Ocular involvement in TEMPI syndrome

Jo-Hsuan Wu; Narine Viruni; Justin Chun; Satish Shanbhag; T Y Alvin Liu

doi:10.1016/j.ajoc.2022.101534

Ocular involvement in TEMPI syndrome

Am J Ophthalmol Case Rep. 2022 Apr 10:26:101534. doi: 10.1016/j.ajoc.2022.101534. eCollection 2022 Jun.

Authors

Jo-Hsuan Wu¹, Narine Viruni², Justin Chun³, Satish Shanbhag⁴, T Y Alvin Liu²

Affiliations

¹ Shiley Eye Institute and Viterbi Family Department of Ophthalmology, University of California, San Diego, CA, 92093, USA.
² Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, 21287, USA.
³ Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, 21205, USA.
⁴ Cancer Specialists of North Florida, Fleming Island, FL, 32003, USA.

Abstract

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.

Keywords: Macular edema; Retinal ischemia; TEMPI syndrome.

Publication types

Case Reports