At. Murray et Gr. Kirkby, Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight, EYE, 14, 2000, pp. 711-716
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.