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Neovascularization of the macula, a common complication of many chorioretinal diseases such as neovascular age-related macular degeneration, polypoidal choroidal vasculopathy, and pathologic myopia results from increased synthesis of vascular endothelial growth factor (VEGF) by the retinal pigment epithelium and/or Müller cells because of localized ischemia and inflammation. The Consensus on Neovascular AMD Nomenclature (CONAN) study group acknowledged that these vessels may originate from either the choriocapillaris or the retinal microvasculature, prompting them to propose the term 'macular neovascularization' (MNV) to include intraretinal, subretinal, and sub-pigment epithelial neovascularization localized to the macula. MNV frequently appears as a grey-green macular lesion with overlying intraretinal thickening and/or subretinal exudation, causing metamorphopsia, reduced central vision, relative central scotoma, decreased reading speed, and problems with color recognition. Multimodal imaging with optical coherence tomography (OCT), OCT angiography, dye-based angiographies, fundus autofluorescence, and multiwavelength photography help establish the diagnosis and aid in selecting an appropriate treatment. The standard of care for MNV is usually intravitreal anti-VEGF injections, though thermal laser photocoagulation, verteporfin photodynamic therapy, and vitreoretinal surgery are occasionally used. This current review discusses the etiology and clinical features of MNV, the role of multimodal imaging in establishing the diagnosis, and the available therapeutic options.

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Vogt-Koyanagi-Harada (VKH) disease is a multisystem inflammatory disease that usually presents with bilateral panuveitis. Unilateral manifestations of VKH, albeit rare, have been described. Choroidal neovascularization may occur as a complication during the disease. Reactivation of uveitis may also occur during treatment. A patient with unilateral features of VKH disease presented with two distinct types of choroidal neovascular membranes and two episodes of reactivation of posterior uveitis. He underwent treatment with a combination of systemic steroids/immunosuppressive agents, intravitreal anti-vascular endothelial growth factor (anti-VEGF) agents, and dexamethasone implants with good clinical response.  Patients with VKH disease need to be diagnosed early, treated adequately with a combination of systemic and ocular medication, and followed up diligently for any complication that may arise, to optimize visual acuity.

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PURPOSE: This study aimed to assess the optical coherence tomography (OCT) characteristics for differentiating scars in the scarred stages of macular neovascularization (MNV) in age-related macular degeneration (AMD). METHODS: Medical records of 20 patients, 10 in each group with type 1 and type 2 MNV, were selected for the study. Participants chosen were above 50 years of age and underwent comprehensive eye examination alongside indocyanine green angiography (ICGA), fundus fluorescence angiography (FFA), and Spectralis optical coherence tomography (SOCT) (Heidelberg Engineering, Germany), respectively. The qualitative and quantitative OCT measurements, such as the frequency of outer retinal tubulations, presence of cystoid spaces, scar area, choroid thickness, retinal thickness, presence of disorganization in retinal layers (DRIL), foveal contour, and involvement of retinal layers in the scar, were meticulously evaluated and compared between the two groups. RESULTS: Significant disparities between type 1 MNV and type 2 MNV in choroidal thickness were identified in the nasal and superior quadrants within 1 mm, in the superior quadrant within 3 mm, and in all quadrants except the inferior quadrant within 6 mm. Overall, type 2 MNV showed thinner choroid than type 1 MNV. CONCLUSION: Although there are several overlapping features noticed between the groups, the OCT was able to pick up characteristic features that aid in differentiating type 1 (polypoidal choroidal vasculopathy (PCV)) and type 2 (classic) MNV in AMD. This precise differentiation has the potential to assist ophthalmologists in making well-informed decisions, thereby enhancing patient care.

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PURPOSE: To report a rare presentation of a proven case of Mycobacterium chimaera infection presenting as multifocal choroiditis with recurrent choroidal neovascular membrane (CNVM) in one eye, initially misdiagnosed as punctate inner choroidopathy and later developed serpiginous-like choroiditis in the other eye. METHODS: Retrospective case report with a review of existing literature. RESULTS: A 30-year-old women presented with metamorphopsia (OD) and best-corrected visual acuity (BCVA) of 6/24 (OD) and was diagnosed to have punctate inner choroidopathy with CNVM (OD). Since then, she had received four intravitreal anti-vascular endothelial growth factor injections over 3 years. Two years later, she developed a slowly progressing choroidal lesion radiating from the disc in a serpiginoid manner in the left eye. There was no vitritis. Labs revealed a positive QuantiFERON-TB Gold test. High-resolution computed tomography of the thorax showed sub-centimetre noncalcified lymph nodes in subcarinal and perivascular regions, minimal pleural thickening in left lower zone, minimal pericardial effusion, bronchiectatic changes, and fibrotic strands in right middle and left lower lobes. Bronchoalveolar lavage grew M. chimaera intracellularae (matrix-assisted laser desorption/ionization time-of-flight mass spectrometry). She was given a course of clarithromycin, moxifloxacin, rifampicin, and doxycycline for 12 months. Though the right eye remained stable, choroidal lesion in the left eye continued to progress threatening the fovea, requiring oral steroids, methotrexate, and an intravitreal dexamethasone implant. At the last follow-up, her BCVA was 6/18 (OD) and 6/6 (OS). Both eyes were stable. CONCLUSION: This case highlights a rare presentation of proven M. chimaera infection presenting as multifocal choroiditis with recurrent CNVM in one eye and serpiginous-like choroiditis in the other eye, requiring aggressive treatment to salvage the vision.

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Angioid streaks (AS) are recognized as irregular, linear dehiscences of Bruch's membrane, often associated with systemic diseases. We present the case of a 50-year-old woman initially diagnosed with AS during a routine optometric examination. Subsequent ophthalmological evaluation revealed bilateral AS with calcified drusen. Two years post-diagnosis, she developed blurred vision in her right eye due to the choroidal neovascular membrane adjacent to the macular AS. Further evaluation uncovered clinical signs consistent with pseudoxanthoma elasticum (PXE), including characteristic skin lesions. A multidisciplinary approach involving ophthalmology, dermatology, and cardiovascular specialists was initiated. Histopathological confirmation of PXE was obtained through a skin biopsy. PXE, an autosomal recessive disorder characterized by elastin calcification, presents systemic manifestations necessitating comprehensive evaluation and monitoring. This case demonstrates the importance of recognizing ocular complications in PXE and advocates for early multidisciplinary intervention to mitigate potential vision and life-threatening outcomes.

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We aim to report the ocular phenotype and molecular genetic findings in two Czech families with Sorsby fundus dystrophy and to review all the reported TIMP3 pathogenic variants. Two probands with Sorsby fundus dystrophy and three first-degree relatives underwent ocular examination and retinal imaging, including optical coherence tomography angiography. The DNA of the first proband was screened using a targeted ocular gene panel, while, in the second proband, direct sequencing of the TIMP3 coding region was performed. Sanger sequencing was also used for segregation analysis within the families. All the previously reported TIMP3 variants were reviewed using the American College of Medical Genetics and the Association for Molecular Pathology interpretation framework. A novel heterozygous variant, c.455A>G p.(Tyr152Cys), in TIMP3 was identified in both families and potentially de novo in one. Optical coherence tomography angiography documented in one patient the development of a choroidal neovascular membrane at 54 years. Including this study, 23 heterozygous variants in TIMP3 have been reported as disease-causing. Application of gene-specific criteria denoted eleven variants as pathogenic, eleven as likely pathogenic, and one as a variant of unknown significance. Our study expands the spectrum of TIMP3 pathogenic variants and highlights the importance of optical coherence tomography angiography for early detection of choroidal neovascular membranes.

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Peripapillary choroidal neovascular membrane (PCNM) is an abnormal growth of blood vessels beneath the retina near the optic disc. We report a case of a 60-year-old Saudi female with a history of hypertension, hypothyroidism, and epilepsy who presented to the emergency room (ER), reporting a sudden decrease in vision over the past month. Ophthalmic examination revealed reduced visual acuity. The patient received aflibercept via intravitreal injection every four weeks. On follow-up, she reported improvement in symptoms. It has been shown that intravitreal anti-vascular endothelial growth factor (VEGF) not only preserves visual acuity but also produces anatomic improvement when used alone or in conjunction with other therapeutic modalities like photodynamic therapy, laser photocoagulation, and subretinal surgery, as PCNM is aberrant blood vessel growth under the retina. Only a few cases have been recorded in Saudi Arabia; we report this case to emphasize the importance of diagnosis and timely treatment with anti-VEGF.

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Purpose: To present a rare case of subfoveal choroidal neovascular membrane (CNVM) secondary to idiopathic intracranial hypertension. Methods: A case was evaluated. Results: A 21-year-old woman presented with a 2-week history of painless blurred vision in the right eye. She described initial metamorphopsia and intermittent bitemporal headaches lasting 30 minutes. She denied pain with eye movements and a history of trauma. Her body mass index was 49 kg/m2. The visual acuity (VA) was 20/320 OD and 20/20 OS; there was no relative afferent pupillary defect. A dilated fundus examination showed bilateral optic disc edema and a subfoveal CNVM in the right eye. The patient was started on oral acetazolamide 500 mg twice daily and treated with 2 intravitreal antivascular endothelial growth factor (anti-VEGF) injections. Three months later, the VA was 20/30 in the right eye and the disc edema had improved. Conclusions: CNVMs in the setting of idiopathic intracranial hypertension-related papilledema may be subfoveal and have an excellent response to anti-VEGF agents.

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The purpose of this case report is to describe the optical coherence tomography (OCT) features of a peripapillary choroidal neovascular membrane (PPCNVM) in an eye with morning glory disc anomaly (MGDA). A PPCNVM appears as a hyper-reflective mass in the peripapillary area. It should be distinguished from peripapillary hyper-reflective ovoid mass-like structures, which are markers of axoplasmic flow stasis. This case report describes the distinguishing features between the two. The presence of intraretinal cystic spaces are indicative of an active PPCNVM. In conclusion, MGDA can be associated with PPCNVM and OCT can be used in its detection.

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Inherited retinal diseases (IRDs) are the most common cause of blindness in working-age adults. Macular neovascularization (MNV) may be a presenting feature or occurs as a late-stage complication in several IRDs. We performed an extensive literature review on MNV associated with IRDs. MNV is a well-known complication of Sorsby fundus dystrophy and pseudoxanthoma elasticum. Those with late-onset Stargardt disease may masquerade as exudative age-related macular degeneration (AMD) when MNV is the presenting feature. Peripherinopathies may develop MNV that responds well to a short course of anti-vascular endothelial growth factor (anti-VEGF) therapy, while bestrophinopathies tend to develop MNV in the early stages of the disease without vision loss. Enhanced S-cone syndrome manifests type 3 MNV that typically regresses into a subfoveal fibrotic nodule. MNV is only a rare complication in choroideraemia and rod-cone dystrophies. Most IRD-related MNVs exhibit a favorable visual prognosis requiring less intensive regimens of anti-vascular endothelial growth factor therapy compared to age-related macular degeneration. We discuss the role of key imaging modalities in the diagnosis of MNV across a wide spectrum of IRDs and highlight the gaps in our knowledge with respect to the natural history and prognosis to pave the way for future directions of research.

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PURPOSE: To describe a complex case of ocular tuberculosis reactivation with anterior uveitis, choroiditis and inflammatory choroidal neovascular membrane (CNVM) following immune checkpoint inhibitor (ICPI) treatment of malignant mucosal melanoma. METHODS: A retrospective collection of medical history, clinical findings and multimodal imaging with literature review of the topic was conducted. RESULTS: A 52-year-old Romanian female developed reduced vision and photophobia after three cycles of ICPI therapy comprised of ipilimumab and nivolumab. Bilateral anterior uveitis, multiple left eye choroidal lesions and a CNVM were confirmed using slit-lamp examination with ancillary multimodal imaging. Retinal changes in the right eye as well as a history of previously treated posterior uveitis and high-risk ethnicity increased clinical suspicion for ocular tuberculosis (TB) reactivation. The diagnosis was confirmed by TB positivity on polymerase chain reaction (PCR) analysis of lung aspirate followed by significant clinical improvement on systemic anti-tubercular therapy (ATT), systemic steroids and anti-vascular endothelial growth factor (VEGF) therapy. CONCLUSIONS: ICPIs can cause a myriad of ocular issues, both by primary immunomodulatory effects as well as secondary reactivation of latent disease.

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Purpose: To report a case of acquired conforming-type focal choroidal excavation (FCE) secondary to a pachychoroid choroidal neovascular membrane (CNVM) triggered by central serous chorioretinopathy (CSCR). Methods: A case and its findings were analyzed. Results: A 54-year-old Asian man who had spontaneous resolution of CSCR in the right eye presented with a pachychoroid CNVM and FCE 1 year after the initial CSCR diagnosis. Intravitreal antivascular endothelial growth factor injections were initiated, and the subretinal fluid and intraretinal hemorrhage resolved. The patient was followed for FCE progression for 3 years. Conclusions: Acquired FCE can occur secondary to CSCR and pachychoroid CNVM. The pathogenesis may be focal choroidal ischemia, choroidal vascular collapse, and fibrosis leading to choroidal excavation. This case highlights the progression of the spectrum of pachychoroid disorders from CSCR, pachychoroid CNVM, and subsequent acquired confirming-type FCE. Further research is needed to assess other diseases leading to acquired FCE and to determine the underlying mechanism.

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Purpose: Cystoid macular degeneration (CMD) is a feature of chronic central serous chorioretinopathy (CSCR). Present study intended to analyze the clinical presentation, risk factors, choroidal features, and outcome of CMD in CSCR. Methods: This was a retrospective, record-based descriptive study, which included chronic CSCR eyes with CMD. Demographic profile and clinical history were obtained from medical records. Spectralis spectral domain optical coherence tomography (SDOCT; Heidelberg Engineering,Germany) was used for acquiring SDOCT images and for performing fluorescein angiography , indocyanine green angiography , and optical coherence tomography (OCT) angiography. Results: The study included 101 eyes of 69 consecutive patients of CSCR having CMD. The mean age of patients was 56 ± 9.4 years (range 40-79 years), and majority (63, 91.3%) of the patients were male. Prior history of corticosteroid use was present in seven (10.1%) patients. Mean time interval between the first diagnosis of CSCR and appearance of CMD was 55.3 ± 33.9 months. CMD was located away from the fovea in majority of eyes (68, 67.3%). Mean subfoveal choroidal thickness was 396.71 ± 90.5 µm. Subretinal pigment epithelium choroidal neovascularization was noted in four (3.96%) eyes. Conclusion: CMD appears as a late complication of CSCR and is usually present away from the fovea. Such eyes had thickened choroid and fewer cases had associated choroidal neovascularization. Further comparative studies would be needed to validate these findings.

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A patient presented with melanocytoma and associated choroidal neovascular membrane with hemorrhage involving the macula. The patient was treated with monthly aflibercept (Eylea) injections with significant improvement of best corrected visual acuity. In this report, we explore the development of a choroidal neovascular membrane (CNVM) formation in a patient with melanocytoma and the effect of intravitreal aflibercept (Eylea) on disease course. Case report study used patient data obtained from examination and imaging. The patient was treated with monthly intravitreal aflibercept injections leading to complete resolution of CNVM and hemorrhage, with significant improvement of best corrected visual acuity. Awareness and proper monitoring for the sequelae of melanocytoma are important for early detection and prevention of visually threatening outcomes. In cases of melanocytoma-associated CNVM formation with large subretinal hemorrhage, intravitreal aflibercept can be an effective tool for inducing CNVM regression and allowing improvement of visual acuity.

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Background and Objectives: Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a peripheral retinal vascular abnormality that is likely underreported. We review the differential diagnoses, etiology, and treatment options for PEHCR. Methods: We present a case of an asymptomatic 72-year-old female referred following left eye fundus photography finding of the peripheral lesion. Results: Fundus photography demonstrated a large temporal pigment epithelial detachment (PED) with adjacent fibrovascular membrane. Optical coherence tomography (OCT) confirmed the PED with trace subretinal fluid. Fluorescein angiography (FA) demonstrated early and late hypofluorescence of the PED with late leakage of the adjacent temporal fibrovascular membrane. Observation was elected, visual acuity remained unaffected, and the PED spontaneously resolved. Conclusions: Due to the peripheral location, patients often present as asymptomatic; however, vision loss can occur due to vitreous hemorrhage or extension of subretinal fluid, hemorrhage, or exudate to the macula. Commonly, these lesions are referred with concern for choroidal melanoma due to their large, dark, elevated presentation in the peripheral retina. Multimodal testing using B-scan, FA, and OCT is important in establishing the proper diagnosis. PEHCR lesions can often be observed without treatment, though intravitreal injection of anti-VEGF is increasingly used to prevent secondary causes of vision loss.

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Purpose: Presumed ocular histoplasmosis syndrome (POHS) is a posterior segment disorder that is usually subclinical unless choroidal neovascular membrane (CNVM) develops. It is thought to be the sequela of a prior systemic infection with Histoplasma capsulatum, and evidence supporting this association is based on epidemiologic, animal, and few enucleation studies. Acute presentation of chorioretinal involvement during an initial histoplasmosis systemic infection in immunocompetent patients is rarely reported, presumably due to the usual lack of or minimal symptoms of both the systemic and ocular disease. We report on an immunocompetent male with choroidal lesions detected during disseminated histoplasmosis infection and characterize the lesions using multimodal imaging. Observations: A 17-year-old male presented when routine optometry screening detected two deep, yellowish-white lesions in the left fundus. Optical coherence tomography (OCT) imaging confirmed a choroidal mass with extension through Bruch's membrane into the subretinal space and a small amount of subretinal fluid. Fluorescein angiography was suggestive of CNVM. There were no clinical findings of intraocular inflammation, and the patient was initially lost to follow-up. Eight weeks after last follow-up, the patient presented to the emergency department with fatigue, mild respiratory symptoms, and abdominal pain for the last month. Imaging revealed a mediastinal mass with hilar extension and innumerable nodules throughout the lung and spleen. Serum Histoplasma IgM/IgG were positive, and biopsy of the mediastinal mass revealed Histoplasma organisms. The patient was treated with antifungals and discharged. The patient underwent an extensive immunologic evaluation while admitted, which did not reveal an underlying immunodeficiency. On last follow-up, the choroidal lesions were smaller and more consolidated, and the subretinal fluid had resolved. Conclusions and Importance: We present a patient with choroidal lesions in the setting of disseminated systemic histoplasmosis infection and characterize a lesion using multimodal imaging. The presentation of acute chorioretinal lesions in the setting of biopsy proven systemic Histoplasma infection supports H. capsulatum as the etiology of POHS.

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Purpose: Swept-source optical coherence tomography angiography (SS-OCTA) was used to detect the presence of macular neovascularization (MNV) in eyes with exudative central serous chorioretinopathy (CSCR). Observations: Case 1 is a 73-year-old man using prednisolone drops with 20/40 vision and a retinal pigment epithelial detachment (PED) associated with subretinal fluid (SRF) and no clinical signs of age-related macular degeneration (AMD) in his left eye. Three months after presentation he underwent SS-OCTA imaging that revealed MNV. He received a series of intravitreal anti-vascular endothelial growth factor (VEGF) injections and SS-OCTA imaging showed a decrease in the MNV lesion size with resolution of the SRF. VA improved to 20/25. Case 2 is a 65-year-old man with recent oral steroid use, presenting with 20/20 vision, a PED, SRF, and no evidence of AMD. SS-OCTA imaging at presentation revealed MNV, which worsened on interval SS-OCTA imaging after the patient deferred treatment. Intravitreal anti-VEGF therapy was then performed with resolution of the SRF and VA improved to 20/15. A total of six cases of type 1 MNV were diagnosed with CSCR. Most were men with a history of steroid or testosterone use and were treated with good response. Conclusions and importance: SS-OCTA imaging provides a convenient non-invasive strategy for identifying CSCR eyes with MNV without the need for dye-based angiography.

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PURPOSE: To report two cases masquerading as TORCH but eventually diagnosed with Enhanced S-cone Syndrome (ESCS). METHODS: Descriptive case report. RESULTS: Case 1: A ten-month-old boy presented with high hypermetropia, strabismus and bilateral chorioretinal pigmented scars with a history of cat scratch of his mother during pregnancy. He was treated for suspected toxoplasma retinitis. Choroidal neovascular membranes (CNV) were diagnosed bilaterally and treated with intravitreal bevacizumab. Genetic testing showed homozygote mutation in NR2E3 gene. Case 2: A two-year old girl presented with bilateral high hypermetropia and strabismus. Funduscopy revealed extrafoveal chorioretinal lesions and surrounding subretinal fibrosis. An elevated titer of anti-toxocara IgG antibodies was detected and managed accordingly. LE CNV was diagnosed and treated with intravitreal bevacizumab. Genetic testing disclosed homozygote mutation in NR2E3. CONCLUSION: Ocular manifestations in ESCS can be reminiscent to TORCH. CNV may develop with an incidence of 15%. We report the youngest patient with ESCS-associated CNV.

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@#A 30-year-old, monocular myopic female consulted for new onset scotoma on her left eye. Best corrected visual acuity was 20/20 for the left eye. Fundus exam revealed a hypopigmented lesion in the inferior juxtafoveal area with no overlying vitreous reaction/opacity. Spectral domain optical coherence tomography (SD-OCT) showed focal disruption and elevation of the inner choroid and retinal pigment epithelium (RPE), indicating punctate inner choroidopathy (PIC). OCT angiography (OCT-A) revealed the presence of a choroidal neovascular membrane (CNV) on RPE-RPE fit segmentation. The CNV and lesion resolved upon treatment with one intravitreal bevacizumab injection and oral prednisone 60 mg (1.2 mg/kg body weight) per day tapering over 2 months. This is the first reported case of PIC in a Filipino that was diagnosed and treated with the guidance of high-resolution SD-OCT and OCT-A. These diagnostic tests proved useful in identifying the lesions based on cross-sectional views of the retina, RPE and choroid.

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As optical coherence tomography angiography is revolutionizing the ophthalmology world, the uveitis community is learning to understand where and how this new powerful imaging tool fits into the management of the panorama of ocular inflammations and infections. A non-invasive method of studying the retinal and choroidal vasculature, OCTA allows for the assessment of vessel density changes during active and inactive uveitis making it the natural imaging application of choice in uveitis clinical trials. However, these data and results are of limited utility to the ophthalmologists who are looking to apply OCTA in their everyday uveitis clinic. If employed strategically, OCTA can be a powerful tool for the uveitis specialist to evaluate iris involvement in viral uveitis; to assess the integrity of the vascular layers in the settings of white dot syndromes; to distinguish inflammatory choroidal neovascularization from outer retinal avascular inflammatory material; and to diagnose and follow infectious choroidal granulomas and satellite foci of chorioretinal inflammation without the need to administer dyes. The present review will analyze all the recent publications that apply OCTA in uveitis to offer the reader a guide on how to maximize the utility of this imaging modality in a clinical practice.

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