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Am J Ophthalmol Case Rep. 2019 Jul 13;15:100517. doi: 10.1016/j.ajoc.2019.100517. eCollection 2019 Sep.

Small lymphaticovenous malformation of the orbital apex clinicopathologic correlation.

Author information

Departments of Ophthalmology, the University of South Florida, USA.
Departments of Radiology, the University of South Florida, USA.
Departments of Pathology and Cell Biology, the University of South Florida, USA.
Neurosurgery at the Morsani College of Medicine, the University of South Florida, the University of South Florida, USA.
Department of Pathology, Tampa General Hospital, Tampa, FL, USA.



To familiarize clinicians with the clinical and magnetic resonance imaging (MRI) features of a small orbital apex lymphaticovenous malformation that resulted in blindness and evaded timely clinical diagnosis.


A 68-year-old man presented with severe vision loss due to a 9 mm mass at the apex of the orbit above the optic nerve. When surgically removed 4 years later, the lesion was characterized by vascular spaces of varying size. Larger ones were filled with fibrin and organized thrombi. Stromal septa of endothelial-lined cavernous spaces were partially necrotic and there was evidence of remote hemorrhage. Some endothelial cells expressed D2-40, a marker of lymphatic channels.

Conclusions and importance:

Unless a high index of suspicion is maintained for a lymphaticovenous malformation the clinical diagnosis of a small but vision-threatening lesion can be overlooked.

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