Orbital venous-lymphatic malformations (lymphangiomas) mimicking cavernoushemangiomas

PURPOSE: To illustrate that orbital venous-lymphatic malformations (lymphan giomas) may rarely simulate cavernous hemangiomas, METHODS: Retrospective case review. RESULTS: Five patients were identified from a series of 85 patients with ve nous-lymphatic malformations. The age range was 21 to 69 years, and all cas es presented with a history of slowly progressive or long-standing proptosi s, Computerized tomography revealed relatively homogeneous intraconal masse s that were well defined anteriorly, Two of the cases had expansion of the orbit, and one had focal calcification. The three who had magnetic resonanc e imaging showed heterogeneous contrast enhancement. The preoperative diagn osis in every case was cavernous hemangioma, and intraoperatively the lesio ns resembled cavernous hemangiomas. However, posterior dissection was diffi cult in all patients because of dense adhesions and, in one case, led to a central retinal artery occlusion, The histology was characteristic of orbit al venous-lymphatic malformations in all five cases. CONCLUSIONS: Deep orbital venous-lymphatic malformations presenting in adul thood may be rarely confused with cavernous hemangiomas. In doubtful cases, significant intralesional heterogeneity, best seen on magnetic resonance i maging, and focal calcification may help distinguish the two entities. This differentiation is important, because dissection of venous-lymphatic malfo rmations is fraught with more complications than surgical excision of a cav ernous hemangioma, (Am J Ophthalmol 2001;131: 364-370. (C) 2001 by Elsevier Science Inc. All rights reserved.).