Orbital lymphangiomas are benign hamartomatous tumours usually diagnosed in early childhood. In contrast with the benign histology, these lesions have a locally aggressive nature. They may enlarge slowly, producing mild proptosis; nevertheless intrinsic haemorrhage can occur, producing a mass effect and leading to intractable, painful, progressive proptosis, and compressive optic neuropathy. A conservative approach is advisable for several reasons: complete resection is difficult because these lesions, unlike haemangiomas, are not encapsulated, do not respect anatomic boundaries, tend to penetrate into normal structures, and can bleed profusely during surgery. For these reasons, an early diagnosis is important. On this subject, ultrasonography appears to be the first-choice diagnostic test, because of its sensitivity and simplicity, particularly in childhood; therefore we recommend to always perform this test in the presence of orbital vascular lesion.
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