Orbital lymphangioma: clinical features and management.

PURPOSE To report 5 cases of orbital lymphangioma. METHODS All patients underwent ophthalmological, clinical and neuroradiological evaluation. RESULTS At presentation all patients (4 children and one adult patient) had unilateral proptosis. Restricted eye movement was present in 3 patients, ptosis and amblyopia in 2. Compressive optic neuropathy with visual loss was noticed in one patient. Palatal localization was found in 2 patients, maxillofacial localization in one. Associated intracranial vascular anomaly was present in one patient. Three patients who underwent surgery, developed recurrences. No regression of lymphangioma was noticed nor with Interferon, nor with steroids. CONCLUSION Lymphangioma has to be included in the differential diagnosis of childhood proptosis. Extraorbital localization, under which associated intracranial developmental venous vascular anomaly, has to be searched for. In most of the cases conservative treatment is mandatory.