Polypoidal choroidal vasculopathy (PCV) is an eye disease primarily affecting the

choroid The choroid, also known as the choroidea or choroid coat, is a part of the uvea, the vascular layer of the eye. It contains connective tissues, and lies between the retina and the sclera. The human choroid is thickest at the far extreme rear o ...

. It may cause sudden blurring of vision or a

scotoma A scotoma is an area of partial alteration in the field of vision consisting of a partially diminished or entirely degenerated visual acuity that is surrounded by a field of normal – or relatively well-preserved – vision. Every normal mamm ...

in the central

field of vision The visual field is "that portion of space in which objects are visible at the same moment during steady fixation of the gaze in one direction"; in ophthalmology and neurology the emphasis is mostly on the structure inside the visual field and it i ...

. Since

Indocyanine green angiography 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 (790–805 nm) rang ...

gives better imaging of choroidal structures, it is more preferred in diagnosing PCV. Treatment options of PCV include careful observation,

photodynamic therapy Photodynamic therapy (PDT) is a form of phototherapy involving light and a photosensitizing chemical substance used in conjunction with molecular oxygen to elicit cell death ( phototoxicity). PDT is used in treating acne, wet age-related macula ...

, thermal laser, intravitreal injection of

anti-VEGF Anti–vascular endothelial growth factor therapy, also known as anti-VEGF () therapy or medication, is the use of medications that block vascular endothelial growth factor. This is done in the treatment of certain cancers and in age-related macu ...

therapy, or combination therapy.

Pathophysiology

PCV is an ocular disease characterised by abnormally shaped vessels in the

. It is described as an exudative maculopathy, characterised by multiple recurrent serosanguineous

retinal pigment epithelial The pigmented layer of retina or retinal pigment epithelium (RPE) is the pigmented cell layer just outside the neurosensory retina that nourishes retinal visual cells, and is firmly attached to the underlying choroid and overlying retinal visual ...

detachments. Elevated reddish to orange lesions on fundus examination, dilated inner choroidal vessels, and polypoidal vascular structures beneath the

retinal detachment Retinal detachment is a condition where the retina pulls away from the tissue underneath it. It may start in a small area, but without quick treatment, it can spread across the entire retina, leading to serious vision loss and possibly blindness. ...

are other features of PCV.

Etiology

PCV is believed to occur due to abnormalities in the inner choroidal vessels. Since it shares many similarities with

age-related macular degeneration Macular degeneration, also known as age-related macular degeneration (AMD or ARMD), is a medical condition which may result in blurred or no vision in the center of the visual field. Early on there are often no symptoms. Some people experien ...

(AMD), 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.

Diagnosis

PCV may be diagnosed during a routine dilated fundus examination by an ophthalmologist. Fluorescein angiography, Indocyanine green angiography and Optical coherence tomography is also used to diagnose PCV. Since it gives better imaging of choroidal structures, ICGA is more preferred.

Signs and symptoms

In PCV, sudden blurring of vision or a

in the central

may occur in one or both eyes. Another symptom is

metamorphopsia Metamorphopsia (from , ) is a type of distorted vision in which a grid of straight lines appears wavy or partially blank. In addition, metamorphopsia can result in misperceptions of an object's size, shape, or distance to the viewer. People can f ...

. Signs include polypoidal lesions, orange-red lesions in fundus, subretinal fluid, retinal detachment, subretinal hemorrhages, subretinal fibrinous material, hard exudates and

drusen Drusen, from the German word for ''node'' or ''geode'' (singular, "Druse"), are tiny yellow or white accumulations of extracellular material that build up between Bruch's membrane and the retinal pigment epithelium of the eye. The presence of a ...

Classification

If there is presence of polyps without clinical signs of subretinal detachment or hemorrhage, it is classified as Quiescent. It is classified as Active, if there is subretinal or intraretinal fluid, pigment epithelial detachment, subretinal hemorrhage, or fluorescein leakage. Active lesions are further classified as Exudative, Hemorrhagic or Mixed forms.

Complications

Irreversible vision loss in the central field of vision is a complication of PCV. It may also cause choroidal ischemia, inflammation and breaks in Bruch's membrane.

Treatment

Treatment options of PCV include careful observation, photodynamic therapy, Thermal laser, intravitreal injection of anti-VEGF therapy, or combination therapy.

Epidemiology

PCV is commonly seen in patients of age 60-72 years, and can affect both sexes. Prevalence is more in Asian and African descent than Caucasians. PCV is commonly seen in males in Asian population and females in Caucasian population. 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%.

History

Lawrence Yannuzzi first described the Polypoidal choroidal vasculopathy in 1982.

References

{{DEFAULTSORT:Polypoidal choroidal vasculopathy Disorders of choroid and retina