The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy (IPCV)

Abstract

A peculiar hemorrhagic disorder of the macula, idiopathic polypoidal choroidal vasculopathy (IPCV) was first described, more than a decade ago¹. The maculopathy was classified and designated by Kleiner et al. as posterior uveal bleeding syndrome^2,3 and by Stern et al. as multiple recurrent retinal pigment epithelial detachments in black women^4,5. Yannuzzi et al. had suggested the term 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, spheroidal, polypoid-like structure^1,6,7. The disorder was characterized as a distinct clinical entity which was associated with multiple, recurrent, serosanguineous detachments of the retinal pigment epithelium and neurosensory retina, secondary to leakage and bleeding from the peculiar choroidal vascular abnormality. Vitreous haemorrhage, relatively minimal fibrous scarring and the absence of drusen, retinal vascular disease, and signs of intraocular inflammation were also features of the maculopathy.