Nongnart R. Chan, MD
PURPOSE: To report the location of premacular hemorrhage in Valsalva retinopathy and epiretinal membrane formation with internal limiting membrane (ILM) wrinkling after neodymium;yttrium-aluminum-garnet (Nd: YAG) laser membranotomy. DESIGN: Interventional case report. METHODS: A 35-year-old man with a massive premacular hemorrhage due to Valsalva retinopathy underwent Nd: YAG laser membranotomy. After membranotomy, his visual acuity improved from 20/200 to 20/25. Ten months later, he developed metamorphopsia with visual acuity of 20/30. A semiopacified membrane with radiating striae was found, and pars plana vitrectomy was performed for membrane removal. RESULTS: Histologic examination of the membrane showed hemosiderin deposits within macrophages on the retinal side of the ILM. One year later, the patient was asymptomatic with visual acuity of 20/20. CONCLUSIONS: To our knowledge, histopathologic confirmation of the sub-ILM hemorrhage in Valsalva retinopathy and epiretinal membrane formation with ILM wrinkling as a complication after Nd:YAG laser membranotomy have not been previously reported. (Am J Ophthalmol 2003;136:763–766. © 2003 by Elsevier Inc. All rights reserved.)
T
HE EXACT ANATOMIC LOCATION OF PREMACULAR
hemorrhage in Valsalva retinopathy, either subinternal limiting membrane (ILM) or subhyaloid, or a combination of both, is not yet defined.1 We treated a patient
Accepted for publication April 11, 2003. From the Department of and Ophthalmology, The Hong Kong Sanatorium and Hospital, Hong Kong, People’s Republic of China (A.K.H.K.); Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong Eye Hospital, Hong Kong, People’s Republic of China (A.K.H.K., T.Y.Y.L., N.R.C.). Inquiries to Alvin K. H. Kwok, FRCS, Department of Ophthalmology, Hong Kong Sanatorium and Hospital, 2 Village Road, Happy Valley, Hong Kong, China; fax: (⫹852) 2835 8008; e-mail: [email protected]
BRIEF REPORTS
763
The management of RD in SW syndrome is often difficult. Surgery in these eyes is extremely dangerous because of the risk of hemorrhage from the dilated abnormal episcleral and choroidal vessels. The traditional method of therapy using external beam radiation is effective; however, there is a slow absorption of the serous RD, usually taking several months.1 Often, there is a recurrence or persistence of the fluid, necessitating additional external beam radiation therapy, which carries risk of radiation retinopathy, cataract formation, and optic neuropathy. Plaque brachytherapy has been suggested in large circumscribed hemangiomas;2 however, this modality is ineffective in an eye with a diffuse hemangioma occupying the whole choroid. Recent reports have shown success with transpupillary thermotherapy, which is effective only in small, localized tumors and may need to be repeated.3 Photodynamic therapy may be the current treatment of choice for small circumscribed choroidal hemangiomas, especially those located around the optic nerve and macular area.4,5 To our knowledge, this is the first report of successful and rapid resolution of RD in SW syndrome in an eye that had failed conventional external beam radiation therapy. The slow blood flow and capillary dilation in hemangiomas lends itself to a successful closure using a relatively noninvasive procedure. The treatment itself carries a relatively low risk, and the rapid shrinkage in the size of the hemangioma is impressive. Multiple spots of treatment were necessary to cover the majority of the lesion because of the large size of the tumor; however, we were careful to avoid overlapping spots by following anatomic landmarks. We do have some concern about the resultant pigment scarring, though it may provide a mechanical barrier to recurrent accumulation of subretinal fluid. The patient had a long-standing retinal detachment and amblyopia, which limit the recovery of central vision; however, there were very limited therapeutic options available in this scenario. Further trials are warranted to evaluate the appropriate first therapy of choice in serous RD with the SW syndrome.
Vanessa Cruz-Villegas, MD, Natalia Villate, MD, Robert W. Knighton, PhD, Patrick Rubsamen, MD, and Janet L. Davis, MD To report two cases of acute exudative polymorphous vitelliform maculopathy. DESIGN: Observational case reports. METHODS: Fluorescein angiography, optical coherence tomography, and electrophysiology were performed. RESULTS: Both patients presented with sudden onset of blurred vision and bilaterally symmetric, central, and perimacular vitelliform lesions. Perimacular lesions were faintly hyperfluorescent early in the disease, but there was no abnormal fluorescence in the macular centers. Optical coherence tomography revealed anterior displacement of the photoreceptor layer by a hypereflective subretinal layer overlying a hyporeflective space above the retinal pigment epithelium– choriocapillaris complex under all lesions and no subretinal fluid. The electrooculogram was abnormal. Near complete resolution occurred over 4 to 8 months, with no improvement in the electrooculogram. CONCLUSIONS: Transient multifocal vitelliform lesions suggest a diagnosis of acute exudative polymorphous vitelliform maculopathy. Optical coherence tomography reveals characteristic anterior displacement of the photoreceptor layer by a subretinal hypereflective deposit similar to that seen in adult-onset foveomacular vitelliform dystrophy. (Am J Ophthalmol 2003;136: 760 –763. © 2003 by Elsevier Inc. All rights reserved.) PURPOSE:
A
REFERENCES
1. Gottleib JL, Murray TG, Gass JD. Low dose external beam irradiation for choroidal hemangiomas. Arch Ophthalmol 1998;116:815–817. 2. Chao AN, Shields CL, Shields JA, Krema H. Plaque radiotherapy for choroidal hemangioma with total retinal detachment and iris neovascularization. Retina 2001;21:682–684. 3. Fuch AV, Mueller AJ, Gruetrich M, Ubig MW. Transpupillary thermotherapy (TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Ophthalmol 2002;240: 7–11. 4. Robertson DM. Photodynamic therapy for choroidal hemangioma associated with serous retinal detachment. Arch Ophthalmol 2002;120:1155–1161. 5. Schmidt-Erfruth UM, Michels S, Kusserow C, Jurkles B, Augustin AJ. Photodynamic therapy for symptomatic choroidal hemangioma: visual and anatomic results. Ophthalmology 2002;109:2284 –2294.
760
AMERICAN JOURNAL
CUTE
EXUDATIVE
POLYMORPHOUS
VITELLIFORM
maculopathy is a rare disorder characterized by the sudden onset of bilateral, yellow-gray subretinal deposits in the macular area that resemble the lesions of Best disease but rapidly subside. We describe the fourth and fifth published cases with the first report of optical coherence tomography (OCT) findings. Review was undertaken according to the guidelines of the Human Subjects Research Subcommittee.
Accepted for publication April 7, 2003. From the Department of Ophthalmology, University of Miami, School of Medicine, Bascom Palmer Eye Institute, Miami, Florida. Inquiries to Janet L. Davis, MD, Department of Ophthalmology, University of Miami, School of Medicine, Bascom Palmer Eye Institute, 900 NW 17th St., Miami, FL 33136; fax: (305) 326- 6417; e-mail: [email protected] OF
OPHTHALMOLOGY
OCTOBER 2003
FIGURE 1. Case 1. (A) Fundus photograph, right eye, illustrates multiple yellow subretinal lesions 10 weeks after onset at the time of the first optical coherence tomogram (OCT). The macula appears elevated with subretinal fluid. (Top inset) Horizontal OCT 2 scan shows loss of the foveal contour and anterior displacement of the photoreceptor layer by a hypereflective area overlying a hyporeflective space (arrow head) that appears to be above the plane of the retinal pigment epithelium. (Bottom inset) Horizontal OCT 2 scan 20 weeks after onset shows some restoration of the foveal contour, persistence of the highly reflective subretinal layer, and disappearance of the hyporeflective space. (B) Fundus photograph, right eye, shows fading of the posterior pole lesions 8 months after onset. There is a mild pigmentary change in the center of the macula with a partial ring of subretinal deposit. (Top inset) Horizontal OCT 3 scan shows marked resolution with a residual hypereflective subretinal deposit corresponding to the temporal crescent seen in the color photograph. (Bottom inset) Horizontal OCT 3 scan corresponding to the scan line in (B), through two perimacular lesions reveals nodular subretinal deposits without anterior or posterior displacement of the retinal pigment epithelium– choriocapillaris complex.
● CASE 1:
A 43-year-old healthy Caucasian woman experienced sudden onset of bilateral ring scotomas and dim vision. Seven days later, the scotomas faded, but she noticed central blurring. Medical and family histories were unremarkable. Vision was 20/50 uncorrected in both eyes with no inflammation and normal visual fields. Retinal examination revealed bilaterally symmetric, subfoveal, 1500-m circular yellow deposits. Creamy yellow subretinal deposits, 200 m to 500 m, encircled the posterior poles. Fluorescein angiography showed early and late faint
VOL. 136, NO. 4
hyperfluorescence corresponding to the lesions. The subfoveal lesion showed no abnormal fluorescence. Ten weeks later vision was 20/25 in both eyes with hyperopic correction. The centers of the maculas appeared elevated with subretinal fluid. The yellow spots surrounding each macula were more conspicuous and compact (Figure 1A). Fluorescein and indocyanine green angiography were unremarkable. Optical coherence tomography revealed anterior displacement of the photoreceptor layer by a hypereflective layer corresponding to the macular and perimacular le-
BRIEF REPORTS
761
FIGURE 2. Case 2. (A) Fundus photograph, right eye, illustrates multifocal, vitelliform yellow lesions along the vascular arcades. The macular center shows a circular, well-delineated macular elevation. (Inset) Vertical optical coherence tomogram (OCT) 2 scan reveals anterior displacement of the photoreceptor layer by a hypereflective layer overlying a hyporeflective space (arrowhead) above the retinal pigment epithelium. Attenuation of the retinal pigment epithelium reflection is seen due to shadowing by the overlying hypereflective deposit. (B) Four months after initial examination, the central macular lesion is barely visible as a yellow pigmentary disruption. The perimacular lesions have subsided. (Inset) Vertical OCT 3 scan shows marked flattening of the subretinal deposit.
sions. In addition, a hyporeflective space was noted underneath the hypereflective layer and above the retinal pigment epithelium in each macular center (Figure 1A, top inset). Reexamination 20 weeks after onset showed further compaction of the lesions. The hyporeflective space under the fovea was absent and the photoreceptor layer less elevated (Figure 1A, bottom inset). An electroretinogram was normal and an electrooculogram was abnormal with an Arden ratio of 1.54 and 1.46. Further flattening of the lesions was noted 8 months after onset (Figure 1, B, and insets), with reversion to plano correction and 20/25 visual acuity. Repeat electrooculogram showed no improvement in light peak:dark trough ratios. Genotyping of the bestrophin gene revealed no known mutations.
antiretroviral treatment; her HIV status was thought to be incidental. The lesions resolved over 4 months (Figure 2B); vision improved from 20/60 to 20/40. The electrooculogram was unchanged. In 1988, Gass reported two patients with sudden onset of submacular exudative detachments and perimacular deposits that resembled Best macular dystrophy.1 A third case of acute exudative polymorphous vitelliform maculopathy was indexed in the Atlas.2 There was no family history of eye disease, and disruption of the vitelliform lesions occurred rapidly. As in Best macular dystrophy, light:dark ratios of the electrooculogram were subnormal. The authors speculated that acute exudative polymorphous vitelliform maculopathy could either be acquired or an inherited dystrophy.1,2 When examined by OCT, the serous macular detachment described in prior cases1,2 was found to be anterior displacement of the photoreceptor layer by a hypereflective layer located over a hyporeflective space above the retinal pigment epithelium. This is similar to the OCT findings in adult-onset foveomacular vitelliform dystrophy3 and distinct from Best macular dystrophy, which is characterized by subneurosensory retina fluid.4 The hyporeflective space above the retinal pigment epithelium in the early stages of
● CASE 2:
A case with identical clinical, angiographic, and OCT features was seen in a 62-year-old HIV-positive woman. Bilaterally symmetric, multiple vitelliform lesions, 200 m to 500 m, were present within the arcades with central 1,500 m vitelliform macular lesions (Figure 2A). The electrooculogram light peak:dark trough ratios were 0.95 and 1.02. There was no chorioretinitis or HIV retinopathy, and CD4 count was 347 on highly active
762
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
OCTOBER 2003
acute exudative polymorphous vitelliform maculopathy probably indicates shadowing by the dense subretinal deposit in the initial stages of the disorder. Resolution of the hyporeflective space precedes resolution of the macular lesions and improvement in vision. Multiple perimacular lesions, lack of family history, sudden onset, and rapid resolution make adult-onset foveomacular vitelliform dystrophy unlikely, despite the similarity of OCT findings. Best macular dystrophy, adult-onset foveomacular vitelliform dystrophy, and acute exudative polymorphous vitelliform maculopathy may each share retinal pigment epithelium dysfunction initiated by different stimuli. Mutations in the VMD2 gene are associated with Best macular dystrophy,5 and novel mutations occur in maculopathies other than Best macular dystrophy, including adult-onset foveomacular vitelliform dystrophy.5,6 In Best macular dystrophy, accumulation of lipofuscin in dysfunctional retinal pigment epithelium cells is the probable cause of the abnormal electrooculogram and the accumulation of subretinal fluid. In acute exudative polymorphous vitelliform maculopathy, acquired retinal pigment epithelium dysfunction may cause deposition of subretinal material, as shown by OCT, that is similar to the pseudovitelliform material of adult-onset foveomacular vitelliform dystrophy. Interestingly, no recovery of the electrooculogram occurred in our patients despite improvement in vision, fundus, and OCT findings. Acute exudative polymorphous vitelliform maculopathy may occur in predisposed patients with abnormal retinal pigment epithelium after an unknown trigger.
REFERENCES
1. Gass JDM, Chuang EL, Granek H. Acute exudative polymorphous vitelliform maculopathy. Trans Am Ophthalmol Soc 1988;86:354 –366. 2. Gass JDM. Stereoscopic atlas of macular diseases: diagnosis and treatment, 4th ed. St Louis: CV Mosby, 1997:168 – 169,312–313,696 – 697. 3. Benhamou N, Souied EH, Zolf R, Coscas F, Coscas G, Soubrane G. Adult-onset foveomacular vitelliform dystrophy: a study by optical coherence tomography. Am J Ophthalmol 2003;135:362–367. 4. Pierro L, Tremolada G, Introini U, Calori G, Brancato R. Optical coherence tomography findings in adult-onset foveomacular vitelliform dystrophy. Am J Ophthalmol 2002;134: 675–680. 5. Kramer F, White K, Pauleikhoff D, et al. Mutations in the VMD2 gene are associated with juvenile-onset vitelliform macular dystrophy (Best disease) and adult vitelliform macular dystrophy but not age-related macular degeneration. Eur J Hum Genet 2000;8:286 –292. 6. Seddon JM, Afshari MA, Sharma S, et al. Assessment of mutations in the Best macular dystrophy (VMD2) gene in patients with adult-onset foveomacular vitelliform dystrophy, age-related maculopathy, and bull’s-eye maculopathy. Ophthalmology 2001;108:2060 –2067.
VOL. 136, NO. 4
Epiretinal Membrane Formation With Internal Limiting Membrane Wrinkling After Nd:YAG Laser Membranotomy in Valsalva Retinopathy Alvin K. H. Kwok, FRCS, Timothy Y. Y. Lai, MRCS, and Nongnart R. Chan, MD
PURPOSE: To report the location of premacular hemorrhage in Valsalva retinopathy and epiretinal membrane formation with internal limiting membrane (ILM) wrinkling after neodymium;yttrium-aluminum-garnet (Nd: YAG) laser membranotomy. DESIGN: Interventional case report. METHODS: A 35-year-old man with a massive premacular hemorrhage due to Valsalva retinopathy underwent Nd: YAG laser membranotomy. After membranotomy, his visual acuity improved from 20/200 to 20/25. Ten months later, he developed metamorphopsia with visual acuity of 20/30. A semiopacified membrane with radiating striae was found, and pars plana vitrectomy was performed for membrane removal. RESULTS: Histologic examination of the membrane showed hemosiderin deposits within macrophages on the retinal side of the ILM. One year later, the patient was asymptomatic with visual acuity of 20/20. CONCLUSIONS: To our knowledge, histopathologic confirmation of the sub-ILM hemorrhage in Valsalva retinopathy and epiretinal membrane formation with ILM wrinkling as a complication after Nd:YAG laser membranotomy have not been previously reported. (Am J Ophthalmol 2003;136:763–766. © 2003 by Elsevier Inc. All rights reserved.)
T
HE EXACT ANATOMIC LOCATION OF PREMACULAR
hemorrhage in Valsalva retinopathy, either subinternal limiting membrane (ILM) or subhyaloid, or a combination of both, is not yet defined.1 We treated a patient
Accepted for publication April 11, 2003. From the Department of and Ophthalmology, The Hong Kong Sanatorium and Hospital, Hong Kong, People’s Republic of China (A.K.H.K.); Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong Eye Hospital, Hong Kong, People’s Republic of China (A.K.H.K., T.Y.Y.L., N.R.C.). Inquiries to Alvin K. H. Kwok, FRCS, Department of Ophthalmology, Hong Kong Sanatorium and Hospital, 2 Village Road, Happy Valley, Hong Kong, China; fax: (⫹852) 2835 8008; e-mail: [email protected]
BRIEF REPORTS
763