Birdshot chorioretinopathy was first characterized by Ryan and Maumenee1 as a chronic intraocular inflammatory condition with discrete, depigmented spots scattered throughout the fundus; mild vitritis; and vasculitis. A very strong association of this condition with the HLA-A29 gene and electrophysiologic abnormalities have since been described.2,3
In most cases, the patient's initial symptoms are blurred vision, floaters, and/or photopsia and ocular signs consistent with birdshot chorioretinopathy.1,4,5 A few reports have described the appearance of the classic fundal spots long after the onset of symptoms, inflammation, and vasculitis, but these seem to be in the minority.6 We are unaware of any report of the appearance of the classic fundal lesions of birdshot chorioretinopathy prior to the onset of symptoms. Herein we describe such a case.
A 35-year-old white man was first seen with a 3-week history of bilateral floaters. There were no associated or preceding ocular or systemic symptoms. His history included moderate myopia and blunt left ocular trauma in early childhood, after which a small scar at the posterior pole associated with decreased visual acuity was noted. Follow-up after this trauma revealed no change in either the scar or visual acuity.
Initial examination findings revealed a best-corrected visual acuity of 6/5 OD and 6/12 OS. Neither eye had dilated conjunctival vessels. Anterior segment examination revealed a trace of cells bilaterally, very few fine inferior keratitic precipitates, and no granulomatous changes. A mild to moderate vitritis was noted in both eyes. Fundal examination (Figure 1) revealed large areas of pale, flat depigmented lesions typical of birdshot chorioretinopathy, associated with a few isolated areas of retinal vasculitis and intraretinal hemorrhages bilaterally. An old atrophic scar was noted in the left macula, with the right macula appearing normal. There were no vitreal snow balls, snow banking, punched-out chorioretinal scars, nor any other complications.
Fundal photographs taken at initial examination showing typical birdshot choroidal lesions (black arrows) in both eyes (A-C) and an old macular scar in the left eye (asterisk)(B). C, Areas of peripheral vasculitis (white arrow) are shown.
Investigations included a full blood examination and film, calcium levels, syphilis serology, angiotensin-converting enzyme assay, chest radiography, and HLA-A29 typing. All results were normal except for the HLA-A29 typing, which was positive. Electrophysiology revealed delayed scotopic blue and photopic red b wave amplitudes. Fluorescein angiography revealed areas of vasculitis and typical late staining of the birdshot lesions.
Six months prior to the onset of symptoms, the patient, who works in an eye research institution, volunteered to have his fundi photographed for staff training purposes. Review of these photographs revealed his preexisting left macular scar and the presence of the typical depigmented birdshot choroidopathy lesions, retinal hemorrhages, and patchy retinal vasculitis (Figure 2).
Fundal photographs taken 6 months prior to initial examination showing an old macular scar (asterisk) in the left eye (A) and choroidal birdshot lesions (black arrows) in both eyes (A and B). A superior area of vasculitis (white filled arrow) and venous sheathing (white outline arrow) in the right eye can be seen (B).
Typically, the multiple depigmented lesions of birdshot chorioretinopathy are seen at the same time as the patient's initial examination for onset of symptoms.1,4,5 This case appears to be a typical manifestation of birdshot chorioretinopathy. It was purely coincidental that fundus photographs were taken 6 months prior to the onset of symptoms. It is quite possible that the fundal lesions of birdshot chorioretinopathy precede the onset of symptoms by some months but remain undetected in an asymptomatic subject who has no reason to be seen by an ophthalmologist.
The pathogenesis of birdshot chorioretinopathy remains unknown but there are several theories.1,5,6 Some place the disease focus in the choroid, which is supported by indocyanine green angiography findings.7 However, fluorescein angiography and electrodiagnostic findings implicate inner retinal dysfunction secondary to retinal vasculitis.1,3,7 Our patient is interesting in that he shows that both the retinal vasculitis and choroidal lesions can be present very early in the disease process.
Correspondence: Dr Guymer, Macular Research Unit, Centre for Eye Research Australia, 32 Gisborne St, East Melbourne, Victoria 3002, Australia (firstname.lastname@example.org).
Financial Disclosure: None reported.
Thank you for submitting a comment on this article. It will be reviewed by JAMA Ophthalmology editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 3
Customize your page view by dragging & repositioning the boxes below.
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.