TRIANGULAR SIGN OF AMALRIC IN INTRAVASCULAR LYMPHOMA

To report a case of bilateral ocular ischemia caused by intravascular lymphoma with the presence of bilateral triangular sign of Amalric.

A 67-year-old woman was admitted to our hospital for progressive paraplegia and a 6-month history of acute painless vision loss in the right eye. Initial examination showed vision of counting fingers in the right eye and 20/20 in the left eye with normal anterior examination. Fundus examination revealed a likely previous central retinal artery occlusion in the right eye 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. Neuroimaging 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 intravenous corticosteroids was initiated. During her hospitalization, she developed acute painless vision loss in the left eye, and examination showed no light perception vision in both eyes with signs of acute retinal and choroidal ischemia in the left eye. A subsequent brain biopsy revealed intravascular lymphoma.

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 chorioretinal ischemia.