Atypical Presentations of Birdshot Chorioretiopathy: A Comparative Longitudinal Study

Abstract

Purpose : To describe the clinical and treatment characteristics of two cohorts of atypical presentations of Birdshot chorioretinopathy. We hypothesize that when compared to a normal bilateral disease cohort, there are identifiable differences in the complications and outcomes of these atypical groups.

Methods : Retrospective chart review of patients seen at the Emory Eye Center from 2002-2019 who had a recorded diagnosis of Birdshot chorioretinopathy (n=63).

Results : The identified patients were grouped into three groups based on their imaging patterns: 1) asymmetric disease, patients with disease either presenting unilaterally or with asymmetric lesions on imaging (Figure 1; n=7); 2) typical disease, patients with bilateral disease presentation with identifiable birdshot lesions both on fundus examination and on indocyanine green angiography (ICG; n=47); and 3) silent disease, patients with no cream colored spots identified on fundus examination, but with hypocyanscent spots identified on ICG (n=9). The mean age in all three groups was in the 5^th decade. 100% of the patients in the asymmetric group and silent group exhibited a complication of their disease, compared to 87% of the typical group. The most common disease complication in all groups was vasculitis (typical 72.3%, silent 77.8%, asymmetric 57.1%). The mean difference in logMAR visual acuity from the first recorded visit to the final was -0.02 logMAR for the typical group (better eye), -0.10 logMAR for the silent group (better eye), and 0.08 logMAR for the asymmetric group (affected eye). Systemic medications were most commonly used for disease treatment in the asymmetric group (85.8%). Local treatments (88.9%), or a combination of both local and systemic treatments (66.7%), were most commonly used in the silent group.

Conclusions : Although a symmetric, bilateral disease pattern is still the most common presentation of Birdshot chorioretinopathy, we describe here two notable atypical cohorts. These atypical groups had distinctive imaging findings but shared the same disease complications as typical patients. All groups required complex treatment strategies including both systemic immunosuppression and local steroid injections.

This is a 2020 ARVO Annual Meeting abstract.

 

Patient with Asymmetric Birdshot Chorioretinopathy. Figure 1A shows hypocyanscent lesions in the right eye, and Figure 1C displays corresponding cystoid macular edema in the right eye.

 

Table 1. Characteristics of Patient Cohorts