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Small lymphaticovenous malformation of the orbital apex clinicopathologic correlation.

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.

SUBMITTER: Hankins M

PROVIDER: S-EPMC6660553 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature

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Small lymphaticovenous malformation of the orbital apex clinicopathologic correlation.

Hankins Mark M Murtagh Ryan R Margo Curtis E CE Bajric Jasmina J Agazzi Siviero S Malafronte Patrick J PJ Drucker Mitch M

American journal of ophthalmology case reports 20190713

<h4>Purpose</h4>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.<h4>Observations</h4>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 fib ...[more]

PMID: 31372579

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