Familial bilateral polypoidal choroidal vasculopathy

Description of the clinical and angiographic manifestations of polypoidal choroidal vasculopathy (PCV) in two brothers of West Indian origin.

Case reports.

In Case 1 (82-year-old), the disease presented when the patient was 50 years old with decreased visual acuity in the right eye. Onset of symptoms in the left eye occurred 30 years later. Visual acuity was limited to good luminous orientation in both eyes. In Case 2 (78-year-old), the disease presented in the right eye when the patient was about 68 years old. Visual acuity in the right eye was reduced to good luminous orientation. Symptoms in the left eye appeared 10 years later. Visual acuity in the left eye was 20/100. In both patients, medical history was significant only for arterial hypertension. There was no intraocular inflammation or ocular hypertonicity. Indocyanine green angiography revealed hyperfluorescent polypoidal dilatations at early and intermediate phases, characteristic of PCV.

These observations argue in favor of a genetic component in PCV.