Orbital lymphangioma and its association with intracranial venous angioma

Abstract

Orbital lymphangioma is an uncommon, benign cystic lesion, generally manifest in childhood. It usually presents with a slowly progressive proptosis, displacement of the globe, ptosis and restriction of eye movements. Occasionally, focal lesions may remain asymptomatic. Spontaneous intraorbital haemorrhage may cause acute proptosis, compressive optic neuropathy and loss of vision. An 8-year-old girl presented with progressive left proptosis and a unilateral soft tissue mass at the supraorbital frontal region. Multiple intracranial venous angiomas were seen on MRI. Surgical decompression of the optic nerve was performed because of progressive decrease in visual acuity. We review the relevant literature; rarity of the orbital lymphangioma and its association with non-contiguous intracranial vascular anomalies were emphasized.