61: Complex labioplasty in patient with congenital primary lymphedema

Abstract

This video summarizes the diagnosis, evaluation, underlying anatomy, surgical intervention, and follow-up in a patient with congenital primary lymphedema undergoing complex labioplasty. We present a case of a 17 year old female with a history of congenital primary lymphedema who underwent complex labioplasty. At birth the patient had thigh and labial swelling increasing in size over time. Imaging was unremarkable. No intervention was felt possible by vascular surgery. After puberty, the left labia minora enlarged to 9 x 7 x 4 cm, the left labia majora to 7 x 3 x 3 cm. This interfered with ADL's including dressing, walking, and pain on sitting. Patient had significant mental stress. Primary lymphedema affects 1 in 100,000 individuals, caused by abnormal development of the lymphatic system (primary) or an insult after birth (secondary). The majority have lower limb involvement, the left leg more often involved. This patient had no prior history of infection, radiation, surgery, or trauma. An awareness of the blood supply of the labia was critical in creating flaps. The initial flap extended from the introitus in a narrow to wider configuration, the lateral portion of labia minora tissue was included to match the color and texture of the right, the pedicle coming from 5 to 7 o'clock with deep dissection 3.5 cm at the base preserving the blood supply. Dissection to the level of the left frenulum. Underlying excess tissue excised allowing the skin flap to be placed in a normal configuration. Left labia majora reduced with an elliptical incision and underlying tissue excised to reduce the bulk. Postoperatively the patient had minimal separation of the superior aspect of labia minora resutured in the office setting with marked improvement in the overall appearance. This case illustrates the importance of conserving the blood supply to the labia in the creation of viable flaps in complex labioplasty.