History of present illness
A 69-year-old White woman with no significant past medical history was referred by an outside ophthalmologist for evaluation and management of bilateral photopsias of 3 months’ duration. She reported mild blurring of her vision that had not resolved after obtaining new eyeglasses. She noted no floaters, pain, or photophobia and had no history of ocular surgery or procedures. She denied nyctalopia. Her family history was remarkable for an aunt with age-related macular degeneration.
Ocular examination findings
Visual acuity with correction was 20/25 in both eyes, pupils were reactive without afferent pupillary defect, but confrontation visual fields were constricted. Intraocular pressures were 9 in the right eye (OD) and 12 in the left eye (OS). There was no evidence of inflammation in the anterior segment of either eye, although trace nuclear sclerosis was noted in both eyes (OU). Dilated examination showed bilateral 2+ anterior and posterior vitreous cells and 2+ central vitreous haze. Fundus examination showed several small drusen along the arcades OU, few intermediate-sized drusen OU, and a large druse OD. The remainder of her examination, including that of optic discs, vasculature, macula, and periphery, was otherwise unremarkable ( Fig. 29.1 , color fundus photographs). No tears, holes, or breaks were identified in either eye.
Imaging
Fluorescein angiography (FA) showed bilateral late staining of the discs and staining with leakage of the retinal venules, which was greater in the right eye. Indocyanine green angiography (ICGA) was performed showing numerous scattered nummular hypocyanescent lesions nasal to the disc OU as well as within the periphery.
Optical coherence tomography (OCT) of each eye showed a few drusen and no cystoid macular edema (CME).
Questions to ask
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Does the patient have a history of systemic inflammatory disease, including sarcoidosis, or other systemic diseases, such as central nervous system lymphoma, that may be related to intraocular disease? Does the patient have a history of symptoms consistent with any of these diseases, including shortness of breath, cough, rashes, or joint pain and swelling?
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No
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Does the patient have family history of systemic inflammatory disease?
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No
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Does the patient have a history of systemic infection, including tuberculosis (TB) or syphilis? Does the patient have risks for exposure to these infections, including being from an endemic area (another country for TB), or high-risk sexual behavior? Does the patient have a history of symptoms consistent with these infections?
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No
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Assessment
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A 69-year-old woman with no past medical history presents with mild blurred vision and persistent photopsias. Clinical findings include vitreous haze with inflammation, FA with optic nerve staining and vascular leakage, and ICGA with numerous hypocyanescent lesions nasal to the disc OU and within the periphery.
Differential diagnosis
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Birdshot chorioretinopathy (BSCR)
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Ocular sarcoidosis
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TB choroiditis
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Primary vitreoretinal lymphoma
Working diagnosis
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Birdshot chorioretinopathy
Multimodal testing and results
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Fundus photographs
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Classically seen “birdshot lesions” of hypopigmented lesions surrounding and nasal to the optic disc were not seen in this patient. There was no evidence of retinal vasculitis on fundus examination.
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OCT
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This patient’s OCT showed no CME, a common complication of BSCR seen in 84% of patients.
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Fundus autofluorescence (FAF)
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FAF imaging obtained at patient presentation showed no abnormalities outside the hyperautofluorescence of the drusen in the right eye ( Figs. 29.2 and 29.3 ). FAF findings in other patients may include hypoautofluorescence of the birdshot lesions.
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