Orbital lymphaticovenous malformations: Current and future treatments

Orbital lymphatic-venous malformation with concomitant spontaneous orbital arteriovenous fistula: case report

“…Histologically, orbital LVMs have multiple dilated sacs and lobules lined with a single layer of endothelium, containing a proteinaceous or serosanguinous fluid with connective tissue stroma, smooth muscle bundles, dense small lymphocytes or follicles, and stromal hemorrhage. 8 Regarding the correlation between orbital LVMs and other vascular malformations, there is a significant association between diffuse orbital LVMs and noncontiguous intracranial vascular anomalies, including developmental venous anomalies, dural arteriovenous malformations, occlusion or absence of dural sinuses and jugular veins, cerebral cavernous malformations, and pial arteriovenous malformations. 1 Coll et al reported a case of concomitant medial extraconal orbital lymphangioma and ipsilateral arteriovenous malformation of the orbit and middle cranial fossa.…”

“…The nonsurgical strategies previously reported for orbital LVMs are sclerosing therapy including ethanol, OK-432, sodium tetradecyl sulfate (Sotradecol, Mylan), morrhuate sodium, doxycycline, bleomycin, and pinyangmycin. 8,11,12 A carbon dioxide laser, beta irradiation, and adjunctive systemic corticosteroids are also options for treatment. 8 Recently, the use of oral sildenafil for the treatment of microcystic lesions has been reported.…”

Orbital Lymphangioma-Case Report and Review of the literature

“…It is therefore more appropriate to use the term recrudescence instead of recurrence. The recrudescence rate of these lesions in other parts of the body can be as high as 40% after incomplete excision and 17% after macroscopically complete excision; however, the size of lymphaticovenous malformations after recrudescence is usually smaller than the initial lesion 5 .…”

“…As the orbit does not normally have lymphatics, therefore is theorized that orbital lymphatico-venous malformations are originated fromprimary orbital varices or from lymphatic or venous systems of the periorbital structures 5 .…”

“…As noted, there are short comings in the published studies describing different treatments for orbital lymphaticovenous malformations. Inconsistent data, incomplete hemodynamic classification or description, small sample sizes, and lack of within-study comparisons with a control group and other treatments limit our ability to develop evidencebased treatment guidelines for these lesions 5 .…”