First Author: J.Moreira PORTUGAL
Co Author(s): C. Teixeira R. Goncalves P. Coelho T. Maio F. Sampaio P. Tenedorio
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Choroidal dystrophies are a group of progressive, hereditary disorders that are characterized by loss of retinal pigment epithelium and atrophy of the choriocapillaris. Herein, we describe a patient with a posterior polar central choroidal dystrophy.
Department of Ophthalmology, Hospital Pedro Hispano, Porto, Portugal.
An 86-year-old female presented with a chief complaint of gradual vision loss over the past 4 decades. Her ocular history was insignificant and there was no family history of eye disease. Medical history was remarkable for hypertension controlled with medication. Upon examination her best corrected visual acuity was finger counting at 40 inches in right eye, and 4 inches in left eye. Slit lamp evaluation exhibited cortical cataracts in both eyes. There was no gross abnormality of the anterior segment. Fundus examination showed a bilateral extensive chorioretinal atrophy involving the posterior pole and extending outside the vascular arcades, and surrounding the optic nerve. There were no drusen, edema or flecks. Fundus autofluorescence imaging showed clearly defined hypofluorescent areas that corresponded to the aforementioned lesions. The electroretinogram was abnormal. Based on the fundus appearance, history and test results, a diagnosis of posterior polar central choroidal dystrophy was made. The patient was educated as to the hereditary pattern of this disease and referred for a low vision evaluation.
There are several forms of primary choroidal distrophies which affect the central macula. Posterior polar central choroidal dystrophy is a rare condition, that may start as a focal degenerative process in the central macula, but the area of atrophy may expand. At first, there may be patchy or zonal atrophy, followed by confluency as the entire process expands outside of the temporal vascular arcades and surround the optic disc.
Even though management options are limited to low vision devices, it is essential for the patient with posterior polar central choroidal dystrophy to be properly counseled regarding genetic issues inherent to their condition and other family members.