Polypoidal Choroidal Vasculopathy: Updates on Risk Factors, Diagnosis, and Treatments.

There have been recent advances in basic research and clinical studies in polypoidal choroidal vasculopathy (PCV). A recent, large-scale, population-based study found systemic factors, such as male gender and smoking, were associated with PCV, and a recent systematic review reported plasma C-reactive protein, a systemic biomarker, was associated with PCV. Growing evidence points to an association between pachydrusen, recently proposed extracellular deposits associated with the thick choroid, and the risk of development of PCV. Many recent studies on diagnosis of PCV have focused on applying criteria from noninvasive multimodal retinal imaging without requirement of indocyanine green angiography. There have been attempts to develop deep learning models, a recent subset of artificial intelligence, for detecting PCV from different types of retinal imaging modality. Some of these deep learning models were found to have high performance when they were trained and tested on color retinal images with corresponding images from optical coherence tomography. The treatment of PCV is either a combination therapy using verteporfin photodynamic therapy and anti-vascular endothelial growth factor (VEGF), or anti-VEGF monotherapy, often used with a treat-and-extend regimen. New anti-VEGF agents may provide more durable treatment with similar efficacy, compared with existing anti-VEGF agents. It is not known if they can induce greater closure of polypoidal lesions, in which case, combination therapy may still be a mainstay. Recent evidence supports long-term follow-up of patients with PCV after treatment for early detection of recurrence, particularly in patients with incomplete closure of polypoidal lesions.

UVEAL EFFUSION SYNDROME. CASE REPORT.

PURPOSE: Purpose of this article is to present a case report of a patient with uveal effusion syndrome who underwent deep posterior sclerotomy. CASE REPORT: A 73-year-old patient with unilateral decrease in the best corrected visual acuity, ablation of choroid and secondary retinal detachment in the right eye was admitted to our clinic for examination in November 2017. At the first examination, the best corrected visual acuity in the right eye was 0.3, in the left eye 1.0. Intraocular pressure was 16 mmHg in the right eye and 21 mmHg in the left eye. After performing ultrasound biomicroscopy of the anterior segment (Accutome, Keeler, USA), ultrasound sonography of the affected eye (Accutome, Keeler, USA), magnetic resonance imaging, computed tomography, abdominal ultrasound and blood tests, we concluded the finding as uveal effusion syndrome. We initiated a conservative treatment consisting of oral administration of carbonic anhydrase inhibitor in combination with topical use of prostaglandin analogue. Despite conservative treatment the best corrected visual acuity of the affected eye decreased to 0.05 so we proceeded to a surgical procedure - deep posterior sclerotomy with perioperative scleral sampling for histological examination (detection of glycosaminoglycans in the sclera wall by Alcian blue staining), which was negative. This histological result ranks the patient as the third type of uveal effusion syndrome (ie, non-nanophthalmic with a normal sclera). After the operation both the ablation of choroid and retinal detachment reattached and the best corrected visual acuity in the right eye improved to 0.3. After the subsequent cataract surgery, the ablation of choroid and retinal detachment occurred again, this time with spontaneous recovery. Postoperatively, the best corrected visual acuity in the right eye was 0.5 and at the last check-up at our clinic 0.6. CONCLUSION: Deep posterior sclerotomy is a method of choice of surgical treatment for uveal effusion syndrome that does not respond to conservative therapy.

Idiopathic polypoidal choroidal vasculopathy: a review of literature with clinical update on current management practices.

PURPOSE: Polypoidal choroidal vasculopathy is a major cause of visual disability in a vast majority of Asian population due to exudative maculopathy. Although it is a distinctive disease entity with characteristic pathophysiology, genetics, immunology and clinical features, but it is still misdiagnosed as neovascular age related macular degeneration as both the diseases are a part of pachychoroid spectrum and have some similar features. Also, there are varied options for the management of this disease, but there are no clear recommendations. So, a detailed review of the literature has been done along with special attention to the recent therapeutic advances to help the readers get a better understanding of the disease and its current management practices. METHOD: Detailed review of literature regarding polypoidal choroidal vasculopathy was done. The disease pathophysiology, genetics, risk factors, diagnostic modalities along with current treatment guidelines were extensively studied and compiled. RESULT: A comprehensive clinical update on polypoidal choroidal vasculopathy was compiled with special emphasis on the recent diagnostic modalities and treatment guidelines. CONCLUSION: Polypoidal choroidal vasculopathy is a distinct clinical entity which can be diagnosed based on indocyanine green angiography and optical coherence tomography. Treatment includes various options like photodynamic therapy, anti VEGF agents and thermal laser ablation. A review of literature has been done and recent diagnostic modalities with management practices have been compiled for the better understanding of the disease.

Latest Developments in Polypoidal Choroidal Vasculopathy: Epidemiology, Etiology, Diagnosis, and Treatment.

Polypoidal choroidal vasculopathy (PCV) is a condition characterized by multiple, recurrent, serosanguineous pigment epithelial detachments, and neurosensory retinal detachments due to abnormal aneurysmal neovascular lesions. It is generally considered as a variant of neovascular age-related macular degeneration, but there are some differences between the clinical presentation, natural history, and treatment response between patients with PCV and typical neovascular age-related macular degeneration patients. Over the past decade, new research and technological advancements have greatly improved our understanding of the PCV disease process and the management of PCV. This review aims to summarize the recent research findings to highlight the epidemiology, pathogenesis, genetics, the application of various diagnostic tools for PCV, and the available treatment options for PCV.

Five-year outcomes of photodynamic therapy combined with intravitreal injection of ranibizumab or aflibercept for polypoidal choroidal vasculopathy.

We report 5-year visual and anatomical outcomes after combination therapy of photodynamic therapy (PDT) and intravitreal injection of ranibizumab or aflibercept for polypoidal choroidal vasculopathy (PCV) and predictive factors for visual outcomes at 5-year and time to recurrence. Medical charts were retrospectively reviewed for 43 consecutive eyes with PCV treated with combination therapy of PDT and intravitreal injection of ranibizumab(n = 13) or aflibercept(n = 30) and completed 5-year follow-up. The variants of ARMS2 A69S and CFH I62V were genotyped using TaqMan assay. Best corrected visual acuity (BCVA) significantly improved at 5-year (P = 0.01) with 20% reduction of subfoveal choroidal thickness irrespective of presence or absence of recurrence. Visual improvement was associated with baseline shorter greatest linear dimension (GLD) (P = 1.0×10-4). Mean time to recurrence was 28.6±23.1 months (95% CI: 21.5-35.7, Median:18.0) and time to recurrence was associated with G allele (protective allele) of ARMS2 A69S and GLD (P = 4.0×10-4 and 1.0×10-2, respectively). Multiple regression analysis revealed that time to recurrence extended by 15.5 months when the G allele of ARMS2 A69S increased by one allele (TT: 15.7±17.0, TG: 30.8±23.5, GG: 41.1±22.6 months). The combination therapy resulted in a favorable visual outcome for PCV during 5-year follow-up.

Diagnosis and treatment of peripheral exudative haemorrhagic chorioretinopathy.

PURPOSE: Peripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder that is often misdiagnosed. The aim of this study was to better characterise PEHCR and to assess treatment options. MATERIAL AND METHODS: Retrospective multicentric chart review. RESULTS: Of 84 eyes (69 patients) with PEHCR referred between 2005 and 2017, the most common referral diagnosis was choroidal melanoma (41.3%). Bilateral involvement was found in 21.7% of cases. Haemorrhagic retinal pigment epithelium detachment was the most common peripheral lesion (53.6%). Maculopathy was associated with peripheral lesions in 65.8% of cases. PEHCR lesions were mostly heterogeneous (58.8%) on B-scan ultrasonography. Choroidal neovascularisation was found in 10 eyes (26.3%) out of 38 eyes that underwent fluorescein angiography. Polyps were observed in 14 eyes (58.3%) out of 24 eyes that underwent indocyanine green angiography. Fifty-one eyes were treated (62.2%). Intravitreal injections (IVTI) of antivascular endothelial growth factor (VEGF) were the most used treatment (36.6%) before laser photocoagulation, photodynamic therapy, vitrectomy and cryotherapy. Only vitrectomy improved visual acuity. Most lesions (65.6%) regressed at the last follow-up visit. CONCLUSION: In case of PEHCR, multimodal imaging is useful to avoid misdiagnosis, to characterise PEHCR lesions and to guide treatment strategies. Regression of PEHCR lesions was observed in two-thirds of the patients. Vitrectomy improved visual acuity. More than a third of patients underwent anti-VEGF IVTI. Further studies are needed to assess IVTI's efficacy.

Approche diagnostique et thérapeutique de la vasculopathie polypoïdale choroïdienne. Recommandations de la Fédération France Macula.

PURPOSE: To update the medical literature on the diagnostic and therapeutic approach to polypoidal choroidal vasculopathy (PCV) and to propose a treatment algorithm in agreement with French market approval, supported by the France Macula Federation (FFM). METHODS: Literature review and expert opinion. RESULTS: The diagnosis of PCV is based on multimodal imaging, including indocyanine green angiography (ICGA), which is considered the gold standard for the diagnosis of PCV. Regarding the therapeutic management of PCV, the FFM recommends treating PCV first-line either by monotherapy with intra-vitreal anti-vascular endothelial growth factor (anti-VEGF) injections, or by a combined treatment of photodynamic therapy (PDT) with Verteporfin and intra-vitreal anti-VEGF injections, depending on the location of the PCV.

The effect of single periocular injection of methylprednisolone and drainage of suprachoroidal fluid in the treatment of rhegmatogenous retinal detachment combined with choroidal detachment.

PURPOSE: In this study we compared the anatomic and functional outcomes of two steroid treatments on rhegmatogenous retinal detachment (RRD) combined with choroidal detachment (CD), namely treatment with oral prednisolone (1 mg/kg daily) for 3-7 days before vitrectomy or a single periocular injection of methylprednisolone (40 mg) 1-3 days before vitrectomy. We also analyzed the outcomes of the eyes with subsided CD and the eyes with persistent CD that underwent drainage of suprachoroidal fluids during the vitrectomy. METHODS: This was a prospective randomized study. Seventy five eyes with RRD combined with CD were divided into 2 groups based on the two different treatment regimens as above. The eyes in each group were further divided into 2 subgroups (A: CD subsided eyes; B: CD persistent eyes) according to the response of CD to the treatment of steroids. Retinal reattachment rates were measured at 6 months after the removal of silicone oil. RESULTS: At 6 months after silicone oil removal, the retinal reattachment rate was similar (p = 0.666) in the oral prednisolone group (91.7%, 33/36) and the periocular injection group (94.9%, 37/39). Similar retinal reattachment rates (p = 0.364) were also found in the CD subsided eyes (97.1%, 34/35) and the CD persistent eyes (90.0%, 36/40). The retinal reattachment rate was comparable among the subgroups (p = 0.395; oral prednisolone A group: 95.2%, 20/21; oral prednisolone B group: 86.7%, 13/15; periocular injection A group: 100%, 14/14; periocular injection B group: 92.0%, 23/25). CONCLUSIONS: For RRD combined with CD, eyes treated with a single periocular injection of methylprednisolone (40 mg, 1-3 days before pars plana vitrectomy) combined with the drainage of suprachoroidal fluids during the surgery had similar anatomic and functional outcomes compared to the eyes treated with oral prednisolone for 3-7 days before vitrectomy.

[Peripheral Exudative Haemorrhagic Chorioretinopathy: Course of Disease and Diagnosis - Including Wide-field Imaging of Associated Diseases Age-related Macular Degeneration and Polypoidal Choroidal Vasculopathy, Therapy]. / Peripher exsudative hämorrhagische Chorioretinopathie: Krankheitsverlauf, Diagnosestellung inklusive Weitwinkelbildgebung der assoziierten Erkrankungen altersabhängige Makuladegeneration und polypoidale Choriovaskulopathie, Therapie.

PEHCR (peripheral exudative haemorrhagic chorioretinopathy) is a disease manifested clinically, particularly by subretinal bleeding, retinal exudates, retinal pigment epithelium detachments (RPE detachments), exudative retinal detachment and sub-RPE bleeding. The PEHCR lesion is often characterized by its polypoidal pattern, which is very similar to PCV (polypoidal choroidal vasculopathy) polyps. Diagnosis is best made with a wide-field ICGA (indocyanine green angiography). In approximately half of patients, macular changes in the form of drusen, up to exudative AMD (age-related macular degeneration), are detected in the affected eye or partner eye. Since there is very little literature directly available on PEHCR, this work also discusses the peripheral changes described in the context of AMD that were investigated with wide-field imaging.

Polypoidal choroidal vasculopathy: Pearls in diagnosis and management.

Polypoidal choroidal vasculopathy (PCV) is increasingly recognized as an important cause of exudative maculopathy in Asians as against Wet age-related macular degeneration in Caucasians. A panel of retinal experts methodically evaluated pertinent updated literature on PCV with thorough PubMed/MEDLINE search. Based on this, the panel agreed upon and proposed the current consensus recommendations in the diagnosis (clinical and imaging), management and follow-up schedule of PCV. Diagnosis of PCV should be based on the gold standard indocyanine green angiography which demonstrates early nodular hyperfluorescence signifying the polyp with additional features such as abnormal vascular network (AVN). Optical coherence tomography is an excellent adjuvant for diagnosing PCV, monitoring disease activity, and decision-making regarding the treatment. Current treatment modalities for PCV include photodynamic therapy, anti-vascular endothelial growth factor agents, and thermal laser. Choice of specific treatment modality and prognosis depends on multiple factors such as the location and size of PCV lesion, presence or absence of polyp with residual AVN, amount of submacular hemorrhage, presence or absence of leakage on fundus fluorescein angiography, visual acuity, and so on. Current recommendations would be invaluable for the treating physician in diagnosing PCV and in formulating the best possible individualized treatment strategy for optimal outcomes in PCV management.

Characteristic appearances of fundus autofluorescence in treatment-naive and active polypoidal choroidal vasculopathy: a retrospective study of 170 patients.

PURPOSE: To investigate the characteristic appearances of fundus autofluorescence (FAF) in patients with treatment-naive and active polypoidal choroidal vasculopathy (PCV). METHOD: Cases with the diagnosis of treatment-naive and active PCV from November 2012 to May 2017 at Peking Union Medical College Hospital were retrospectively reviewed. All patients underwent comprehensive ophthalmologic examination. Autofluorescence (AF) findings were described at the retinal sites of the corresponding lesions identified and diagnosed using indocyanine green angiography and spectral-domain optical coherence tomography. RESULTS: One hundred seventy patients with 192 affected eyes were included. The logMAR BCVA of the patients were 0.53 ± 0.28. The six AF patterns of 243 polypoidal lesions were confluent hypo-AF with hyper-AF ring (49.8%), confluent hypo-AF (22.6%), hyper-AF with hypo-AF ring (3.7%), granular hypo-AF (7.0%), blocked hypo-AF due to hemorrhage (8.6%), and polyps without apparent AF changes (8.2%). For 146 branching vascular networks (BVNs), 97.3% were granular hypo-AF, and others were blocked hypo-AF due to hemorrhage. CONCLUSION: In eyes with treatment-naive and active PCV, the polypoidal lesions and BVNs induce characteristic FAF changes. FAF images provide reliable adjunct reference for the diagnosis of PCV.

Primary acute angle-closure glaucoma complicating ciliochoroidal detachment: report of four cases and review of the literature.

PURPOSE: To describe the ultrasound biomicroscopy (UBM) and B-scan ultrasonography findings and therapeutic approach for ciliochoroidal detachment secondary to acute primary angle-closure glaucoma (APACG) in four patients. We also reviewed the literature. METHODS: Case report and systematic literature review. RESULTS: The four patients were referred to our department for further management of APACG. The UBM and B-scan ultrasonography examinations were conducted 2 days after the beginning of medical treatment and demonstrated ciliochoroidal detachment in four eyes of the four patients. The patients all received intravenous infusion of corticosteroid therapy (10 mg dexamethasone once daily for 5-7 days). We reviewed the UBM findings, which confirmed that the ciliochoroidal detachment disappeared. The patients then underwent trabeculectomy combined with peripheral iridotomy surgery. The patients all ultimately recovered very well. CONCLUSION: APACG with ciliochoroidal detachment is rare and has hidden clinical manifestations, and the pathophysiological mechanism is not yet fully understood. Anti-glaucoma surgery may increase the detachment. UBM and B-scan ultrasounds are useful tools for ciliochoroidal detachment diagnosis in APACG patients before operative treatment.

Retinopathy of prematurity: inflammation, choroidal degeneration, and novel promising therapeutic strategies.

Retinopathy of prematurity (ROP) is an important cause of childhood blindness globally, and the incidence is rising. The disease is characterized by initial arrested retinal vascularization followed by neovascularization and ensuing retinal detachment causing permanent visual loss. Although neovascularization can be effectively treated via retinal laser ablation, it is unknown which children are at risk of entering this vision-threatening phase of the disease. Laser ablation may itself induce visual field deficits, and there is therefore a need to identify targets for novel and less destructive treatments of ROP. Inflammation is considered a key contributor to the pathogenesis of ROP. A large proportion of preterm infants with ROP will have residual visual loss linked to loss of photoreceptor (PR) and the integrity of the retinal pigment epithelium (RPE) in the macular region. Recent studies using animal models of ROP suggest that choroidal degeneration may be associated with a loss of integrity of the outer retina, a phenomenon so far largely undescribed in ROP pathogenesis. In this review, we highlight inflammatory and neuron-derived factors related to ROP progression, as well, potential targets for new treatment strategies. We also introduce choroidal degeneration as a significant cause of residual visual loss following ROP. We propose that ROP should no longer be considered an inner retinal vasculopathy only, but also a disease of choroidal degeneration affecting both retinal pigment epithelium and photoreceptor integrity.

Atypical Presentation of Chorioretinal Folds-Related Maculopathy.

PURPOSE: Chorioretinal folds are undulations that anatomically include the inner choroid, Bruch's membrane, and the retinal pigment epithelium, and secondarily affect the overlying neurosensory retina. We analyzed clinical data and management of six patients diagnosed with chorioretinal folds-related maculopathy with atypical presentations. CASE REPORT: The mean age of the six patients (five women) was 77 years. Best-corrected visual acuity (BCVA) ranged between 20/200 and 20/80. None of the patients had history of hypertension, cardiovascular diseases, or autoimmune disease, and they were all diagnosed with idiopathic chorioretinal folds. Case 1, 2, and 3 received intravitreal antivascular endothelial growth factor (VEGF) therapy; case 4 received intravitreal anti-VEGF and photodynamic therapy; case 5 received only photodynamic therapy; and case 6 received intravitreal injections of sustained-release dexamethasone implant (Ozurdex). In case 1 and 2, the use of ranibizumab resulted in BCVA improvement and resolution of sub-/intraretinal exudation. In case 3, ranibizumab led to a mild reduction of the intraretinal exudation but no changes in BCVA. In case 4 and 5, six intravitreal injections of ranibizumab with two photodynamic therapies and three photodynamic therapies, respectively, led to a mild reduction of the sub-/intraretinal exudation but no changes in BCVA. In case 6, five intravitreal injections of Ozurdex in both eyes led to reduction of the subretinal or intraretinal fluid accumulation and BCVA improvement. DISCUSSION: Choroidal vessel dilation and hyperpermeability may be involved in atypical presentations of chorioretinal folds-related maculopathy characterized by sub-/intraretinal fluid accumulation. Dilated and hyperpermeable choroidal vessels may result in focal retinal pigment epithelium alterations that can progress to choroidal neovascularization or chronic central serous chorioretinopathy-like maculopathy with or without telangiectatic retinal capillaries. Intravitreal anti-VEGF administration seems effective to treat choroidal neovascularization in stage 3 chorioretinal folds-related maculopathy, both anti-VEGF and photodynamic therapy seem to have only limited efficacy on chronic central serous chorioretinopathy-like maculopathy (and telangiectatic retinal capillaries), whereas intravitreal injection of Ozurdex seems efficacious to treat chronic central serous chorioretinopathy-like maculopathy.

Adrenomedullin: A potential therapeutic target for retinochoroidal disease.

Adrenomedullin (AM) is a 52-amino acid peptide with anti-inflammatory, anti-apoptotic, and anti-oxidative properties discovered in a human pheochromocytoma. It is a member of the calcitonin peptide superfamily, and its signal is mediated by calcitonin receptor-like receptor (CLR). CLR interacts with receptor activity-modifying proteins (RAMPs), among which RAMP-2 and RAMP-3 carry CLR from the endoplasmic reticulum to the cellular membrane to confer high affinity for AM. In addition to being implicated in a variety of systemic diseases, AM is a critical contributor to the pathogenesis of retinochoroidal disease. It is robustly upregulated in retinochoroidal disease models of oxygen-induced retinopathy (OIR) and laser-induced choroidal neovascularisation (CNV) as well as in human patients with retinochoroidal diseases. In this review, we discuss the most salient recent findings that strongly illustrate the role of AM in retinochoroidal disease. In the OIR model, AM was identified as a key angiogenic mediator of retinal vascularisation, and AM inhibition suppressed only pathological angiogenesis, not physiological angiogenesis. On the contrary, lesion size was larger in AM(+/-) CNV model mice, presumably due to the anti-inflammatory function of AM. Despite the success of anti-vascular endothelial growth factor agents for the treatment of retinochoroidal disease, therapeutic shortcomings remain. Finding ways to modulate AM activity will provide new treatment avenues. Potential treatment strategies modulating the action of AM and its signaling pathway have been studied extensively. AM and its signaling molecules are intriguing future treatment targets for retinochoroidal disease.