Idiopathic Polypoidal Choroidal Vasculopathy

Abstract

More than three decades ago, a peculiar hemorrhagic disorder of the macula, polypoidal choroidal vasculopathy (PCV), was first described (see Yannuzzi LA’s “Idiopathic polypoidal choroidal vasculopathy” presented at the annual meeting of the Macula Society, Miami, FL, 1982). This entity has also been designated by Kleiner and associates as “posterior uveal bleeding syndrome” [1, 2] and by Stern and co-workers as “multiple recurrent retinal pigment epithelium (RPE) detachments in black women.” [3] In 1990, Yannuzzi and colleagues suggested the term idiopathic polypoidal choroidal vasculopathy (IPCV) because the pathogenesis was unknown, the primary abnormality involved the choroidal circulation, and the characteristic lesion was 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 [4]. Given its appearance and location between RPE and inner collagenous layer of Bruch’s membrane, the alternative classification “type 1 aneurysmal neovascularization” has also been proposed [5]. However, emerging evidence suggests that many lesions thought to be aneurysmal may instead appear as tangles of vessels upon use of advanced imaging techniques [6]. Regardless, this entity has characteristic morphological features that distinguish it from other exudative maculopathies. Clinically, it was associated with multiple, recurrent, serosanguineous detachments of the RPE and neurosensory retina secondary to leakage and bleeding from the peculiar choroidal vascular lesion (Fig. 1).