Orbital Cavernous Lymphangioma with Intracranial Extension: Case Report

ABSTRACT

Lymphangioma is a rare benign developmental vascular tumor that may be found in orbit, skull and elsewhere in head and neck. Few cases of extension of this benign but insidious tumor posteriorly out of the bony orbital cavity have been reported. The patient was 40-year-old man complaining of proptosis of right eye for one month. Physical examination revealed severe right exophthalmus, impairment of eyeball movement in all directions. Visual acuity was much impaired and he could percept only light with right eye. CT and MRI scans showed intraconal and extraconal involvement of ill-defined, heterogenous mass with extension of the tumor posteriorly beyond the orbital cavity involving right frontal and temporal lobe, skull and subcutaneous tissue. The tumor was subtotally removed via orbito-frontal approach without damaging vital neural and orbital component. Then, orbital roof reconstruction and cranioplasty were done with resin. Successful surgical removal of lymphangioma is very difficult due to its severe infiltration to surrounding tissue and tendency to bleed during debulking. We report a rare case of orbital cavernous lymphangioma with intracranial extension treated with surgical decompression, with review of literatures.