[Skip to Content]
[Skip to Content Landing]

Pigmented Paravenous Chorioretinal Atrophy With Macular Hole

To identify the key insights or developments described in this article
1 Credit CME

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.

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 Credit(s)™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

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
1.
Brown  TH .  Retinochoroiditis radiata.   Br J Ophthalmol. 1937;21(12):645-648. doi:10.1136/bjo.21.12.645PubMedGoogle ScholarCrossref
2.
Ghosh  B , Goel  N , Batta  S , Raina  UK .  SD-OCT in pigmented paravenous retinochoroidal atrophy.   Ophthalmic Surg Lasers Imaging. 2012;43(3):e41-e43.PubMedGoogle Scholar
Close
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close

Name Your Search

Save Search
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Close
Close

Lookup An Activity

or

My Saved Searches

You currently have no searches saved.

Close

My Saved Courses

You currently have no courses saved.

Close