Diagnosis and Management of Acute Thrombosis in Venous Dominant Orbital Venolymphatic Malformations.

Purpose: To report the characteristic clinical and imaging findings in a series of patients with thrombosed orbital venolymphatic malformations.

Methods: Patients affected by acute thrombosis of an orbital venolymphatic malformation were reviewed. Clinical findings including symptoms and signs of presentation, characteristic imaging features, and clinical course are presented.

Results: Ten patients were identified. The mean age at presentation was 56 years. All patients presented with acute pain or pressure sensation, with the most common additional presenting symptoms being proptosis (6/10) and diplopia (5/10). CT imaging typically demonstrated a nonspecific orbital mass. Nine patients underwent MRI which revealed a soft tissue mass with peripheral rim enhancement and a central, typically T2 hypointense, core. Seven out of 10 patients were observed and had improvement in symptoms and signs without surgical intervention. Two patients underwent surgical intervention for intractable pain.

Conclusion: Patients with thrombosis of a venolymphatic malformation often present with acute pain, proptosis, and diplopia. Characteristic MRI findings of a peripheral rim enhancing mass with a T2 hypointense core can be noted. Careful observation is a reasonable management option for cases without visual compromise or intractable pain.