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Bilateral Central Retinal Vein OcclusionA Presenting Sign of an Ongoing Life-threatening Condition

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 47-year-old woman, with dyslipidemia, presented to the emergency department with sudden vision loss in the right eye. Visual acuity was 20/40 OD and 20/20 OS. Anterior segment, tonometry, and pupillary light reflex were unremarkable. Ophthalmoscopy of the right eye showed discretely blurred optic disc margins, tortuous and dilated retinal veins, and intraretinal hemorrhages in all quadrants; the left eye was unremarkable. An optical coherence tomography (OCT) scan showed a cystoid macular edema (CME) in the right eye; the left was unremarkable. Retinal nerve fiber layer thickness was 133 μm OD and 82 μm OS (reference value, 95.4 ± 10.48 μm).1 A fluorescein angiography was scheduled. One week later, the patient returned to our department due to multiple episodes of amaurosis fugax in left eye. By that time, ophthalmoscopy disclosed a mild optic disc swelling, tortuous venous vessels, and retinal hemorrhages bilaterally (Figure 1). The OCT scan showed a CME spontaneous resolution in the right eye while the left eye was unremarkable. Fluorescein angiography revealed a slight delay in venous filling but no capillary nonperfusion areas. These features were compatible with bilateral nonischemic central retinal vein occlusion (CRVO), with spontaneous reperfusion in the right eye.

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Occult tumor presenting with bilateral CRVO

B. Perform a thoracoabdominopelvic computed tomography scan

The fundus findings were consistent with bilateral CRVO. An extensive hypercoagulability and autoimmune workup producing normal results raised the suspicion of an underlying paraneoplastic syndrome. The patient was sent for an urgent thoracoabdominopelvic computed tomography scan that revealed a right paratracheal adenopathy conglomerate with a dimension of 39 × 28 × 62 mm (Figure 2) and a thrombosis of the middle suprahepatic veins (Budd-Chiari syndrome). Meanwhile, the patient promptly initiated anticoagulation treatment with enoxaparin 1 mg/kg every 12 hours. Endobronchial ultrasound–guided transbronchial needle aspiration biopsy and anatomopathological results were inconclusive. A positron emission tomography scan was performed. In addition to the mediastinal mass, a supraclavicular adenopathy with increased fluorine 18–labeled fluorodeoxyglucose uptake was detected. A biopsy of this adenopathy revealed a poorly differentiated squamous cell carcinoma. To identify the primary tumor, extensive workups by gastroenterology, otorhinolaryngology, gynecology, and dermatology were performed. Despite the efforts, the primary location of the tumor was not identified. As such, the patient was diagnosed with an occult tumor and chemotherapy was initiated with carboplatin and placlitaxel.

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Article Information

Corresponding Author: Carolina Madeira, MD, Department of Ophthalmology of São João Hospital, Avenida Professor Hernâni Monteiro, 4202-451 Porto, Portugal (tania.carolina.madeira@gmail.com).

Published Online: September 16, 2021. doi:10.1001/jamaophthalmol.2021.0993

Conflict of Interest Disclosures: Dr Barbosa Breda reported receiving personal fees from Allergan, Davi, and Thea. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

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Segev  F , Segev  A , Livne  A , Assia  EI , Mekori  YA .  Bilateral central retinal vein occlusion in a patient with occult colon cancer.   Arch Ophthalmol. 2001;119(10):1552-1553.PubMedGoogle Scholar
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