Punctate inner choroidopathy: A review of the current diagnostic and therapeutic approaches.

Punctate inner choroidopathy (PIC) is an uncommon idiopathic inflammatory condition characterized by multifocal chorioretinopathy that primarily affects young adults, with a predilection for myopic females. Clinically, it manifests as small, yellowish-white lesions in the inner choroid and outer retina, often associated with inflammatory changes. Accurate diagnosis remains a challenge due to its resemblance to other posterior uveitic entities, necessitating an astute clinical eye and advanced imaging techniques for differentiation. Multimodal imaging plays a crucial role by offering valuable insights, as it enables the visualization of various abnormalities related to uveitis. The pathogenesis of PIC is still a subject of debate, with a complex interplay of genetic, immunological, and environmental factors proposed. Managing PIC presents multiple challenges for clinicians. Firstly, variable disease severity within and among patients requires diverse treatments, from observation to aggressive immunosuppression and/or anti-VEGF therapy. Secondly, treatment must distinguish between primary causes of vision loss. New or worsening PIC lesions suggest active inflammation, while new neovascular membranes may indicate secondary neovascular processes. Thirdly, deciding on maintenance therapy is complex, balancing PIC prognosis variability against immunosuppression risks. Some patients have long periods of inactivity and remission, while others face sudden, vision-threatening episodes during quiescent phases. Through a systematic review of the literature, this paper sheds light on the current understanding of PIC, its challenges, and the prospects for future research. By synthesizing existing knowledge, it aims to aid clinicians in accurate diagnosis and guide treatment decisions for improved visual outcomes in individuals affected by PIC.

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.

Suprachoroidal Delivery of Viral and Nonviral Gene Therapy for Retinal Diseases.

Retinal gene therapy is a rapidly growing field with numerous clinical trials underway, and route of delivery is a critical contributor to its success. Subretinal administration, which involves pars plana vitrectomy in the operating room, offers targeted delivery to retinal-pigment epithelium cells and photoreceptors. Due to the immune-privileged nature of the subretinal space, the risk of an immune reaction against viral capsid antigens is minimized, an advantage of subretinal administration in patients with preexisting neutralizing antibodies. Intravitreal administration, with fewer procedure-related complications, is challenged by potential immune response and incomplete vector penetration through the internal limiting membrane. However, novel vectors, optimized by "directed evolution" may address these issues. Nonsurgical in-office suprachoroidal gene delivery offers the potential for greater surface-area coverage of the posterior segment compared to focal subretinal injection, and is not hindered by the internal limiting membrane. However, the vector must pass through multiple layers to reach the targeted retinal layers, and there is a risk of immune response. This review highlights recent developments, challenges, and future opportunities associated with viral and nonviral suprachoroidal gene delivery for the treatment of chorioretinal diseases. While ocular tolerability and short-term effectiveness of suprachoroidal gene delivery have been demonstrated in preclinical models, durability of gene expression, long-term safety, potential systemic exposure, and effective delivery to the macula require further exploration. Although the safety and efficacy of suprachoroidal gene delivery are yet to be proven in clinical trials, further optimization could facilitate nonsurgical in-office suprachoroidal gene therapy.

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.

[White dot syndromes : Principles, diagnostics, and treatment]. / "White-dot-Syndrome" : Grundlagen, Diagnostik und Therapie.

The white dot syndromes include a group of diseases which are characterized by multiple yellowish-white foci in the outer retina, retinal pigment epithelium, and choroid. For clinicians and researchers alike they present significant diagnostic and therapeutic challenges. White dot syndromes include primary inflammatory choriocapillaropathies, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE)/acute multifocal ischemic choriocapillaropathy (AMIC), multiple evanescent white dot syndrome (MEWDS)/acute idiopathic blind spot enlargement (AIBSE), multifocal choroiditis (MFC), punctate inner choroidopathy (PIC), serpiginous choroiditis (SC), acute zonal occult outer retinopathy (AZOOR), and acute macular neuroretinopathy (AMN). Among the primary stromal choroiditis is birdshot retinochoroidopathy (BSRC); however, the pathogenesis of these disorders is largely unknown. Immunological reactions to previous viral infections with a genetic disposition seem to be a common denominator.

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.

Choroidal thickening in a case of carotid cavernous fistula.

A 47-year-old woman was diagnosed with dural carotid cavernous fistula (CCF) in her right eye (RE). Scans of the choroid using Spectralis optic coherence tomography (OCT) demonstrated significant asymmetry in subfoveal choroidal thickness (RE 451 µm, left eye (LE) 367 µm). This asymmetry disappeared after the fistula was embolizated through the ophthalmic artery (RE 341 µm; LE 340 µm). This case suggests that OCT should be considered as an ancillary test in the diagnosis of CCF.