Major labiaectomy as surgical management of vulvar lymphangioma circumscriptum: three cases and a review of the literature

Abstract

Vulvar lymphangioma circumscriptum (LC) is a developmental defect of lymphatics in deep dermal and subcutaneous layers. The entity may be acquired that induced by surgical intervention and radiation therapy for malignancy or congenital. Lymphangiomas of the vulva are rare. Thirteen cases of the congenital and 24 cases of acquired form have been reported in the literature presently. To evaluate major labiaectomy as surgical management of vulval lymphangioma, we report our experiences on surgical treatment of three cases of this rare disease from Gynecologic Oncology Department of Vali-E-Asr Hospital. Three female patients, aged 18, 17 and 63 years, presented with persistent edema, papules, and vesicles of the labia major, among which two first cases had congenital LC and another case had developed 9 years after radical hysterectomy, lymph node dissection, and adjuvant radiotherapy of cervical cancer. Overall there was a clear improvement in the symptoms and signs associated with these conditions in all patients. In particular, all patients reported an improvement in the amount of edema following surgery. In particular, major labiaectomy seems to be more successful than methods such as lymphovenous anastomoses and lymph angioplasties. A single operation may prove beneficial; also, this approach has the potential to allow patients to rehabilitate to normal life and activity.