Orbital Venous Lymphatic Malformation
A 2-year-old previously healthy female presented with 2 days of sudden onset left lid swelling immediately following an episode of vomiting. She was found on exam to have ptosis, mild proptosis, and left upper lid edema, induration, and fullness. MRI of the orbits showed a left intraconal mass that was slightly hyperintense relative to rectus muscles on T1 and hypointense with hyperintense rim and peripheral enhancement on T2. Given atypical imaging characteristics, further evaluation was undertaken with CT of the orbits which showed a hyperdense mass with density suggestive of blood. Given clinical presentation, spontaneous improvement during time admitted for workup, and upon multidisciplinary discussion, diagnosis of venous lymphatic malformation with intralesional hemorrhage was made.
Orbital venous lymphatic malformations are vascular malformations composed of dilated lymphatic channels lined by endothelial cells which commonly present in infancy or childhood with proptosis, ptosis, and ophthalmoplegia. They typically develop slowly over time but can occasionally cause acute symptoms during intralesional hemorrhage as a result of trauma, upper respiratory infection, or Valsalva. Imaging findings are not always typical and multiple imaging modalities are necessary for diagnosis. Lesions that are small, asymptomatic, and not affecting vision can often be managed with observation. There are several surgical and non-surgical management options, but treatment is often difficult and complicated by recurrence and unsatisfactory outcome.
Presentation Date: 12/19/2019
Issue Date: 08/01/2020
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