Cerebral vasculitis presenting as acute posterior multifocal placoid pigment epitheliopathy in a 16-year-old male.

Purpose: To report on a case of the successful treatment of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) in a pediatric patient with a prior diagnosis of cerebral vasculitis. Observations: A 16-year-old male with a prior diagnosis of cerebral vasculitis presented without ocular complaints. Visual acuity was 20/20, and color vision remained normal. Fundus examination revealed yellowish-white placoid lesions and retinal pigmented epithelial changes involving the posterior pole. A work-up including a rapid plasma reagin test, complete cell blood count, comprehensive metabolic panel, and urinalysis was within normal limits. A head computed tomography angiography without contrast and a brain magnetic resonance imaging scan were compatible with acute and past episodes of ischemia. Ancillary testing was compatible with an assessment APMPPE. Immunosuppressive and monoclonal antibody therapy resulted in the improvement and remission without residual neurologic deficits and with a BCVA of 20/20. Conclusionand Importance: This case suggests that a diagnosis of cerebral vasculitis should prompt physicians to consider an ophthalmic evaluation that includes a dilated fundus exam, regardless of the presence or absence of ocular symptoms. Ophthalmic findings may affect the diagnostic processes, particularly concerning infectious and non-infectious etiologies, or potentially neoplastic diseases.

Optic Disc Edema Is an Under-Recognized Feature of Birdshot Chorioretinitis.

BACKGROUND: Optic disc edema is a feature of many ophthalmic and neurologic conditions. It remains an underappreciated feature of birdshot chorioretinitis (BSCR), leading to delay in diagnosis and treatment. The purpose of our study was to identify clinical features that are concomitant with optic disc edema and suggest a diagnosis of BSCR. METHODS: Retrospective multicenter case series of 29 patients who were referred to a neuro-ophthalmologist or uveitis specialist for evaluation of disc edema and were ultimately diagnosed with BSCR. RESULTS: Fifty-four eyes of 30 patients, from the practices of 15 uveitis specialists, met the eligibility criteria. In addition to disc edema, concomitant features in all patients included vitritis, chorioretinal lesions, and retinal vasculitis. Visual recovery to 20/40 or better occurred in 26 of 29 patients. Visual acuity remained 20/100 or worse in 2 patients previously diagnosed with idiopathic intracranial hypertension, 1 patient previously diagnosed with optic neuritis, and 1 patient for whom treatment was delayed for years, leading to optic disc atrophy. CONCLUSIONS: Optic disc edema is a presenting feature in some cases of BSCR. A diagnosis of BSCR should be considered when disc edema occurs with vitritis, chorioretinal inflammation, and retinal vasculitis. Patients should be referred to a uveitis specialist for treatment.