Ipsilateral Lymphatic and Venous Malformations Affecting the Midface Area

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.

Figure 1

(a) Facial appearance of the patient at presentation showing slight lateral displacement of the left eye. (b) Anterior segment photograph shows lymphatic malformation cysts in the inferonasal conjunctiva (arrow). (c) Lesion similar to lymphatic malformation affecting the left hard palate (arrow).

Figure 2

(a) Anterior segment swept source optical coherence tomography of the left eye demonstrates dilated lymphatic channels manifesting as hyporeflective spaces with different sizes demarcated by hyperreflective septae (arrows). (b) T1-weighted coronal MR image shows the inferonasally located intraconal tumor that is isointense with respect to muscle (arrow) in the left orbit. (c) T2-weighted axial MR image shows that the left orbital tumor is hyperintense (arrow).

Figure 3

(a) Gross photograph of the excised left orbital tumor showing the reddish well-circumscribed mass measuring approximately 1.5×1.5 cm in base dimensions. (b) Histopathological examination of the orbital tumor reveals enlarged vascular channels with thick walls, the lumens of which are filled with erythrocytes embedded in a fibrous stroma (arrows), consistent with orbital cavernous venous malformation (H.E. ×100). (c) Histopathological examination of the conjunctival lesion shows several ectatic bloodless lymphatic channels, lined with flattened endothelium (arrows). There is prominent lymphoid infiltration and aggregates in the stroma, consistent with the diagnosis of conjunctival lymphatic malformation (H.E. ×100).

Figure 4

Facial photograph of the patient 3 months after surgery shows excellent outcome with an orthophoric appearance.