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A Chinese woman had painless, gradually declined vision for 2 years without redness. She had a medical history of vomiting and emaciation in her early 40s. Pars plana vitrectomy (PPV) had been done for her left eye owing to vitreous opacity half a year prior. She claimed that her younger brother died of liver cancer and her mother had lost vision because of vitreous hemorrhage and glaucoma. She denied any history of systemic disorders.
On initial examination, best-corrected visual acuity was measured at 0.1 (20/200) OU. Intraocular pressure was 14 mm Hg OD and 16 mm Hg OS. Her anterior segment examination results were within normal limits. Dilated fundus examination demonstrated bilateral vitreous opacity without cells (Figure). The peripheral residual vitreous cortex in the left eye was as cloudy as the cortex in the right eye. Fundus was not visible in the right eye and was normal in the left eye. A bilateral ocular ultrasonography showed normal results except for vitreous opacity. The patient weighed 37.0 kg and was 155 cm tall. Her blood pressure was 113/70 mm Hg. Mild anemia (hemoglobin level 10.5 g/dL vs a normal range of 11.0-15.0 g/dL [to convert to grams per liter, multiply by 10]) was found in routine blood test results. Liver and renal functions were normal according to serum test results. The results of an electronic cardiogram were negative.
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Vitreous amyloidosis owing to familial amyloid polyneuropathy
B. PPV plus pathological examination plus genetic test
The differential diagnosis of vitreous opacity includes inflammatory, infectious, hemorrhagic, and degenerative diseases. Vitreous cells usually indicate intraocular inflammation, with the opacity often severe in the inferior part of the vitreous. However, the vitreous of this patient was a uniform glassy, woollike appearance. The patient had no signs of inflammation. Additionally, the dehemoglobinized vitreous hemorrhage is similarly white. However, red blood cell ghosts could be seen in the vitreous, usually accompanied by sudden vision loss. Furthermore, primary intraocular lymphoma should be differentiated because primary intraocular lymphoma could also lead to vitreous opacity. However, primary intraocular lymphoma typical leopard spot–like subretinal lesion and vitreous cells were not seen in this case. Therefore, option A is inappropriate.
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Corresponding Author: Xiaoling Liang, MD, PhD, State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, 7 Jinsui Rd, Guangzhou, 510623, China (firstname.lastname@example.org).
Published Online: October 17, 2019. doi:10.1001/jamaophthalmol.2019.4098
Conflict of Interest Disclosures: None reported.
Funding/Support: The Chinese government provided financial support in the form of the National Natural Science Foundation of China (No. 81870668).
Role of the Funder/Sponsor: The funding source had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
Additional Contributions: We thank the patient for granting permission to publish this information.
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