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...

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Main Authors: Risako Sone, Kousuke Noda, Kiriko Hirooka, Michiyuki Saito, Susumu Ishida
Format: Article
Language:English
Published: Elsevier 2022-12-01
Series:American Journal of Ophthalmology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2451993622004546
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author Risako Sone
Kousuke Noda
Kiriko Hirooka
Michiyuki Saito
Susumu Ishida
author_facet Risako Sone
Kousuke Noda
Kiriko Hirooka
Michiyuki Saito
Susumu Ishida
author_sort Risako Sone
collection DOAJ
description 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.
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spelling doaj.art-81246ca25e4d461386c3f590330ed15f2022-12-22T04:36:20ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362022-12-0128101708Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophyRisako Sone0Kousuke Noda1Kiriko Hirooka2Michiyuki Saito3Susumu Ishida4Department of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N-15, W-7, Kita-ku, Sapporo, 060-8638, JapanCorresponding author. N-15, W-7, Kita-ku, Sapporo, 060-8638, Japan.; Department of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N-15, W-7, Kita-ku, Sapporo, 060-8638, JapanDepartment of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N-15, W-7, Kita-ku, Sapporo, 060-8638, JapanDepartment of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N-15, W-7, Kita-ku, Sapporo, 060-8638, JapanDepartment of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N-15, W-7, Kita-ku, Sapporo, 060-8638, JapanPurpose: 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.http://www.sciencedirect.com/science/article/pii/S2451993622004546Posterior polar annular choroidal dystrophyCase report
spellingShingle Risako Sone
Kousuke Noda
Kiriko Hirooka
Michiyuki Saito
Susumu Ishida
Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
American Journal of Ophthalmology Case Reports
Posterior polar annular choroidal dystrophy
Case report
title Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
title_full Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
title_fullStr Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
title_full_unstemmed Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
title_short Insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
title_sort insidious progression of atrophic lesions in a case of posterior polar annular choroidal dystrophy
topic Posterior polar annular choroidal dystrophy
Case report
url http://www.sciencedirect.com/science/article/pii/S2451993622004546
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