Czech and Slovak Ophthalmology 488 Original article Lymphangioma of the Orbitopalpebral Area Krásný Jan https://orcid.org/0000-0002-0341-0094 Baráková Drahomíra Chodounský Zdeněk Šach Josef https://orcid.org/0000-0001-9287-7915 18 6 2014 4 5

Aim: The authors refer about five patients with different types of lymphangioma, who were followedup at the Department of Ophthalmology, Faculty Hospital Královské Vinohrady (King’s Vinegards), Charles University, Prague, Czech Republic, E.U., during the period 1995 – 2013; the follow-up period lasted from 5 to 17 years. The lymphangioma of the orbitopalpebral area is discussed according to the evaluation of the tumor development, histological verification, treatment, and its results. Methods: In four boys, the first signs of tumor were eyeball protrusion (exophthalmos) and bleeding into the conjunctiva or palpebral skin before the age of 5 years. In all four patients, the histological confirmation of the orbital lymphangioma was performed in the beginning of the disease. In three cases, it was the orbital type, and the fourth one was frontal type with bilateral orbital lymphangiomatosis. In one girl, there were present conjunctival changes only, appearing as one-sided hyperplastic changes. For these changes, she was followed-up since her 13 years of age under the false diagnosis of chronic conjunctivitis. The definite histological confirmation of only conjunctival lymphangioma was done from the diagnostic probatory biopsy not until ten years of symptoms and unsatisfactory treatment. Results: In the girl with superficial conjunctival lymphangioma and in the patient with lymphangiomatosis, the follow-up was recommended only. In two patients with extraconal type of orbital tumor, the total or sub-total resection was performed. In the years of the follow-up, the remission of the disease was observed. In the patient with mostly intraconal type of the tumor, causing decrease of the visual acuity according to the optic nerve neuropathy and macular cystoid edema, the focused actinotherapy by means of linear accelerator treatment with the dose of 30 Gy after previous evacuation of chocolate cysts under ultrasound control. The regression of the tumor and normalized visual functions lasted for 17 years. Conclusion: As method of treatment of extraconal lymphangiomas, it seems, it is its resection, and in the intraconal localization of the tumor it is the focused actinotherapy by means of linear accelerator.

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