Author: Sara Mosquera Pedreiro (Spain)
Co-authors: Santiago López-Arbués, Esther Compains-Silva, Nerea Zubieta-Gonzalo, Sandra Pérez-García, Pablo González de los Mártires, Gonzalo Guerrero-Pérez
Purpose
To report a clinical case of Birdshot chorioretinopathy (BCR) in a middle-aged male whose primarily manifestation featured as bilateral branch retinal artery occlusion with emboli, acute hearing loss and silent lacunar infarction.
Setting/Venue
Department of Ophthalmology. Complejo Hospitalario de Navarra. Pamplona, Navarra, Spain. Tertiary Hospital.
Methods
A 56-year-old healthy man was referred for several acute episodes of blurred vision, nyctalopia, bilateral acute hearing loss and various scotomata in both eyes (BE) for the last two months. Slit lamp examination was normal except for embolism of peripheral arteries in BE. Perimetric examination discovered a severe reduction in peripheral vision in BE. Magnetic resonance imaging showed silent lacunar infarction, whilst carotideal and cardiac examination were performed, without significant findings. Eight months after the inicial symptoms, classical signs of BCR appeared, such as bilateral hypopigmented choroidal spots inferior and nasal to the disc, cystid macular oedema, vitreitis, retinal vascular leakage with vasculitis and HLA-A29 positivity. After a differential diagnoses with other infectious and autoinmune diseases that may resemble BCR, corticoid and inmunomodulation therapy was offered, which resulted in greater clinical improvement.
Results
BCR is a chronic inflammatory disease whose pathophysiology remains poorly understood. The autoinmune response may trigger inflamatory infiltration throughout the choroidal and retinal microarchitecture. Typical ocular criteria is well characterized whereas systemic signs and symptoms remain uncertain. It has been described a relation with systemic hypertension (23-46%), hearing loss, vitiligo and mood disorders in small series. Furthermore, pacients with multiple lacunar infarcts, as in this pacient, have been associated with systemic hypertension, smoking, and renal impairment that is also believed to reflect an inflammatory diffuse small vessel arteriopathy.
Conlusions
There is usually a challenge in identifying the condition prior to evolution of the characteristic symptoms and in some cases a delay in diagnose can lead to irreversible damages. Systemic association, such as hypertension is referred more frequent than in general population and might negatively influence visual prognosis, but further investigation is neccessary. Hearing loss is an uncommon feature described, that is thought to be probably caused by endolymphatic hydrops.
Financial Disclosure
There are no financial conflicts of interest to disclose
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