Retin Cases Brief Rep. 2020 Aug 18. doi: 10.1097/ICB.0000000000001042. Online ahead of print.
PURPOSE: To report a case of bilateral ocular ischemia caused by intravascular lymphoma with presence of bilateral Triangular Sign of Amalric.
OBSERVATIONS: A 67-year-old female was admitted to our hospital for progressive paraplegia and a 6-month history of acute painless vision loss OD. Initial exam showed vision of CF OD and 20/20 OS with normal anterior exam. Fundus exam revealed a likely previous CRAO OD with pale nerve and attenuated vessels. Both fundi had triangular regions of pigmentary change known as the Triangular Sign of Amalric, indicative of choroidal ischemia. However, the left eye was asymptomatic. Neuro-imaging revealed multifocal enhancing lesions throughout the central nervous system of unclear etiology. An extensive neurologic and systemic workup was unrevealing, including a brain biopsy, and empiric treatment for an unspecified inflammatory condition with IV corticosteroids was initiated. During her hospitalization, she developed acute painless vision loss OS, and exam showed NLP vision OU with signs of acute retinal and choroidal ischemia OS. A subsequent brain biopsy revealed intravascular lymphoma.
CONCLUSIONS: and Importance: Triangular pigmentary changes indicate choroidal ischemia, and can be seen in many conditions. This patient presented with the Triangular Sign of Amalric in both eyes, including her asymptomatic left eye. Intravascular lymphoma should be considered in cases of concomitant inflammatory brain lesions and chorio-retinal ischemia.