The role of thrombosis as a mechanism of exacerbation in venous and combined venous lymphatic vascular malformations of the orbit.

PURPOSE: To describe venous thrombosis as a mechanism of clinical change in venous and combined venous lymphatic malformations of the orbit and to attempt histopathologically to distinguish the various vascular components of these lesions using immunohistochemistry with CD31 and D2-40 antibodies.
DESIGN: Retrospective, comparative, interventional case series. PARTICIPANTS: Twelve patients with clinically and radiologically well-documented episodes of thrombosis in venous malformations (n = 7; group A) and combined venous lymphatic malformations (n = 5; group B). INTERVENTION: Surgical excision of lesion in selected patients, 2 from group A and 5 from group B. MAIN OUTCOME MEASURES: Age at presentation, gender, onset, symptoms and signs, investigative findings (imaging and histopathologic review), management, and outcome.
RESULTS: In group A, 4 patients were male and 3 were female, and in group B, 4 patients were female and 1 was male. The mean age+/-standard deviation at presentation was 57.6+/-10.9 years (range, 45-71 years) and 11+/-11.6 years (range, 1.5-26 years), respectively. The pattern of onset was acute in all cases. The most common signs and symptoms in group A were pain (n = 7), proptosis (n = 6), and nausea (n = 5), whereas in group B they were periorbital swelling (n = 5), proptosis (n = 5), and ecchymosis (n = 4). The immunohistochemistry results were positive for vascular (CD31) and lymphatic (D2-40) endothelium in all of the specimens. The combined venous lymphatic lesions divided themselves into 2 main categories based on the D2-40 findings in relationship to percentage of lymphatic vessels. These were lesions that were either lymphatic dominant (n = 3) or venous dominant (n = 2).
CONCLUSIONS: Clinically, the 2 groups behave differently. Group A lesions present in adults with acute pain, proptosis, and nausea and may resolve spontaneously on follow-up. Intervention may be required in cases of severe pain, proptosis, or dysfunction. Group B lesions present early in life with frequent bouts of periorbital swelling, progressive proptosis, and ecchymosis. Therefore, early intervention is advised. Finally, when requesting imaging for such lesions, early- and late-phase contrast imaging should be used because thrombosis typically is better demonstrated in the late phase. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.