Double skull lymphangioma. Case report and review of the literature

It is still controversial whether lymphangiomas are a true entity or a combination of congenital venous and lymphatic malformations. Lymphangioma diagnosis is made on a multifactorial basis and surgery should provide cosmetic and functional preserving results. Only few multiple localization have been described in the literature and to our knowledge this is the first case reported on a double localization (orbital and temporal) in the skull.

A 10-year-old girl was referred to our department with a rapidly enlarging subcutaneous mass in the right eyebrow and upper eyelid, displacing the eyeball and causing proptosis and diplopia that occurred synchronous with her menarche. A head MRI scan showed two distinct lesions, one into the orbit causing bony destruction and the other one in the sub-temporal area. The little girl underwent an open biopsy, suggesting the diagnosis of lymphangioma. Surgery through an anterior approach with total removal of both lesions was performed. Pathological examination disclosed a lesion consisting of multiple cysts with erythrocytes and lymphs, confirming the diagnosis. At a 6- and 12-months follow-up, the girl was clinically intact with normal ocular movements and a follow-up MRI showed no evidence of residual disease and/or recurrence.