Ocular involvement in TEMPI syndrome

American Journal of Ophthalmology Case Reports

卷:26

Justin Chun¹  Satish Shanbhag²  Jo-Hsuan Wu³  T.Y. Alvin Liu⁴  Narine Viruni⁴ 

[1] Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, 21205, USA;

[2] Cancer Specialists of North Florida, Fleming Island, FL, 32003, USA;

[3] Shiley Eye Institute and Viterbi Family Department of Ophthalmology, University of California, San Diego, CA, 92093, USA;

[4] Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, 21287, USA;

关键词: TEMPI syndrome;    Macular edema;    Retinal ischemia;   

DOI  :  

来源: DOAJ

【 摘 要 】

Purpose: We report the first case of ocular involvement in TEMPI syndrome, a rare disease characterized by telangiectasias, elevated erythropoietin with erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intra-pulmonary shunting. Observations: A 64-year-old Caucasian man with history of TEMPI syndrome presented with subacute bilateral painless vision loss. Ocular examination showed chronic retinal ischemia with microvascular damage, which was likely associated with the chronic systemic hypoxemia, and spontaneous wax and wane of cystoid macular edema, presumedly related to the systemic bortezomib treatment. Conclusions and importance: Our case demonstrates that pathologic retinal vascular changes could be seen in association with TEMPI syndrome and suggests that a comprehensive ophthalmological examination may be beneficial for these patients.

【 授权许可】

Unknown