Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disorder of unknown origin characterised by bone corpuscle pigmentation accumulation al ong the distribution of the retinal veins. In addition there are peripapill ary pigmentary changes as well as areas of chorioretinal atrophy adjacent t o the perivenular pigmentary changes. The finding of PPRCA is usually incid ental and does not affect vision. The literature regarding this condition i s reviewed. Its natural course has been considered controversial. Observati on of the initial insult leading to PPRCA has not previously been reported. A case that provides a photographic record over 20 years of the developmen t of this condition from the initial insult, including the gradual developm ent of the characteristic fundus appearance, is described. The initial pres entation was with a sudden reduction of vision and gross diffuse macular oe dema in one eye, which was rapidly followed by similar involvement of the f ellow eye despite treatment. Thus it was possible to examine the patient at the stage of the initial insult, 5 years before the development of the typ ical and pathognomonic retinal changes of PPRCA. The clinical and electroph ysiological findings are discussed, as also is the relevance of this case t o the literature reviewed.