A Case of Adult Orbital Intraconal Lymphangioma

Orbital lymphangioma is an intra-orbital, nonencapsulated, congenital vascular tumor with a propensity for recurrent hemorrhage. It is a common vascular tumor in children below 10 years of age. Adult orbital intraconal lymphangiomas are very rare. The authors present a case of 68-year-old male patient presented with left eye symptoms of decreased vision, proptosis, restricted eye movement, and diplopia. The symptoms started following a trauma to the left eye 6 months back. Contrast magnetic resonance imaging scan showed a contrast enhancing well-defined 2.2 cm × 1.8 cm × 1.8 cm fairly rounded, slightly lobulated intraconal tumor in the retrobulbar region inferior to optic nerve. Patient underwent the left fronto-temporo-orbito-zygomatic (FTOZ) craniotomy. The tumor was moderately vascular, firm in consistency with lobulated surface. Few foci of hemorrhages were seen. Near total excision of the tumor was done. Histopathology and immunohistochemistry confirmed the diagnosis of orbital lymphangioma. Although rare, intraconal orbital lymphangioma should be kept in the differential diagnosis of orbital tumors presenting in adult patients following a trauma. It can radiologically mimic other intraorbital tumors. It is a surgical challenge and FTOZ craniotomy provides direct access to the orbital intraconal compartment.