Polypoidal choroidal vasculopathy | |
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Specialty | Ophthalmology |
Symptoms | Blurring of vision, scotoma |
Complications | Irreversible vision loss in the central field of vision |
Types | Quiescent or Active |
Risk factors | Smoking [1] |
Diagnostic method | fundus examination, Indocyanine green angiography, Fluorescein angiography or Optical coherence tomography |
Treatment | observation, photodynamic therapy, thermal laser, anti-VEGF therapy or combination therapy |
Polypoidal choroidal vasculopathy (PCV) is an eye disease primarily affecting the choroid. It may cause sudden blurring of vision or a scotoma in the central field of vision. Since Indocyanine green angiography gives better imaging of choroidal structures, it is more preferred in diagnosing PCV. Treatment options of PCV include careful observation, photodynamic therapy, thermal laser, intravitreal injection of anti-VEGF therapy, or combination therapy.
PCV is an ocular disease characterised by abnormally shaped vessels in the choroid. [2] It is described as an exudative maculopathy, characterised by multiple recurrent serosanguineous retinal pigment epithelial detachments. [3] Elevated reddish to orange lesions on fundus examination, dilated inner choroidal vessels, and polypoidal vascular structures beneath the retinal detachment are other features of PCV. [4]
PCV is believed to occur due to abnormalities in the inner choroidal vessels. [3] Since it shares many similarities with age-related macular degeneration (AMD), [3] it was previously considered as a subtype of AMD, but later studies shown that PCV may be a variant of type 1 neovascularization of any origin. [5] [4]
PCV may be diagnosed during a routine dilated fundus examination by an ophthalmologist. [2] Fluorescein angiography, Indocyanine green angiography and Optical coherence tomography is also used to diagnose PCV. [2] Since it gives better imaging of choroidal structures, ICGA is more preferred. [3]
In PCV, sudden blurring of vision or a scotoma in the central field of vision may occur in one or both eyes. [2] Another symptom is metamorphopsia. [1] Signs include polypoidal lesions, orange-red lesions in fundus, subretinal fluid, retinal detachment, subretinal hemorrhages, subretinal fibrinous material, hard exudates and drusen. [1]
If there is presence of polyps without clinical signs of subretinal detachment or hemorrhage, it is classified as Quiescent. [3] It is classified as Active, if there is subretinal or intraretinal fluid, pigment epithelial detachment, subretinal hemorrhage, or fluorescein leakage. [3] Active lesions are further classified as Exudative, Hemorrhagic or Mixed forms. [3]
Irreversible vision loss in the central field of vision is a complication of PCV. [2] It may also cause choroidal ischemia, inflammation and breaks in Bruch's membrane. [3]
Treatment options of PCV include careful observation, photodynamic therapy, Thermal laser, intravitreal injection of anti-VEGF therapy, or combination therapy. [6]
PCV is commonly seen in patients of age 60-72 years, and can affect both sexes. [7] Prevalence is more in Asian and African descent than Caucasians. [2] PCV is commonly seen in males in Asian population and females in Caucasian population. [7] In Chinese and Japanese population, prevalence rates in patients with presumed AMD is approximately 24.5%-54.7%, 49% in the Taiwanese population and 24.6% in the Korean population, while in Caucasian patients with presumed AMD, prevalence rate is only 4% - 9.8%. [7]
Lawrence Yannuzzi first described the Polypoidal choroidal vasculopathy in 1982. [6]
Fluorescein angiography (FA), fluorescent angiography (FAG), or fundus fluorescein angiography (FFA) is a technique for examining the circulation of the retina and choroid using a fluorescent dye and a specialized camera. Sodium fluorescein is added into the systemic circulation, the retina is illuminated with blue light at a wavelength of 490 nanometers, and an angiogram is obtained by photographing the fluorescent green light that is emitted by the dye. The fluorescein is administered intravenously in intravenous fluorescein angiography (IVFA) and orally in oral fluorescein angiography (OFA). The test is a dye tracing method.
Central serous chorioretinopathy, also known as central serous retinopathy (CSR), is an eye disease that causes visual impairment, often temporary, usually in one eye. When the disorder is active it is characterized by leakage of fluid under the retina that has a propensity to accumulate under the central macula. This results in blurred or distorted vision (metamorphopsia). A blurred or gray spot in the central visual field is common when the retina is detached. Reduced visual acuity may persist after the fluid has disappeared.
Intravitreal is a route of administration of a drug, or other substance, in which the substance is delivered into the vitreous humor of the eye. "Intravitreal" literally means "inside an eye". Intravitreal injections were first introduced in 1911 when Ohm gave an injection of air into the vitreous humor to repair a detached retina. In the mid-1940s, intravitreal injections became a standard way to administer drugs to treat endophthalmitis and cytomegalovirus retinitis.
Presumed ocular histoplasmosis syndrome (POHS) is a syndrome affecting the eye, which is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the optic disc and maculopathy.
Angioid streaks, also called Knapp streaks or Knapp striae, are small breaks in Bruch's membrane, an elastic tissue containing membrane of the retina that may become calcified and crack. Up to 50% of angioid streak cases are idiopathic. It may occur secondary to blunt trauma, or it may be associated with many systemic diseases. The condition is usually asymptomatic, but decrease in vision may occur due to choroidal neovascularization.
Fundus photography involves photographing the rear of an eye, also known as the fundus. Specialized fundus cameras consisting of an intricate microscope attached to a flash enabled camera are used in fundus photography. The main structures that can be visualized on a fundus photo are the central and peripheral retina, optic disc and macula. Fundus photography can be performed with colored filters, or with specialized dyes including fluorescein and indocyanine green.
Choroidal neovascularization (CNV) is the creation of new blood vessels in the choroid layer of the eye. Choroidal neovascularization is a common cause of neovascular degenerative maculopathy commonly exacerbated by extreme myopia, malignant myopic degeneration, or age-related developments.
Intraocular hemorrhage is bleeding (hemorrhage) inside the eye. Bleeding can occur from any structures of the eye where there is a presence of vasculature or blood flow. It can bleed inside the anterior chamber, vitreous cavity, retina, choroid, suprachoroidal space, or Optic disc. Intraocular hemorrhage can be subdivided depending on the location of the bleed. It may be the result of physical trauma, an uncommon side effect due to post op ocular surgery or other diseases, injuries or disorders. Severe bleeding may cause high pressures inside the eye, leading to blindness.
Macular telangiectasia is a condition of the retina, the light-sensing tissue at the back of the eye that causes gradual deterioration of central vision, interfering with tasks such as reading and driving.
The Fuchs spot is a degeneration of the macula in case of high myopia. It is named after the two persons who first described it: Ernst Fuchs, who described a pigmented lesion in 1901, and Forster, who described subretinal neovascularisation in 1862. It occur due to proliferation of retinal pigment epithelium associated with choroidal hemorrhage. The size of the spots are proportionate to the severity of the pathological myopia.
Multiple evanescent white dot syndrome (MEWDS) is an uncommon inflammatory condition of the retina that typically affects otherwise healthy young females in the second to fourth decades of life.
Punctate inner choroiditis (PIC) is an inflammatory choroiditis which occurs mainly in young women. Symptoms include blurred vision and scotomata. Yellow lesions are mainly present in the posterior pole and are between 100 and 300 micrometres in size. PIC is one of the so-called White Dot Syndromes. PIC has only been recognised as a distinct condition as recently as 1984 when Watzke identified 10 patients who appeared to make up a distinct group within the White Dot Syndromes.
Joan Whitten Miller is a Canadian-American ophthalmologist and scientist who has made notable contributions to the treatment and understanding of eye disorders. She is credited for developing photodynamic therapy (PDT) with verteporfin (Visudyne), the first pharmacologic therapy for retinal disease. She also co-discovered the role of vascular endothelial growth factor (VEGF) in eye disease and demonstrated the therapeutic potential of VEGF inhibitors, forming the scientific basis of anti-VEGF therapy for age-related macular degeneration (AMD), diabetic retinopathy, and related conditions.
Geographic atrophy (GA), also known as atrophic age-related macular degeneration (AMD) or advanced dry AMD, is an advanced form of age-related macular degeneration that can result in the progressive and irreversible loss of retinal tissue which can lead to a loss of visual function over time. It is estimated that GA affects >5 million people worldwide and approximately 1 million patients in the US, which is similar to the prevalence of neovascular (wet) AMD, the other advanced form of the disease.
Pachychoroid disorders of the macula represent a group of diseases affecting the central part of the retina of the eye, the macula. Due to thickening and congestion of the highly vascularized layer underneath the macula, the choroid, damage to the retinal pigment epithelium and the retinal photoreceptor cells ensues. This leads to impaired vision. The best known representative of the pachychoroid disease spectrum, central serous chorioretinopathy, is the fourth most common cause of irreversible damage to the macula:.
Indocyanine green angiography (ICGA) is a diagnostic procedure used to examine choroidal blood flow and associated pathology. Indocyanine green (ICG) is a water soluble cyanine dye which shows fluorescence in near-infrared range, with peak spectral absorption of 800-810 nm in blood. The near infrared light used in ICGA penetrates ocular pigments such as melanin and xanthophyll, as well as exudates and thin layers of sub-retinal vessels. Age-related macular degeneration is the third main cause of blindness worldwide, and it is the leading cause of blindness in industrialized countries. Indocyanine green angiography is widely used to study choroidal neovascularization in patients with exudative age-related macular degeneration. In nonexudative AMD, ICGA is used in classification of drusen and associated subretinal deposits.
Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.
Conbercept, sold under the commercial name Lumitin, is a novel vascular endothelial growth factor (VEGF) inhibitor used to treat neovascular age-related macular degeneration (AMD) and diabetic macular edema (DME). The anti-VEGF was approved for the treatment of neovascular AMD by the China State FDA (CFDA) in December 2013. As of December 2020, conbercept is undergoing phase III clinical trials through the U.S. Food and Drug Administration’s PANDA-1 and PANDA-2 development programs.
Choroidal nevus is a type of eye neoplasm that is classified under choroidal tumors as a type of benign (non-cancerous) melanocytic tumor. A choroidal nevus can be described as an unambiguous pigmented blue or green-gray choroidal lesion, found at the front of the eye, around the iris, or the rear end of the eye.
Hypotony maculopathy is maculopathy due to very low intraocular pressure known as ocular hypotony. Maculopathy occurs either due to increased outflow of aqueous humor through angle of anterior chamber or less commonly, due to decreased aqueous humor secretion by ciliary body.