Geographical choroidopathy.

Atrophy of the retinal pigment epithelium and choriocapillaris takes many forms, and attempts have been made to identify distinct clinical entities within this group of diseases: central senile areolar choroidal sclerosis, peripapillary choroidal sclerosis, generalized choroidal sclerosis (Sorsby, I939), subacute multifocal placoid pigment epitheliopathy (Gass, I968), pigment epitheliitis (Krill and Deutmann, 1972), and serpiginous choroido- pathy (Gass, 1970).Franceschetti (I962) collected sixteen cases of circumscribed atrophy of the posterior pigment epithelium and choroid from his own practice and from the previous literature, and he termed the condition "helicoid peripapillary chorioretinal degeneration".Some patients included in this category had progressive disease (Kraffel, 1955; Maumenee, 1968), whilst in others the disease was static (Rubino, 1940; Sveinsson, I939); some patients were considered to have congenital defects (Rubino, 1940; Sveinsson, 1939) and others were shown to have infective disease (Brown, 1937; Hsin-Hsiang, 1948; Weve, I957).In those cases considered to be infective in origin, the lesions frequently corresponded to the retinal veins, and pigment migration into the retina was a feature.In cases of "degenerative" and "congenital" disease, it was noted that the lesions did not underly the retinal vessels and that there was no pigment migration into the retina.It is clear that helicoid degeneration, as described, is not a single disease entity, and that this term has been used to denote separate diseases with similar ophthalmoscopic features.It is the purpose of this paper to present the morphological characteristics and evolution of a disease formally included in the "helicoid" group of disorders, which has an acute onset, and in which the initial changes are seen at the level of the pigment epithelium.Fluorescein fundus angiography has been used as an aid to define the features of the disease. Material and methodsThe patients described in this paper were referred to the Retinal Unit from the ophthalmic practice in Moorfields Eye Hospital.Fluorescein angiography Five ml. of 20 per cent sodium fluorescein were injected into the antecubital vein, and photographs were taken at i-second intervals during the initial transit of dye through the ocular fundus with a Zeiss (Oberkochen) fundus camera using Ilford FP4 film.A Baird Atomic interference filter with an 80 per cent.transmission at 4,800 A and a broad transmission band of 400 A was used for excitation, and an Ilford I I0 gelatin filter as a barrier.Patient I, a 42-year-old female clerical assistant, presented in February, 1972, having noticed a paracentral defect in the visual field of the right eye i month previously.