Polypoidal choroidal vasculopathy.

Polypoidal choroidal vasculopathy was first described as a peculiar hemorrhagic disorder of the macula, characterized by recurrent sub-retinal and sub-retinal pigment epithelium bleeding in middle aged black women. The use of indocyanine green angiography and subsequently of optical coherent tomography has widened our ability to study and understand the pathophysiology of this disorder. The primary abnormality involves the choroidal circulation, and the characteristic lesion is an inner choroidal vascular network of vessels ending in an aneurysmal bulge or outward projection, visible clinically as a reddish orange, spheroid, polyp-like structure. We have also recognized that individuals of African-American and Asian descents are more at risk for developing polypoidal choroidal vasculopathy as the disorder seems to preferentially affect pigmented individuals. However, it has been shown that while that still holds true, patients of other racial backgrounds may be afflicted. Particularly, polypoidal choroidal vasculopathy has been found to be present in about 8-13% of white patients with clinical appearance of exudative age-related macular degeneration. Polypoidal choroidal vasculopathy has also been reported in Irish, French, German, and Italian patients. The natural course of the disease often follows a remitting-relapsing course, and clinically, it is associated with chronic, multiple, recurrent serosanguineous detachments of the retinal pigment epithelium and neurosensory retina with long-term preservation of good vision. Photodynamic treatment appears to be a promising alternative to conventional laser therapy, for the treatment of polypoidal choroidal vasculopathy. In conclusion, polypoidal choroidal vasculopathy seems to be a distinct clinical entity that should be differentiated from other types of choroidal neovascularization associated with age-related macular degeneration and other known choroidal degenerative, inflammatory, and ischemic disorders.

Antiangiogenesis agents.

Although these preliminary results on the use of antiangiogenesis drugs for the treatment of neovascular AMD appear promising, double-masked, placebo-controlled, multicenter clinical trials are needed to demonstrate the therapeutic efficacy of such treatments. For example, the first antiangiogenesis drug tested in AMD, interferon alpha-2a, raised great enthusiasm. Indeed, interferon alpha-2a had been shown to be antiangiogenic in animal and in vitro models. It proved to be ineffective, however, in halting the progression of neovascular AMD in a double-masked, placebo-controlled clinical trial [28]. Another antivasogenesis drug tested in a phase 3 clinical trial is thalidomide [67]. Although the enrollment of patients is finished, the results are not yet known.

Polypoidal choroidal vasculopathy.

Polypoidal choroidal vasculopathy seems to be a distinct clinical entity that should be differentiated from other types of CNV associated with AMD and other known choroidal degenerative, inflammatory, and ischemic disorders. The principle abnormality seen in PCV, notably the branching vascular network and polypoidal structures at the borders of the lesion, seem to be unique to the disorder. In patients with serosanguineous detachment of the pigment epithelium, particularly in those with increased risk factors such as African American or Asian race, ICG should be performed to evaluate the choroidal vascular abnormality to establish a more definitive diagnosis. If the characteristic vascular lesion of PCV is seen, a conservative approach to management should be taken unless there is a persistent or progressive exudative change that threatens the central macula. In that event, there may be a rationale for photo-coagulation treatment of leaking aneurysmal or polypoidal components within the vascular lesion, but not the entire vascular complex. Photodynamic therapy seems to be a promising therapeutic modality, but randomized clinical trials are needed to establish the efficacy and safety of the PDT treatment in the management of these patients.