Hemorrhagic Pigment Epithelial Detachment Research Articles

Three-dimensional heads-up system assisted pars plana vitrectomy and subretinal recombinant tissue plasminogen activator injection for submacular hemorrhage

BackgroundTo evaluate the outcomes of three-dimensional (3D) heads-up system assisted pars plana vitrectomy (PPV) and subretinal injection of recombinant tissue plasminogen activator (rt-PA) for submacular hemorrhage (SMH).MethodsMedical records of SMH patients who underwent 3D heads-up system assisted-PPV and subretinal injection of rt-PA from June 2021 to January 2022 were reviewed. The main outcomes included best-corrected visual acuity (BCVA), SMH absorption, and perioperative complications.ResultsWe finally included 18 SMH eyes, most of which happened secondary to polypoidal choroidal vasculopathy (PCV) (10, 55.56%), followed by retinal arterial microaneurysm (RAM) (5, 27.78%), traumatic retinopathy (2, 11.11%) and neovascular age-related macular degeneration (nAMD) (1, 5.56%). The greatest linear dimension (GLD) and height of SMH were 6538.17 ± 2533.11 μm and 937.36 ± 420.21 μm, respectively. After an average postoperative follow-up period of 131.06 ± 38.96 days, patients’ BCVA improved significantly from 1.85 ± 0.62 to 1.14 ± 0.82 logMAR (P < 0.05). SMH was completely and partially absorbed in 9 (50.00%) and 6 (33.33%) eyes, with no occurrence of iatrogenic retinal break. However, 4 additional PPVs were performed to manage the postoperative SMH and/or vitreous hemorrhage (VH) recurrence (2, 11.11%) and retinal detachment (RD) occurrence (2, 11.11%). Preoperative BCVA was significantly correlated with postoperative BCVA in multiple linear regression analysis (P < 0.05), and hemorrhagic pigment epithelial detachment (PED) was significantly correlated with SMH and VH recurrence in univariate binary logistic regression analysis (P < 0.05).ConclusionsThe 3D heads-up system assisted-PPV and subretinal injection of rt-PA were efficacious in eliminating SMH and improving visual prognosis with satisfactory safety profile, while caution should be taken for PCV patients with hemorrhagic PED and massive SMH.

A rare case of macular telangiectasia type 2 associated with polypoidal choroidal vasculopathy

Polypoidal choroidal vasculopathy (PCV) was first described as subretinal polypoidal vascular lesions associated with serous and hemorrhagic pigment epithelial detachments. Macular telangiectasia (Mac Tel) refers to a heterogenous group of disease entities presenting with incompetence, ectasia, and/or irregular dilatations of the juxtafoveal capillary network of one or both the eyes. Here, we report a rare case presenting with features of PCV and Mac Tel-2 simultaneously. To the best of our knowledge, this is the second reported case to show this rare clinical association. This may point toward an unifying underlying pathophysiologic mechanism, especially in patients with Mac Tel and thick choroid.

SPATIAL PATTERN OF RETINAL PIGMENT EPITHELIUM TEAR DEVELOPMENT AND PROGRESSION AFTER ANTIVASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY FOR NEOVASCULAR AGE-RELATED MACULAR DEGENERATION.

The aim of this study was to demonstrate the spatial pattern of retinal pigment epithelium (RPE) tear development and progression after antivascular endothelial growth factor therapy for neovascular age-related macular degeneration. We retrospectively reviewed six eyes with neovascular age-related macular degeneration that showed RPE tears after administration of intravitreal antivascular endothelial growth factor agents and were followed up for 12 months. The patterns of RPE tear development and progression were evaluated by analyzing positional relationships among the locations of the choroidal neovascularization membrane and pigment epithelial detachment (PED) area at baseline and the tear area using spectral-domain optical coherence tomography, color photography, fluorescein angiography, and fundus autofluorescence images. Pretear OCT images revealed fibrovascular PED in all eyes, one of which showed complications of hemorrhagic PED after treatment. In five eyes, RPE tears developed at the PED edge located on the opposite side of the choroidal neovascularization membrane. In the eye showing hemorrhagic PED, the RPE tear developed along the wide area of the PED edge. The torn RPE monolayer contracted toward the side of the choroidal neovascularization membrane in all eyes, and RPE loss involved the fovea in five eyes that showed significantly worse visual acuity (VA) after 12 months in comparison with the baseline value before the tear (logMAR VA; 0.3 vs. 1.29; P < 0.02). The location of choroidal neovascularization membrane in PED determines the spatial pattern of RPE tear development and progression and helps to predict the visual outcome after RPE tears.

Ophthalmic Manifestations of Newly Diagnosed Acute Leukemia Patients in a Tunisian Cohort.

PurposeTo describe ocular manifestations of acute leukemia in a Tunisian cohort and to assess the associations between ophthalmic findings and epidemiological, clinical, and biological features of the disease.MethodsA prospective study included patients newly diagnosed with acute leukemia referred to our clinics between January 2019 and July 2020. All patients underwent a complete ophthalmic evaluation and spectral-domain optical coherence tomography (SD-OCT) at presentation, then every two months during one year. We defined two groups: Group 1 included patients with leukemic ophthalmopathy and group 2 included patients with normal ophthalmic examination.ResultsForty-six patients were enrolled. The mean age of patients was 32.1±15.3 years. The sex ratio M/F was 1.55 (28 male patients and 18 females). Twenty-nine patients (63%) had acute myeloid leukemia (AML), and 17 (37%) had acute lymphoblastic leukemia (ALL). The average follow-up was 9.1 months (range: 3–12 months). We observed ophthalmic manifestations in 28 patients (61%). Among them, 17 (61%) had vision-threatening complications. The posterior segment was the most common site of ocular involvement (82% of group1). Primary leukemic infiltration (Disc edema, ptosis, exophthalmos) was present in 13 eyes (14.1%). Twenty-seven eyes (29.3%) had secondary involvement lesions (Subconjunctival hemorrhage, periorbital ecchymosis, retinal/sub-hyaloid hemorrhage, dilated/tortuous veins). Twenty-one eyes (22.8%) showed other ocular manifestations which etiopathogenesis is not yet fully understood (White-centred hemorrhages, cotton-wool spots, serous retinal detachment, hemorrhagic pigment epithelial detachment). Leukemic retinopathy was significantly more frequent in adults (23/39 and 1/7 in adult and pediatric groups, respectively; p=0.003). Patients suffering from AML were more likely to have secondary ocular involvement (20/29 and 7/17 in AML and ALL patients, respectively; p=0.047). Retinal hemorrhages were statistically associated with anemia and thrombocytopenia (p=0.041 and p=0.034; respectively).ConclusionLeukemic ophthalmopathy seems to be frequent and may lead to severe visual impairment. An ophthalmic assessment complemented with SD-OCT has paramount importance in all newly diagnosed acute leukemic patients.

THE INCIDENCE, CHARACTERISTICS, MANAGEMENT, PROGNOSIS, AND CLASSIFICATION OF BREAKTHROUGH VITREOUS HEMORRHAGE SECONDARY TO POLYPOIDAL CHOROIDAL VASCULOPATHY.

To describe breakthrough vitreous hemorrhage secondary to polypoidal choroidal vasculopathy (PCV). Patients with the diagnosis of PCV from January 2005 to March 2020 at Peking Union Medical College Hospital were retrospectively reviewed, cases with breakthrough vitreous hemorrhage were analyzed. Subgroup analysis was conducted regarding pachychoroid PCV and nonpachychoroid PCV. Among 722 PCV patients (834 eyes), 103 eyes with breakthrough vitreous hemorrhage (12.4%) were included. Pars plana vitrectomy and proper further interventions could significantly improve the best-corrected visual acuity from logMAR 2.15 ± 0.48 (Snellen 20/2825) to 1.65 ± 0.67 (20/893). Hemorrhagic retinal detachment, baseline central macular thickness, and best-corrected visual acuity were factors associated with final best-corrected visual acuity (P < 0.05). In the pachychoroid PCV group, patients were younger, all had hemorrhagic pigment epithelial detachment, with a higher prevalence of choroidal vascular hyperpermeability and hemorrhagic retinal detachment, thicker subfoveal choroidal thickness, and thinner central macular thickness; besides, the initial pars plana vitrectomy were more complicated, more additional surgeries had to be performed. More eyes in the nonpachychoroid PCV group had received anti-vascular endothelial growth factor or photodynamic therapy, mostly fibrovascular pigment epithelial detachment, the best-corrected visual acuity and the status of the fellow eye were significantly worse. For the final ocular status, more eyes in nonpachychoroid PCV group were taking anti-vascular endothelial growth factor monotherapy, whereas more eyes in pachychoroid PCV group were stable. The choroidal parameters of these two groups were all significantly different. Breakthrough vitreous hemorrhage is a troublesome complication of PCV. Pars plana vitrectomy and additional interventions are required for better prognosis. Vitreous hemorrhage secondary to pachychoroid PCV or nonpachychoroid PCV have different characteristics and prognosis.

THE OCCURRENCE, CHARACTERISTICS, MANAGEMENT, AND PROGNOSIS OF RETINAL PIGMENT EPITHELIUM TEARS IN PATIENTS WITH POLYPOIDAL CHOROIDAL VASCULOPATHY: A Retrospective Study of 397 Patients.

To investigate retinal pigment epithelium (RPE) tears in patients with polypoidal choroidal vasculopathy. A retrospective review of polypoidal choroidal vasculopathy cases with confirmed RPE tears was conducted. Patients' comprehensive clinical data were collected and analyzed. The treatment strategy was a loading dose of one intravitreal antivascular endothelial growth factor injection, combined with additional injections if exudative activities or visual deterioration were detected. Among 397 polypoidal choroidal vasculopathy patients, 33 patients with RPE tears (8.3%) were included. 42.4% of them happened spontaneously. Pigment epithelial detachment (PED) occurred more frequently in RPE tear patients and most of them had serous vascularized or hemorrhagic PED. The height and greatest linear diameter of PED, and the subfoveal choroidal thickness of these cases were significantly larger, whereas the central foveal thickness was significantly smaller. Most of the RPE tears occurred at the edge of the PED. After our treatment strategy, patients' best-corrected visual acuity improved significantly from 2.13 ± 1.24 (median 20/52) to 1.32 ± 1.31 (median 20/166). Large subretinal hemorrhage may increase the risk of the formation of subretinal fibrosis (P < 0.05). Retinal pigment epithelium tears in polypoidal choroidal vasculopathy are associated with high subRPE hydrostatic pressure, produced by a large PED or hemorrhage. After our intervention strategy, this condition may not necessarily result in poor prognosis.

Fluorescence Lifetime Imaging Ophthalmoscopy (FLIO) in Eyes With Pigment Epithelial Detachments Due to Age-Related Macular Degeneration.

PurposeTo investigate fluorescence lifetime imaging ophthalmoscopy (FLIO) in neovascular AMD and pigment epithelial detachments (PEDs).MethodsA total of 46 eyes with PEDs (>350 μm) as well as age-matched healthy controls were included in this study. We found 28 eyes showed neovascular AMD (nvAMD), and 17 had nonneovascular (dry) AMD (dAMD). The Heidelberg Engineering FLIO excited fluorescence at 473 nm. Fluorescence decays were detected in two spectral channels (498–560 nm; 560–720 nm) to determine fluorescence lifetimes of endogenous fluorophores in their specific spectral emission ranges. Mean fluorescence lifetimes (τm) were investigated. Multimodal imaging was reviewed by two ophthalmologists who circumscribed and classified PEDs as either serous (n = 4), hemorrhagic (n = 4), fibrovascular (n = 16), drusenoid (n = 17), or mixed (n = 5). Blood samples from a healthy subject and a patient with PED were investigated in a quartz cuvette.ResultsEyes with nvAMD show similar FLIO patterns to dAMD: ring-shaped prolongations of τm 3 to 6 mm from the fovea. Different PED-forms show characteristic τm, while serous and hemorrhagic PEDs exhibit shortened τm, drusenoid PEDs show prolonged τm, and τm in fibrovascular PEDs is variable. Areas corresponding to sub-/intraretinal fluid display shortened τm. Ex vivo studies of blood also show short τm.ConclusionsThe previously described dAMD-related FLIO pattern is also present in nvAMD. Short τm in serous, fibrovascular, and hemorrhagic PEDs as well as sub/intraretinal fluid may disrupt this pattern. FLIO appears to differentiate between PEDs, hemorrhage, and fluid. Additionally, ex vivo studies of human blood help to better interpret FLIO images.

Diagnostic performance of confocal scanning laser ophthalmoscope imaging and compared with color fundus camera in retinal pigment epithelial detachment of polypoidal choroidal vasculopathy

Objective To observe confocal scanning laser ophthalmoscope (cSLO) based retinal imaging and color fundus camera in pigment epithelial detachment (PED) of polypoidal choroidal vasculopathy (PCV). Methods PED of 30 patients (32 eyes) were recruited from June 2016 to June 2017 in the Beijing Tongren Hospital who were detected in high-definition OCT (HD-OCT) and diagnosed as PCV by FFA and ICGA. There were 16 males (17 eyes) and 14 females (15 eyes); aged from 50-83 years, with the mean age of 66.59 years. The photographs of ocular fundus including color fundus camera, cSLO imaging, HD-OCT, FFA and ICGA were analyzed. Multimodal imaging results were regarded as gold standard. Sensitivity and specificity were calculated in serous and hemorrhagic PED diagnosis using color fundus camera and cSLO imaging. The positive number of PED was used to compare between two modes fundus imaging by using χ2 test. Results Twenty serous PED eyes, 3 hemorrhagic PED eyes and 9 serous/hemorrhagic PED eyes were determined using multimodal imaging. The sensitivity and specificity of color fundus camera were 45% and 100% in detecting serous PED and 100% and 91 % in detecting hemorrhagic PED. The sensitivity and specificity of cSLO imaging were 83% and 100% in detecting serous PED and 50% and 86% in detecting hemorrhagic PED. The positive number of serous PED in cSLO imaging was significantly higher than color fundus camera (χ2=7.752, P=0.011). The positive number of hemorrhagic PED in cSLO imaging shows no obvious difference compared with color fundus camera (χ2=1.164, P=0.419). Conclusion The sensitivity and positive number of detecting serous PED with PCV in cSLO fundus imaging were higher than the color fundus camera technology. Key words: Choroid diseases; Retinal pigment epithelial detachment; Confocal scanning laser ophthalmoscope; Color fundus camera

Sensitivity and Specificity of Potential Diagnostic Features Detected Using Fundus Photography, Optical Coherence Tomography, and Fluorescein Angiography for Polypoidal Choroidal Vasculopathy

ImportanceThe use of indocyanine green angiography (ICGA) is a criterion standard for diagnosing polypoidal choroidal vasculopathy (PCV), an endemic and common cause of vision loss in Asian and African individuals that also presents in white individuals. However, the use of ICGA is expensive, invasive, and not always available at clinical centers. Therefore, knowing the value of certain features detected using fundus photography (FP), optical coherence tomography (OCT), and fluorescein angiography (FA) to diagnose PCV without ICGA could assist ophthalmologists to identify PCV when ICGA is not readily available.ObjectiveTo explore the sensitivity, specificity, and predictive accuracy of potential diagnostic features detected using FP, OCT, and FA in diagnosing PCV without ICGA.Design, Setting, and ParticipantsDeidentified images of FP alone, OCT alone, and FA alone were graded by 3 retina specialists masked to ICGA findings for potentially diagnostic features of PCV prespecified before grading compared with the criterion standard grading of 2 other retina specialists with access simultaneously to FP, OCT, FA and ICGA. Specialists graded images of 124 eyes of 120 patients presenting between January 1, 2013, and December 31, 2016, with newly identified serous or serosanguinous maculopathy who had undergone FP, OCT, FA, and ICGA before treatment at a large referral eye center in Thailand.Main Outcomes and MeasuresSensitivity, specificity, positive predictive value, negative predictive value, and predictive accuracy from the area under the receiver operating characteristic curve (AUC).ResultsThe mean (SD) age of the patients was 57.7 (12.6) years, 52 were women, 68 were men, and the diagnosis (from ICGA) was PCV for 65 eyes (52.4%), central serous chorioretinopathy for 45 eyes (36.3%), and typical neovascular age-related macular degeneration for 12 eyes (9.7%). With the use of FP, a potential diagnostic feature for PCV was notched or hemorrhagic pigment epithelial detachment (AUC, 0.77; 95% CI, 0.70-0.85). With the use of OCT, potential diagnostic features for PCV were pigment epithelial detachment notch (AUC, 0.90; 95% CI, 0.85-0.96), sharply peaked pigment epithelial detachment (AUC, 0.86; 95% CI, 0.80-0.92), and a hyperreflective ring (AUC, 0.86; 95% CI, 0.80-0.92). When at least 2 of these 4 signs were present, the AUC was 0.93 (95% CI, 0.89-0.98), with a sensitivity of 0.95 (95% CI, 0.87-0.99), a specificity of 0.95 (95% CI, 0.82-0.97), a positive predictive value of 0.92 (95% CI, 0.83-0.97), and a negative predictive value of 0.95 (95% CI, 0.86-0.99).Conclusions and RelevanceThese data suggest that the potential diagnostic features detected using FP and OCT provide high sensitivity and specificity for a diagnosis of PCV, especially when at least 2 of 4 highly suggestive signs are present.

Class 2 Gene Expression Profile of a Hemorrhagic Pigment Epithelial Detachment Misdiagnosed as Melanoma

Abstract Purpose To describe a case of a hemorrhagic pigment epithelial detachment that was initially misdiagnosed as a choroidal melanoma and had a class 2 gene expression profile (GEP) on prognostication fine needle aspiration biopsy (FNAB). Methods Retrospective case report Results A 79-year-old man with a history of atrial fibrillation treated with warfarin presented for guidance regarding systemic surveillance after undergoing prognostic FNAB at the time of episcleral plaque brachytherapy for a presumed cilio-choroidal melanoma. The results of GEP testing were consistent with a class 2 molecular profile. At three month and eight month follow up, the clinical, echographic, and optical coherence tomography (OCT) findings were consistent with a hemorrhagic pigment epithelial detachment (PED) rather than melanoma. By eight months, the lesion had completely resolved. Conclusions This is the first reported case of a GEP class 2 profile obtained from prognostic FNAB of a non-malignant lesion. Given that GEP is not a diagnostic test, it and can lead to erroneous prognostic information in the setting of non-melanoma choroidal lesions.

Current management strategy of polypoidal choroidal vasculopathy.

Polypoidal choroidal vasculopathy (PCV) is a retinal disorder commonly found in Asians presenting as neovascular age-related macular degeneration and is characterized by serous macular detachment, serous or hemorrhagic pigment epithelial detachment, subretinal hemorrhage, and occasionally visible orange-red subretinal nodular lesions. PCV is diagnosed using indocyanine green angiography (ICGA), and the lesions appear as polypoidal aneurysmal vascular lesions with or without abnormal branching vascular network. Although ICGA remains the gold standard for the diagnosis of PCV, various imaging modalities have also facilitated the diagnosis and monitoring of PCV. Recent advances in imaging technology including the use of high resolution spectral domain optical coherence tomography (OCT) and OCT angiography have provided new insights on the pathogenesis of PCV, suggesting a link between PCV and pachychoroid spectrum of macular disorders. With the evolving understanding on the pathogenesis and clinical characteristics of PCV, different therapeutic options have been proposed. These include intravitreal anti-vascular endothelial growth factor (anti-VEGF) monotherapy, combination therapy with anti-VEGF and verteporfin photodynamic therapy, and thermal laser photocoagulation. In recent years, major multi-center randomized clinical trials such as EVEREST, EVEREST II, and PLANET studies have been conducted to compare the efficacy and safety of various treatment options for PCV. This review aims to summarize the results of recent literature, clinical trials and studies to provide an update on the management options of PCV. An overall management strategy for PCV will also be proposed.

Anatomical and Functional Outcome: A Case of Idiopathic Polypoidal Choroidal Vasculopathy

Idiopathic Polypoidal Choroidal vasculopathy (PCV) is characterized by subretinal vascular lesions leading to multiple, recurrent serosanguinous or hemorrhagic retinal pigment epithelial detachment. The classical PCV occurrence was initially reported in Asian descendent hypertensive middle aged women. Although anti-vascular endothelial growth factor (VEGF) is commonly used as first line treatment in Western countries, photodynamic therapy is the mainstay therapy for PCV in Asia. Here we describe a case of IPCV in a Malay lady who did not respond to initial anti-VEGF monotherapy and subsequently achieved stabilization of the disease with PDT. However, her functional vision was not able to be restored despite the anatomical improvement.

Pigment epithelial tears after ranibizumab injection in polypoidal choroidal vasculopathy and typical age-related macular degeneration.

The purpose of the study was to compare the rates and characteristics of retinal pigment epithelial (RPE) tears between typical exudative age-related macular degeneration (tAMD) and polypoidal choroidal vasculopathy (PCV) after injection of intravitreal ranibizumab (IVR). In total, 836 eyes from 784 patients with exudative AMD treated with IVR were analyzed. The presence, type, size, and height of pigment epithelial detachment (PED) in OCT before injection were evaluated, and the occurrence rate of RPE tears within threemonths of injection between tAMD and PCV was compared. In total, 515 eyes (61.6%) had tAMD and 321 eyes (38.4%) were diagnosed as PCV. RPE tears developed in 18 eyes (3.5%) in the tAMD group, while only two eyes (0.62%) were associated with PCV (p = 0.009, Chi-square test). Eleven of the eighteen eyes with RPE tears in tAMD had fibrovascular PED with contractile neovascular tissue under the surface of the RPE and a cleft at baseline. Two eyes with RPE tears in PCV showed large hemorrhagic PED before presenting with an RPE tear. RPE tears after IVR were significantly less common in PCV than in tAMD. The different characteristics of RPE tears between the two disease entities suggest differences in the pathogenesis underlying the event.

VARIABLE RESPONSE OF VASCULARIZED PIGMENT EPITHELIAL DETACHMENTS TO RANIBIZUMAB BASED ON LESION SUBTYPES, INCLUDING POLYPOIDAL CHOROIDAL VASCULOPATHY

The purpose of this study was to evaluate the prognosis and response to intravitreal ranibizumab (IVR) of neovascular age-related macular degeneration, according to the type of pigment epithelial detachment (PED). The authors prospectively studied 57 eyes of 57 consecutive patients with PED associated with exudative age-related macular degeneration, who were treated by IVR. All patients received 3 consecutive monthly injections of 0.5 mg/0.05 mL of ranibizumab as induction treatment. Retreatment was allowed if evidence of clinical deterioration was noted or spectral domain optical coherence tomography at the 1-month follow-up showed intraretinal edema, subretinal fluid, or recurrent PED. The best-corrected visual acuity (BCVA) values measured before and at 3, 6, and 12 months after the first injection were compared according to the type of PED. Changes in the height of PED to treatment with IVR were also investigated. Fifty-six eyes were assessed at the 12-month follow-up examination. There were 4 types of PED, including serous PED in 11 patients (19.6%), fibrovascular PED in 28 patients (50.0%), mixed PED with serous and fibrovascular component in 7 patients (12.5%), and hemorrhagic PED in 10 patients (17.9%). Eyes with serous PED showed significant improvement of the mean logarithm of the minimum angle of resolution (logMAR) BCVA as compared with the value at the baseline, which was sustained throughout the 12-month period (P < 0.05). Regarding the eyes with fibrovascular and mixed PED, significant improvement of the mean logMAR BCVA was observed compared with the value at the baseline at 3 months; however, a slight decrease was observed at 6 and 12 months. In the eyes with hemorrhagic PED, no significant difference in the mean BCVA values compared with the value at the baseline was observed at any follow-up time point. In relation to the height of the PED, all eyes in the serous and mixed PED group, 17 eyes in the fibrovascular PED group (60.7%), and 9 eyes in the hemorrhagic PED group (90.0%) showed reduction of the maximum PED height by 100 μm or more. The PED response to IVR was not correlated with the final BCVA. Intravitreal ranibizumab for the treatment of exudative age-related macular degeneration is effective for stabilizing vision in patients with PED, but it may be better tolerated in patients with serous PED. Although it may be important to consider the type of PED to predict the visual acuity in patients treated by IVR, the anatomical response of the PED may not correlate directly with the visual outcome.

Pattern of presentation of idiopathic polypoidal choroidal vasculopathy in Ibadan, Sub-Saharan Africa

BackgroundIdiopathic polypoidal choroidal vasculopathy is an abnormal choroidal vascular pathology similar to age-related macular degeneration. It may present with sudden visual loss from hemorrhagic retinal pigment epithelial detachment and breakthrough vitreous hemorrhage or with chronic recurrent episodes. The condition is not uncommon in the retina clinic at the University College Hospital, Ibadan, Sub-Saharan Africa. This study presents the pattern of presentation in Ibadan.MethodsWe review all cases of idiopathic polypoidal choroidal vasculopathy seen from 2007 to 2012 in the retina clinic at the University College Hospital, Ibadan, to determine the major pattern of presentations, available treatment modalities, and visual outcomes.ResultsTen cases were seen during the study period. Their mean age was 58 years, with a male to female ratio of 1:4. The most common presenting complaint was sudden visual loss. Major examination findings were retinal pigment epithelial detachment, orange subretinal lesions, and breakthrough vitreous hemorrhage. The modalities of treatment available included vitrectomy to clear vitreous hemorrhage. Intravitreal bevacizumab reduced the height of the pigment epithelial detachment and cleared vitreous hemorrhage. Thermal laser was applied for extrafoveal lesions. Two patients with subfoveal lesions were referred abroad for photodynamic therapy. Visual outcome showed significant improvement in vitrectomized patients who presented with vitreous hemorrhage. Presenting vision of hand motion and light perception improved to vision ranging from counting fingers to 6/12 after vitrectomy.ConclusionIdiopathic polypoidal choroidal vasculopathy may not be uncommon in Sub-Saharan Africa. A high index of suspicion is warranted in the diagnosis so as to provide timely intervention.

Intravitreal bevacizumab monotherapy for treatment-naïve polypoidal choroidal vasculopathy

Dear Editor, Polypoidal choroidal vasculopathy (PCV) is primarily a choroidal vascular disease, which can lead to acute severe visual loss secondary to massive submacular or vitreous hemorrhage. Various treatment modalities, such as direct thermal laser photocoagulation, tissue plasminogen activator (t-PA) injection with gas displacement, submacular surgery and macular translocation surgery have been proposed. Photodynamic therapy (PDT) with verteporfin has shown good results for PCV, but extensive subretinal hemorrhages and exudation limits its application.[1] Favorable results have been reported with combined therapy, including PDT and intravitreal bevacizumab (IVB), to treat PCV.[2] We evaluated nine eyes of eight patients with PCV who were treated with IVB monotherapy. We excluded patients that had any previous treatment for PCV or wet age-related macular degeneration (AMD) in the study or contralateral eye. All participants received a comprehensive ocular examination including digital fluorescein angiography (FA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT) at baseline, and at 3-monthly interval visits till last follow-up. IVB injection was performed as per standard protocol. Repeat treatment was performed every 4 weeks if the OCT showed persistent pigment epithelial detachment (PED)/subretinal fluid (SRF) or active polypoidal lesion on fundus fluorescein angiography (FFA)/ICGA. The mean age was 60.6 years and 37.5% of the patients were female. At baseline, the median Snellen best corrected visual acuity (BCVA) was 20/2000 (range 20/30–20/10,000). The mean follow-up duration was 8.4 months (range 4–12 months). All eyes showed resolution of subretinal hemorrhage, PED [Fig. 1] and angiographic activity [Fig. 2], but persistence of polypoidal lesion was noted in three eyes. At the last follow-up, the median Snellen BCVA was 20/100 (range 20/25–20/400). The mean log MAR BCVA showed a statistically significant improvement from 1.41 to 0.63 (P = 0.04). Seven (77.7%) eyes showed significant improvement in visual acuity, with mean number of line improvement of 6.5. The remaining two eyes maintained the baseline visual acuity till last follow-up. The mean number of injections was 2.3. The mean longest treatment-free period was 5.88 months. No significant systemic or ocular side-effects were observed in any of the eyes receiving IVB injections. Figure 1 Fundus photo at baseline (a) shows hemorrhagic pigment epithelial detachment (PED) involving the fovea with subretinal hemorrhage. (b) (at 1-month follow-up) Reduction in the PED with decrease in subretinal hemorrhage. (c) (at 3-month follow-up) Disappearance ... Figure 2 Indocyanine green angiographic images (a) before treatment and (b) 1 year after treatment, showing regression of polyps (arrows) after treatment In our study, mean BCVA improvement of 2 or more lines was seen in 72% of the patients, and the remaining 28% showed a stabilization of visual acuity at mean follow-up of 8.4 months. Kokame et al.,[3] reported stabilization of vision at 6 months, with monthly intravitreal injection of ranibizumab in PCV. Lai et al., reported stabilization of vision and reduction in exudative detachment with intravitreal bevacizumab, but a limited role in regression of polypoidal lesions in ICGA. Persistent lesions were treated with PDT.[4] In our study, persistent lesions were closely followed-up, which did not bleed again during the follow-up. Complete regression of polypoidal lesion in ICGA may not be the therapeutic target, but close follow-up is mandatory. In conclusion, our study demonstrated that IVB injection helps to improve or maintain the vision in patients with symptomatic treatment-naive PCV.

Polypoidal choroidal vasculopathy in a case with retinitis pigmentosa

There have been no reports describing polypoidal choroidal vasculopathy (PCV) in eyes with retinitis pigmentosa (RP). A 63-year-old woman who had been diagnosed as having RP was referred to us because of sudden onset of blurred vision in her right eye. Funduscopic examination revealed retinal findings typical of RP in both eyes. The macular area of the right fundus showed polypoidal lesions with massive hemorrhages. Fluorescein angiography and indocyanine green angiography showed multiple polypoidal lesions. Optical coherence tomography showed a large hemorrhagic retinal pigment epithelial (RPE) detachment and polypoidal lesions. The PCV subsided after three applications of anti-vascular endothelial growth factor (VEGF) therapy and a single application of photodynamic therapy, but “mottled lesions” with hyper- and hypofluorescence appeared temporal to the macula after disappearance of hemorrhage. We present a case of PCV in an eye with RP. Further studies are necessary to clarify whether anti-VEGF therapies could affect RPE status in eyes with RP.

Comparison of different treatment intervals between bevacizumab injection and photodynamic therapy in combined therapy for age-related macular degeneration

To compare the results of combination therapy with different intervals between intravitreal bevacizumab and photodynamic therapy (PDT) with verteporfin for age-related macular degeneration. Treatment-naïve eyes (n = 184) with 12 months' follow-up were included in this retrospective case series. Eyes were classified according to the interval between bevacizumab and PDT administration: group D1, 1-day interval (n = 116); group D7, 7-day interval (n = 68). The study was conducted at two hospitals, with group D1 evaluated in one hospital and group D7 evaluated in the other. The main outcome measure was comparison of the increases in best-corrected visual acuity (BCVA) of the two groups 3 and 12 months after the initial treatment by means of analysis of covariance (ANCOVA). Group D1 gained 1.3 lines and group D7 gained 1.5 lines of BCVA at 3 months; group D1 gained 0.8 lines and group D7 gained 1.4 lines at 12 months. There was a significant difference between the groups in the increased BCVA levels at 3 months (P = 0.0450) and a trend toward significance at 12 months (P = 0.0516). ANCOVA analysis revealed that baseline BCVA, hemorrhagic pigment epithelial detachment, subretinal fluid, lesion size, and a 7-day treatment interval were significantly correlated with the increase in the BCVA at 3 months. A 7-day treatment interval might offer slightly better visual acuity gain in the short term than a 1-day interval.

Role of Complement Factor H I62V and Age-Related Maculopathy Susceptibility 2 A69S Variants in the Clinical Expression of Polypoidal Choroidal Vasculopathy

To investigate the role of complement factor H (CFH) I62V (rs800292) and age-related maculopathy susceptibility 2 (ARMS2) A69S (rs10490924) variants in the clinical characteristics of polypoidal choroidal vasculopathy (PCV). Cross-sectional study. A total of 226 Japanese patients with PCV in both eyes (44 cases) or in 1 eye (182 cases). Genotyping was performed in all cases for CFH I62V using TaqMan technology and for ARMS2 A69S by denaturing high-performance chromatography. The incidence of 5 characteristic funduscopic findings was studied, including serous retinal detachment, subretinal hemorrhage, serous pigment epithelial detachment (PED), hemorrhagic PED, and classic choroidal neovascularization (CNV). The association of clinical phenotypes, including the incidence of each of 5 specific fundus findings, bilaterality of the disease, and age at onset, with variants of CFH I62V or ARMS2 A69S. Although there was no association of CFH I62V variants with any of the phenotypes in PCV, at-risk variants of ARMS2 A69S were associated with higher incidences of subretinal hemorrhage, serous PED, and hemorrhagic PED. In particular, the at-risk allele homozygosity of ARMS2 A69S increased the likelihood for hemorrhagic PED by 12.4-fold compared with non-carriers of the allele (confidence interval, 1.60-95.1, P = 0.0001). However, the at-risk allele of ARMS2 A69S was associated with a lower incidence of serous retinal detachment (P = 0.0092). Classic CNV was not associated with either variant. The mean age at the onset of PCV was significantly younger (68.8 years) in those with homozygosity of the at-risk allele of ARMS2 A69S than in those with heterozygosity (71.6 years) or in non-carriers (72.6 years) (P = 0.026). Moreover, the at-risk allele frequencies of the ARMS2 A69S were significantly higher in bilateral cases than in unilateral cases (75.0% vs. 59.3%, P = 0.007). ARMS2 A69S variants were significantly associated with hemorrhagic or subpigment epithelium lesions of PCV, and with earlier onset and bilateral involvement. The genotyping of ARMS2 A69S is more informative than that of CFH I62V in understanding the clinical features in patients with PCV.

RETINAL MICROSTRUCTURAL ABNORMALITIES IN CENTRAL SEROUS CHORIORETINOPATHY AND POLYPOIDAL CHOROIDAL VASCULOPATHY

To compare retinal morphologic alterations in eyes with polypoidal choroidal vasculopathy (PCV) and central serous chorioretinopathy (CSC) using speckle noise-reduced spectral-domain optical coherence tomography. We retrospectively reviewed 63 eyes of 62 patients with active PCV and 38 eyes of 38 patients with active CSC. Patients underwent fundus photography, angiography, and speckle noise-reduced spectral-domain optical coherence tomography examinations, and retinal morphologic alterations were evaluated. Cystoid macular edema, lipid deposits, subretinal hemorrhage, and hemorrhagic pigment epithelial detachment were not seen in any eye with CSC but were seen in eyes with PCV. In PCV, mean visual acuity was significantly poorer in eyes with fibrin infiltration (P = 0.027) or hemorrhagic infiltration (P = 0.002) in the fovea than in eyes without fibrin or hemorrhagic infiltration. Differentiating factors between PCV and CSC noted on spectral-domain optical coherence tomography include a lack of cystoid macular edema, lipid deposition, subretinal hemorrhage, and hemorrhagic pigment epithelial detachment in eyes with CSC, which makes spectral-domain optical coherence tomography helpful in differentiating CSC from PCV. More severe retinal alterations were seen in PCV than in CSC because of infiltration of fibrin and hemorrhage in the outer retina, which also correlated with poorer vision.