Orbital lymphaticovenous malformations: Current and future treatments

Abstract

Orbital lymphaticovenous malformations consist of abnormal vascular channels lined by endothelial cells with a spectrum from venous to lymphatic characteristics. They may be venous-dominant or lymphatic-dominant. These lesions continue to present management challenges. Total excision or obliteration is not always achievable, recrudescence is common, and interventions carry a risk of damaging normal structures. Patients likely benefit most from a multidisciplinary approach, including both surgical and nonsurgical (e.g., sclerosants and liquid polymers) therapeutic modalities. Targeted biologic therapy would be ideal; nevertheless, this goal is complicated by the heterogeneous venous-lymphatic and stromal characteristics of these lesions. Ideally, antiangiogenic agents targeting both lymphatic and blood vascular endothelial cells will be developed to treat these lesions and reduce their regrowth. Further studies are warranted to enhance our understanding of these orbital lesions with regard to their angiogenic (proliferative) activities and profiles of marker expression, with a goal to produce effective medical therapies.

Keywords: anti-lymphangiogenesis; lymphangiogenesis; lymphangiomas; lymphatic system development; orbital lymphangiomas; orbital lymphatic-venous malformation; orbital vascular malformation; vascular malformation.