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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

1
Departments of Ophthalmology, the University of South Florida, USA.
2
Departments of Radiology, the University of South Florida, USA.
3
Departments of Pathology and Cell Biology, the University of South Florida, USA.
4
Neurosurgery at the Morsani College of Medicine, the University of South Florida, the University of South Florida, USA.
5
Department of Pathology, Tampa General Hospital, Tampa, FL, USA.

Abstract

Purpose:

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.

Observations:

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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