PURPOSE

To evaluate the results of surgical intervention and rate of recurrence in patients with periocular lymphangioma with respect to the anatomic extent of tumor involvement determined by neuroimaging studies.

DESIGN

Retrospective noncomparative case series.

PARTICIPANTS

Eighteen patients with periocular lymphangioma.

METHODS

All patients underwent complete ocular examination and preoperative orbital imaging with either computed tomography or magnetic resonance imaging. Tumor location in the orbit was defined as intraconal, extraconal, or diffuse. For extraconal cases, a further classification was made as intraorbital, extraorbital, or mixed. All patients underwent orbitotomy via either a conjunctival or a skin-crease approach, depending on the tumor location. The indications for surgical intervention were varied and included preservation of visual function or cosmetic appearance in some cases and exploration of an unidentified orbital mass in others. The diagnosis of lymphangioma was established histopathologically in each case. Cases that showed recurrence at follow-up also were treated using an orbitotomy approach.

MAIN OUTCOME MEASURES

Anatomic extent of tumor location in the orbit as determined by neuroimaging studies, completeness of surgical excision, pathological findings, recurrence, and visual outcome.

RESULTS

Seven patients had extraconal tumors; 4, intraconal tumors, and 7, diffuse tumors. Of the patients with extraconal tumors, 4 had mixed lymphangioma and 3 had extraorbital lymphangioma. In 5 cases, the extraconal lymphangiomas were totally resected. In the remaining 13 cases with diffuse, intraconal, and infiltrating extraconal lymphangiomas, the tumors were treated with a subtotal resection procedure. At a mean follow-up of 29.4 months (range: 3-77), 2 patients with diffuse tumors developed 4 recurrences and underwent further surgical intervention for 3 recurrent events. The mean interval between treatment and recurrence was 16 months (range: 12-20). Two patients experienced decreased visual acuity due to compressive optic neuropathy in one case and operative damage in the other.

CONCLUSIONS

Surgically well-delineated extraconal lymphangiomas can be resected totally with no clinical recurrence at short-term follow-up. Although intraconal, diffuse, and some extraconal lymphangiomas were treated with a subtotal resection procedure, many such cases did not demonstrate recurrence. These results are encouraging because they demonstrate that many periocular lymphangiomas can be treated successfully with good visual outcome and satisfactory cosmesis.