Combined venous lymphatic malformations of the orbit (so-called lymphangiomas)

Pediatric Orbit Tumors and Tumorlike Lesions: Nonosseous Lesions of the Extraocular Orbit

“…They found, as confirmed by others (31,34), that superficial lesions (and the superficial components of combined lesions) contain lymphatic components at pathologic evaluation. In contrast, deep lesions, as well as the deep component of combined lesions, are predominantly or completely venous in nature, reflecting the vessel distribution of the normal orbit (26,34).…”

“…Those lesions that involve the superficial or anterior orbital structures are diagnosed earlier (36). Frequently, anterior lesions extend to the forehead, temporal region, and cheek (31,32,35). Most patients with deep lesions present with acute proptosis, which develops due to intralesional hemorrhage or, less commonly, due to acute enlargement accompanying an upper respiratory tract infection.…”

“…When questioned, parents often recount a prior history from birth of eyelid fullness or purple discoloration of the skin, usually in the superomedial orbit (34). Vesicles may be noted in the conjunctiva, facial skin, or oral mucosa (31,32,36). Less commonly, patients present with lesions that demonstrate a more gradual growth with orbital expansion.…”

Late ocular manifestation of a childhood venous-lymphatic malformation

“…They are thought to represent a spectrum of malformations influenced by their occurrence during embryonic vasculogenesis. 1 The Orbital Society 2 has proposed the use of the term 'orbital vascular malformations' with a functional classification, which emphasises haemodynamic relationships, more pertinent to management.…”

“…Intervention is advised only in cases of compressive optic neuropathy, strabismus, or cosmetic deformity. 1,3 MRI shows irregular, multi-loculated, heterogeneous masses that are not confined to any fascial planes. 4 Phleboliths occur in one-third of cases.…”