Abstract
A 22-year-old woman presented with progressive swelling of the nasal conjunctiva in the left eye. Anterior segment examination revealed a diffuse cystic appearance to the inferonasal bulbar conjunctiva and plica semilunaris. Anterior segment swept-source optical coherence tomography (OCT) revealed clear hyporeflective spaces demarcated by hyperreflective septae in the affected conjunctiva, consistent with the diagnosis of lymphatic malformation (LM). Magnetic resonance imaging revealed a well circumscribed intraconal mass located inferonasally in the left orbit. Systemic examination revealed a lesion similar to LM on the left hard palate. The left conjunctival mass was excised subtotally. Subsequently, a transconjunctival anterior orbitotomy was performed and the left orbital mass was completely removed intact. Histopathologically, the conjunctival mass was diagnosed as LM and the orbital mass as venous malformation (VM). This case represents a rare coexistence of histopathologically proven conjunctival LM and orbital VM as well as a presumed LM of the hard palate, all 3 lesions occurring in the ipsilateral midface area.
Highlights
Hemangiomas develop as a result of abnormal changes in angiogenesis that allow overproliferation of vascular tissues
The Orbital Society created a new classification of vascular lesions based on hemodynamic behavior
Capillary hemangiomas and venous malformation (VM) are considered as hamartomas by this classification
Summary
Hemangiomas develop as a result of abnormal changes in angiogenesis that allow overproliferation of vascular tissues. Many of the angiogenic markers including fibroblast growth factor (FGF), vascular endothelial growth factor (VEGF), E-selectin, and type IV collagenase are increased during the proliferative growth phase of hemangiomas [2]. It has been speculated that hemodynamic changes cause opening of new channels allowing for budding of the vascular channels into surrounding soft tissues. The formation of LMs may reflect a failure of lymph ducts to connect with the venous system during embryogenesis, abnormal sequestration of lymphatic structures, or both [3]. Many LMs are initially asymptomatic until an inciting factor causes an increase in the size of the lesion. Growth of the LMs is usually related to upper respiratory tract infections and minor trauma to the periocular region. We report an interesting case demonstrating ipsilateral coexistence of orbital VM, conjunctival LM, and hard palate LM
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