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Retinal Disorders

Pigmented Paravenous Chorioretinal Atrophy With Macular Hole

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JAMA Ophthalmology

Published Online: August 18, 2022

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Article Information and Disclosures

Wu Xiang, PhD^1,2;

Yantao Wei, PhD^1,2

Author Affiliations

¹State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, China
²Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangdong Provincial Clinical Research Center for Ocular Diseases, Guangzhou, China

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A 56-year-old woman presented with decreased visual acuity in the right eye. The corrected visual acuity was 20/100 OU. On examination, both eyes revealed mild cataracts, perivenous bone-spicule pigmentation, and chorioretinal atrophy in the peripapillary and perivenous areas (Figure). Optical coherence tomography showed a macular hole in the right eye (Figure, A) and severe atrophied retinal nerve fiber layer (RNFL) in both eyes. Electroretinography revealed decreased photopic and scotopic responses, and fluorescein angiography showed diffuse window defects and blocked fluorescence. These findings indicated a diagnosis of pigmented paravenous chorioretinal atrophy (PPCRA) in both eyes combined with macular hole in the right eye. PPCRA was first reported in 1937.¹ Visual function is usually good in these patients unless the macula is involved. The visual impairment in this patient can be attributed to severe atrophied RNFL. To our knowledge, lamellar,² but not full-thickness, macular holes have been reported in PPCRA. The patient agreed to undergo a pars plana vitrectomy, after which the macular hole closed. The patient’s final best-corrected visual acuity was approximately 20/67 OD.

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Article Information

Corresponding Author: Yantao Wei, State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, 7 Jinsui Road, Guangzhou 510623, China (weiyantao75@126.com).

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References

Brown TH . Retinochoroiditis radiata. Br J Ophthalmol. 1937;21(12):645-648. doi:10.1136/bjo.21.12.645 PubMed Google Scholar Crossref

Ghosh B , Goel N , Batta S , Raina UK . SD-OCT in pigmented paravenous retinochoroidal atrophy. Ophthalmic Surg Lasers Imaging. 2012;43(3):e41-e43.PubMed Google Scholar

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.