Polypoidal choroidal vasculopathy

Polypoidal Choroidal Vasculopathy (PCV) is a disease of the eye that affects the choroidal blood vessels and can lead to vision loss. It is considered a variant of age-related macular degeneration (AMD), but with distinct clinical features and a different natural history. PCV is characterized by the presence of polyp-like lesions in the choroid that can lead to serous and hemorrhagic detachments of the retinal pigment epithelium (RPE) and the neurosensory retina.

Etiology and Pathogenesis

The exact cause of PCV is not fully understood, but it is believed to involve a combination of genetic, environmental, and systemic factors. Risk factors for PCV include age, hypertension, and a history of smoking. Genetic predispositions have also been identified, with certain genes and genetic polymorphisms being associated with an increased risk of developing the condition.

Clinical Presentation

Patients with PCV may present with a variety of symptoms, including visual distortion, metamorphopsia, and sudden vision loss. The disease is often bilateral, though the severity can vary significantly between eyes. On examination, characteristic orange-red, spheroidal, polypoidal lesions can be observed, typically located beneath the RPE. These lesions are best visualized using indocyanine green angiography (ICGA), which is considered the gold standard for diagnosing PCV.

Diagnosis

The diagnosis of PCV is primarily based on clinical examination and imaging studies. Fluorescein angiography (FA) and ICGA are key diagnostic tools, with ICGA showing a network of abnormal choroidal vessels ending in polypoidal lesions. Optical coherence tomography (OCT) is also useful for identifying the presence of subretinal fluid and the characteristic elevation of the RPE.

Treatment

Treatment options for PCV include laser photocoagulation, photodynamic therapy (PDT) with verteporfin, and intravitreal injections of anti-VEGF medications. The choice of treatment depends on the specific characteristics of the lesions and the overall health of the patient. Recent studies have shown that anti-VEGF therapy, either alone or in combination with PDT, can be effective in improving visual acuity and reducing lesion size in patients with PCV.

Prognosis

The prognosis for patients with PCV varies. While some patients may experience a stabilization of their condition with treatment, others may suffer from recurrent bleeding and persistent fluid, leading to progressive vision loss. Early detection and treatment are crucial in managing PCV and preserving vision.

Epidemiology

PCV is more commonly diagnosed in individuals of Asian and African descent, though it can occur in people of any ethnicity. It tends to present later in life, usually affecting individuals in their 50s and 60s.