Case of the Month | May 2022

Case of the Month
May 25, 2022

The Case

The patient was an 18-year-old man with acute loss of vision in the left eye two days previously. His past ophthalmic history had been unremarkable. His past medical history was remarkable for prior obesity that resolved due to a 100-pound weight loss with diet and exercise.

He denied other medical problems, including arthritis, breathing difficulties, and ulcers. He denied sleep apnea, dehydration, intravenous drug use, or any oral supplements.

The visual acuity was 20/25 OD and 20/300 OS with an inferior scotoma in the left eye. The anterior segment examination was unremarkable in both eyes. The vitreous was quiet in both eyes. The right fundus was normal. In the left eye, there was retinal whitening superotemporally, a few blot hemorrhages, and dilation and tortuosity of veins.

What is the most likely diagnoses? What treatment, if any, would you recommend?

The patient had dilated and tortuous veins and several intraretinal hemorrhages, consistent with a central retinal vein occlusion (CRVO). There was also superotemporal whitening inner edema found on OCT, consistent with a branch retinal artery occlusion. The fluorescein angiogram showed early superotemporal arterial filling followed by more complete filling 13 seconds later. The paradox of a superotemporal artery occlusion in a patient with early superotemporal arterial filling on fluorescein angiography can be explained by a cilioretinal artery occlusion that has recanalized. In fact, clinical examination confirmed the presence of a superotemporal cilioretinal artery. The mechanism of this patient’s condition was likely a central retinal vein occlusion causing swelling of the optic nerve, which compressed a cilioretinal artery. As the CRVO spontaneously resolved, the disc swelling diminished and the cilioretinal artery reopened. Unfortunately, there had been permanent visual loss, and the final visual acuity was 20/300 in the left eye. OCT 2 weeks later showed significant reduction of the edema of the inner retinal layers.

After presenting to our practice, we arranged for an emergent stroke work-up. MRI of the brain was unremarkable. CT angiography with contrast of the head did not reveal any stenotic lesions, and CT angiography with contrast of the neck was unremarkable. Trans-esophageal echography was remarkable for mild atheromatous disease of the descending thoracic aorta with mildly increased intimal thickness.  

The following tests were normal and/or unremarkable: CBC, lipid panel, PT, PTT, thrombin time, Factor X, protein S free Ag, Factor VIII:C, diluted Russell viper venom, TTI inhibition, anticardiolipin Ab IgG and IgM, antithrombin III activity, protein C clot activity, beta 2 glycoprotein IgG and IgM, homocysteine level, G20210A variant of prothrombin/factor II gene, and Factor V Leiden mutation. Central vein occlusions in young adults is uncommon, and it is appropriate to evaluate for a hypercoagulable state, blood dyscrasias, and other conditions that predispose to thrombosis. Often, no clear cause is found when there is a CRVO in a young adult patient.

The patient was prescribed a daily baby aspirin and has been stable subsequent to the initial event.

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The patient had dilated and tortuous veins and several intraretinal hemorrhages, consistent with a central retinal vein occlusion (CRVO). There was also superotemporal whitening inner edema found on OCT, consistent with a branch retinal artery occlusion. The fluorescein angiogram showed early superotemporal arterial filling followed by more complete filling 13 seconds later. The paradox of a superotemporal artery occlusion in a patient with early superotemporal arterial filling on fluorescein angiography can be explained by a cilioretinal artery occlusion that has recanalized. In fact, clinical examination confirmed the presence of a superotemporal cilioretinal artery. The mechanism of this patient’s condition was likely a central retinal vein occlusion causing swelling of the optic nerve, which compressed a cilioretinal artery. As the CRVO spontaneously resolved, the disc swelling diminished and the cilioretinal artery reopened. Unfortunately, there had been permanent visual loss, and the final visual acuity was 20/300 in the left eye. OCT 2 weeks later showed significant reduction of the edema of the inner retinal layers.

After presenting to our practice, we arranged for an emergent stroke work-up. MRI of the brain was unremarkable. CT angiography with contrast of the head did not reveal any stenotic lesions, and CT angiography with contrast of the neck was unremarkable. Trans-esophageal echography was remarkable for mild atheromatous disease of the descending thoracic aorta with mildly increased intimal thickness.  

The following tests were normal and/or unremarkable: CBC, lipid panel, PT, PTT, thrombin time, Factor X, protein S free Ag, Factor VIII:C, diluted Russell viper venom, TTI inhibition, anticardiolipin Ab IgG and IgM, antithrombin III activity, protein C clot activity, beta 2 glycoprotein IgG and IgM, homocysteine level, G20210A variant of prothrombin/factor II gene, and Factor V Leiden mutation. Central vein occlusions in young adults is uncommon, and it is appropriate to evaluate for a hypercoagulable state, blood dyscrasias, and other conditions that predispose to thrombosis. Often, no clear cause is found when there is a CRVO in a young adult patient.

The patient was prescribed a daily baby aspirin and has been stable subsequent to the initial event.

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