Orbital lymphangiomas: surgical treatment and clinical outcomes.

Background: Orbital lymphangiomas (OL) are rare benign lesions that represent less than 4% of all space-occupying orbital lesions. Total surgical resection is usually difficult because of its infiltrative nature. Our objective was to analyze the indications for surgical treatment, the selection of surgical approach, and the clinical outcome after surgical treatment of OL.

Methods: Five patients with OL who were surgically treated between January 2003 and December 2009 were included. Clinical, surgical, and follow-up data were retrospectively analyzed.

Results: Mean age was 32 years, with two patients treated when they were adults. Proptosis was present in all patients, and the mean duration of symptoms was 9.4 years. Two patients had clinically relevant hemorrhagic episodes. Three patients presenting with large retrobulbar tumors were submitted to the pterional approach with superolateral orbitotomy; in one patient, a lateral approach was used and in another an anterior superomedial approach was used. In all cases a subtotal removal was achieved. There was no permanent morbidity. With a mean follow-up of 3.2 years, no patient presented new hemorrhagic episodes or tumor recurrence.

Conclusion: Subtotal resection of OL is an effective and safe treatment option, may prevent new hemorrhagic episodes, and is not associated with a high recurrence rate after a relatively short-term follow-up. Progressive proptosis, visual or oculomotor impairment, and repeated symptomatic hemorrhagic episodes are considered indications for surgery. Transcranial access should be performed in large tumors extending posteriorly.