SUCCESSFUL TREATMENT OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO PERSISTENT PLACOID MACULOPATHY WITH INTRAVITREAL BEVACIZUMAB James A. Eadie, MD, Justin L. Gottlieb, MD
Purpose: To report a case of posterior placoid maculopathy with secondary choroidal neovascularization that responded favorably to repeat intravitreal injections of bevacizumab with long-term follow-up. Methods: Patient data from all clinic visits were reviewed. These include visual acuity measurements and clinical examination findings, optical coherence tomography, color fundus photography, and fluorescein angiography images from the initial visit and follow-up encounters. Results: This patient showed clear evidence of active leakage from choroidal neovascularization on presentation that promptly resolved with treatment. After his initial course of injections, all evidence of active exudation disappeared from his optical coherence tomography scans. However, when treatment was extended beyond 8 weeks, choroidal neovascularization leakage returned. In addition, the patient developed subretinal fibrosis in one eye despite regular treatments. Nevertheless, visual acuity has remained excellent through his most recent encounter. Conclusion: Persistent placoid maculopathy is an exceedingly rare clinical entity. In their original descriptive series, Golchet et al revealed that 9 of the 12 eyes went on to develop poor vision from complications of choroidal neovascularization. There is currently only one case in the literature that describes the use of anti–vascular endothelial growth factor medications in the treatment of persistent placoid maculopathy with promising results. This case adds supporting evidence for this treatment modality and highlights the need for continued follow-up and treatment till 18 months from presentation. RETINAL CASES & BRIEF REPORTS 8:37–40, 2014
well-defined early hypofluorescent areas that partially filled in the late phase.”1 They distinguished this entity from serpiginous choroiditis by the relative preservation of good vision in the early stages of the disease until complications from choroidal neovascularization (CNV) develop. In their original series of 6 patients, 11 of 12 of eyes (91%) developed CNV in contrast to 35% of eyes with serpiginous choroiditis described in the literature.1,2 The patients described in the initial report were treated with a variety of therapies including sub-Tenon steroid injections, photodynamic therapy, oral steroids, and thermal laser with limited success.1
From the Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, Wisconsin.
G
olchet et al1 first defined persistent placoid maculopathy (PPM) as a distinct clinical entity, separate from serpiginous choroiditis in 2007. They described “well-delineated whitish plaquelike lesions involving the macula and sparing the peri-papillary areas of both eyes. Fluorescein angiography revealed None of the authors have any financial/conflicting interests to disclose. Reprint requests: James A. Eadie, MD, Department of Ophthalmology and Visual Sciences, University of Wisconsin, 2880 University Avenue, Madison, WI 53705-3611; e-mail: [email protected]
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We present a case of PPM, followed for 18 months, and treated with regular bevacizumab injections with excellent preservation of visual acuity.
Case Report A 46-year-old man was referred to the University of Wisconsin Retina clinic in September 2011 with several weeks of decreasing vision, distortion, and monocular diplopia in the right eye. His medical history was remarkable for hypertension, type II diabetes mellitus, hypothyroidism, and nonalcoholic fatty liver. His past ocular history was noncontributory. Medications included hydrochlorothiazide, lisinopril, synthroid, and metformin. On examination, his vision was 20/30−1 in the right eye and 20/20 in the left eye. Pupils, intraocular pressure, and anterior slit-lamp examination
Fig. 1. Color fundus photographs of both eyes with early and late phases of fluorescein angiography at presentation.
were all unremarkable. Fundus biomicroscopic examination showed bilateral hypopigmented macular plaques with areas of irregular pigmentation and with bilateral CNV (Figure 1). On fluorescein angiography, these macular plaques were hypofluorescent in the early phase with late hyperfluorescence. Optical coherence tomography images showed subretinal neovascularization and scar tissue with focal thickening in both eyes and subretinal fluid in the left eye (Figure 2). The clinical picture was consistent with PPM and bilateral CNV. The patient was treated with a series of 3 bilateral bevacizumab injections at 4 weeks intervals. Repeat fluorescein angiography 3 months after presentation showed redemonstration of bilateral macular plaques with staining but no obvious leakage. The patient’s symptoms improved, but he continued to note mild metamorphopsia. Vision has fluctuated from 20/20 to 20/40 in both eyes on subsequent follow-up examinations. He has undergone continued bevacizumab injections for mild recurrent retinal edema on follow-up optical
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BEVACIZUMAB FOR CNV SECONDARY TO PPM
coherence tomography images. Anatomically, as is often seen with regressed CNV, he developed subretinal pigment epithelial fibrosis more in the right eye than in the left despite regular treatment. Additionally, multiple drusenoid deposits developed under the retinal pigment epithelium that coincided with subtle hypopigmented spots on ophthalmoscopy. The pathophysiology of these deposits is unclear but may be indicative of abnormal underlying retinal/retinal pigment epithelium metabolism. Before his last examination, the treatment interval was extended from 10 weeks to 12 weeks. With this increase, the patient returned with asymptomatic new hemorrhage and exudation in the right eye (Figures 3 and 4) though vision remained excellent at 20/30.
Discussion
Fig. 2. Optical coherence tomography images of both eyes at presentation.
Fig. 3. Color fundus photography of both eyes with early and late fluorescein angiography at 18 months.
Persistent placoid maculopathy is an exceedingly rare clinical entity. It bears resemblance to serpiginous choroiditis and acute posterior multifocal placoid pigment epitheliopathy with several key distinguishing characteristics. In their original descriptive series, Golchet et al revealed that 9 of the 12 eyes went on to develop poor vision from complications of CNV. However, these eyes presented and were treated in the pre–vascular endothelial growth factor era. There is only one case in the literature that describes the use of anti–vascular endothelial growth factor medications in the treatment of PPM with promising results but limited follow-up.3 This case adds supporting evidence for the use of anti–vascular endothelial growth factor
Fig. 4. Optical coherence tomography images of both eyes at 18 months. The images depict subretinal pigment epithelial drusenoid deposits, fibrosis more in the right eye than in the left eye, and active exudation in the right eye.
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RETINAL CASES & BRIEF REPORTS´ 2014 VOLUME 8 NUMBER 1
medications in the treatment of PPM complicated by CNV. The clinical course has, thus far, been followed for 18 months. With extension of time between bevacizumab injections to 12 weeks, there was reactivation of CNV though vision was relatively preserved. It has been proposed that there exists an underlying choroidal hypoperfusion that accounts for the early hypofluorescence on fluorescein angiography and indocyanine green angiography.4 This presumed hypoperfusion has persisted and was redemonstrated on his last fluorescein angiography, 18 months from presentation (Figure 3). This presumed persistent hypoperfusion may be the inciting stimulus for the late recurrence of active CNV with extension of the treatment interval. This case highlights the potential for long-term visual decline, despite initial satisfactory management of neovascular complications and the need for extended
follow-up and treatment for patients with this disease entity. Key words: bevacizumab, persistent placoid maculopathy, choroidal neovascularization. References 1. Golchet PR, Jampol LM, Wilson D, et al. Persistent placoid maculopathy: a new clinical entity. Ophthalmology 2007;114: 1530–1540. 2. Christmas NJ, Oh KT, Oh DM, Folk JC. Long-term follow-up of patients with serpiginous choroiditis. Retina 2002;22: 550–556. 3. Parodi MB, Iacono P, Bandello F. Juxtafoveal choroidal neovascularization secondary to persistent placoid maculopathy treated with intravitreal bevacizumab. Ocul Immunol Inflamm 2010;18:399–401. 4. Khairallah M, Ben Yahia S. Persistent placoid maculopathy. Ophthalmology 2008;115:220–221.
Purpose: To report a case of posterior placoid maculopathy with secondary choroidal neovascularization that responded favorably to repeat intravitreal injections of bevacizumab with long-term follow-up. Methods: Patient data from all clinic visits were reviewed. These include visual acuity measurements and clinical examination findings, optical coherence tomography, color fundus photography, and fluorescein angiography images from the initial visit and follow-up encounters. Results: This patient showed clear evidence of active leakage from choroidal neovascularization on presentation that promptly resolved with treatment. After his initial course of injections, all evidence of active exudation disappeared from his optical coherence tomography scans. However, when treatment was extended beyond 8 weeks, choroidal neovascularization leakage returned. In addition, the patient developed subretinal fibrosis in one eye despite regular treatments. Nevertheless, visual acuity has remained excellent through his most recent encounter. Conclusion: Persistent placoid maculopathy is an exceedingly rare clinical entity. In their original descriptive series, Golchet et al revealed that 9 of the 12 eyes went on to develop poor vision from complications of choroidal neovascularization. There is currently only one case in the literature that describes the use of anti–vascular endothelial growth factor medications in the treatment of persistent placoid maculopathy with promising results. This case adds supporting evidence for this treatment modality and highlights the need for continued follow-up and treatment till 18 months from presentation. RETINAL CASES & BRIEF REPORTS 8:37–40, 2014
well-defined early hypofluorescent areas that partially filled in the late phase.”1 They distinguished this entity from serpiginous choroiditis by the relative preservation of good vision in the early stages of the disease until complications from choroidal neovascularization (CNV) develop. In their original series of 6 patients, 11 of 12 of eyes (91%) developed CNV in contrast to 35% of eyes with serpiginous choroiditis described in the literature.1,2 The patients described in the initial report were treated with a variety of therapies including sub-Tenon steroid injections, photodynamic therapy, oral steroids, and thermal laser with limited success.1
From the Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, Wisconsin.
G
olchet et al1 first defined persistent placoid maculopathy (PPM) as a distinct clinical entity, separate from serpiginous choroiditis in 2007. They described “well-delineated whitish plaquelike lesions involving the macula and sparing the peri-papillary areas of both eyes. Fluorescein angiography revealed None of the authors have any financial/conflicting interests to disclose. Reprint requests: James A. Eadie, MD, Department of Ophthalmology and Visual Sciences, University of Wisconsin, 2880 University Avenue, Madison, WI 53705-3611; e-mail: [email protected]
37
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RETINAL CASES & BRIEF REPORTS´ 2014 VOLUME 8 NUMBER 1
We present a case of PPM, followed for 18 months, and treated with regular bevacizumab injections with excellent preservation of visual acuity.
Case Report A 46-year-old man was referred to the University of Wisconsin Retina clinic in September 2011 with several weeks of decreasing vision, distortion, and monocular diplopia in the right eye. His medical history was remarkable for hypertension, type II diabetes mellitus, hypothyroidism, and nonalcoholic fatty liver. His past ocular history was noncontributory. Medications included hydrochlorothiazide, lisinopril, synthroid, and metformin. On examination, his vision was 20/30−1 in the right eye and 20/20 in the left eye. Pupils, intraocular pressure, and anterior slit-lamp examination
Fig. 1. Color fundus photographs of both eyes with early and late phases of fluorescein angiography at presentation.
were all unremarkable. Fundus biomicroscopic examination showed bilateral hypopigmented macular plaques with areas of irregular pigmentation and with bilateral CNV (Figure 1). On fluorescein angiography, these macular plaques were hypofluorescent in the early phase with late hyperfluorescence. Optical coherence tomography images showed subretinal neovascularization and scar tissue with focal thickening in both eyes and subretinal fluid in the left eye (Figure 2). The clinical picture was consistent with PPM and bilateral CNV. The patient was treated with a series of 3 bilateral bevacizumab injections at 4 weeks intervals. Repeat fluorescein angiography 3 months after presentation showed redemonstration of bilateral macular plaques with staining but no obvious leakage. The patient’s symptoms improved, but he continued to note mild metamorphopsia. Vision has fluctuated from 20/20 to 20/40 in both eyes on subsequent follow-up examinations. He has undergone continued bevacizumab injections for mild recurrent retinal edema on follow-up optical
39
BEVACIZUMAB FOR CNV SECONDARY TO PPM
coherence tomography images. Anatomically, as is often seen with regressed CNV, he developed subretinal pigment epithelial fibrosis more in the right eye than in the left despite regular treatment. Additionally, multiple drusenoid deposits developed under the retinal pigment epithelium that coincided with subtle hypopigmented spots on ophthalmoscopy. The pathophysiology of these deposits is unclear but may be indicative of abnormal underlying retinal/retinal pigment epithelium metabolism. Before his last examination, the treatment interval was extended from 10 weeks to 12 weeks. With this increase, the patient returned with asymptomatic new hemorrhage and exudation in the right eye (Figures 3 and 4) though vision remained excellent at 20/30.
Discussion
Fig. 2. Optical coherence tomography images of both eyes at presentation.
Fig. 3. Color fundus photography of both eyes with early and late fluorescein angiography at 18 months.
Persistent placoid maculopathy is an exceedingly rare clinical entity. It bears resemblance to serpiginous choroiditis and acute posterior multifocal placoid pigment epitheliopathy with several key distinguishing characteristics. In their original descriptive series, Golchet et al revealed that 9 of the 12 eyes went on to develop poor vision from complications of CNV. However, these eyes presented and were treated in the pre–vascular endothelial growth factor era. There is only one case in the literature that describes the use of anti–vascular endothelial growth factor medications in the treatment of PPM with promising results but limited follow-up.3 This case adds supporting evidence for the use of anti–vascular endothelial growth factor
Fig. 4. Optical coherence tomography images of both eyes at 18 months. The images depict subretinal pigment epithelial drusenoid deposits, fibrosis more in the right eye than in the left eye, and active exudation in the right eye.
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RETINAL CASES & BRIEF REPORTS´ 2014 VOLUME 8 NUMBER 1
medications in the treatment of PPM complicated by CNV. The clinical course has, thus far, been followed for 18 months. With extension of time between bevacizumab injections to 12 weeks, there was reactivation of CNV though vision was relatively preserved. It has been proposed that there exists an underlying choroidal hypoperfusion that accounts for the early hypofluorescence on fluorescein angiography and indocyanine green angiography.4 This presumed hypoperfusion has persisted and was redemonstrated on his last fluorescein angiography, 18 months from presentation (Figure 3). This presumed persistent hypoperfusion may be the inciting stimulus for the late recurrence of active CNV with extension of the treatment interval. This case highlights the potential for long-term visual decline, despite initial satisfactory management of neovascular complications and the need for extended
follow-up and treatment for patients with this disease entity. Key words: bevacizumab, persistent placoid maculopathy, choroidal neovascularization. References 1. Golchet PR, Jampol LM, Wilson D, et al. Persistent placoid maculopathy: a new clinical entity. Ophthalmology 2007;114: 1530–1540. 2. Christmas NJ, Oh KT, Oh DM, Folk JC. Long-term follow-up of patients with serpiginous choroiditis. Retina 2002;22: 550–556. 3. Parodi MB, Iacono P, Bandello F. Juxtafoveal choroidal neovascularization secondary to persistent placoid maculopathy treated with intravitreal bevacizumab. Ocul Immunol Inflamm 2010;18:399–401. 4. Khairallah M, Ben Yahia S. Persistent placoid maculopathy. Ophthalmology 2008;115:220–221.
