Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy

PURPOSE: To describe the clinical course of a case of posterior polar annular choroidal dystrophy (PPACD) followed for 5 years. OBSERVATIONS: A 64-year-old female patient presented with blurred vision. The patient had no subjective symptoms of night blindness or visual field defects. At the initial visit, the patient's visual acuity was 20/20 in both eyes. Bilateral fundus examination revealed atrophic lesions surrounding the optic nerve head, extending to the temporal arcades in an annular pattern. Fundus autofluorescence (FAF) revealed hypoautofluorescent areas corresponding to atrophic lesions, and Goldmann perimetry revealed ring scotomas consistent with lesions in the fundus. Swept-source optical coherence tomography revealed retinal pigment epithelium atrophy, loss of the choriocapillaris, and dilation of the choroidal medium and large vessels in the atrophic area. Full-field electroretinography revealed a mild reduction in the combined rod–cone response. Laser speckle flowgraphy revealed a cold color in the posterior pole of both eyes. Based on clinical and imaging findings, the patient was diagnosed with PPACD and followed up for 5 years. At the 5-year visit, visual acuity remained unchanged, while FAF and Goldmann perimetry revealed a slight enlargement of the atrophic lesions and scotoma in both eyes, respectively. CONCLUSIONS AND IMPORTANCE: In the present case, atrophic lesions insidiously progressed and resulted in a slight enlargement of the hypoautofluorescent area and scotoma over a 5-year follow-up period, indicating that PPACD is a gradually progressive dystrophy.