Radical resection and reconstruction with bilateral gluteal fold perforator flaps for vulvar hemangiolymphangioma

Abstract

Hemangiolymphangioma is a rare type of lymphatic malformation characterized by vascular and lymphatic elements on histologic examination [1]. The present paper reports a rare case of vulvar hemangiolymphangioma and the successful surgical reconstruction with bilateral gluteal fold perforator flaps. A 16-year-old patient was referred in August 2012 for progressive swelling of the genitals that had been ongoing for 8 years. The patient complained of intermittent discharge of a white fluid mixed with blood from her perineum since the age of 8 years. On physical examination, erythema and tenderness of the labia majora were present, with friable bleeding upon palpation. Seven months prior to evaluation, the patient had experienced an episode of massive perineal hemorrhage resulting in severe anemia (7 g/dL). The hemorrhage was managed conservatively with pressure and blood transfusions. The following month the patient underwent sclerotherapy, but no subsequent reduction in mass size or symptoms was noted. Given the severity of the patient's condition, the decision was ultimately made to proceed with a radical resection of the involved areas. Bilateral vulvectomy was performed and the resulting defect was reconstructed with bilateral gluteal fold flaps based on internal pudendal artery perforators (Fig. 1). The flap dimensions were 15×6 cm on the right and 17×8 cm on the left side. Both the defect and donor sites were primarily closed (Fig. 2). The postoperative course was