Vulvar congenital dysplastic angiopathy

Congenital dysplastic angiopathy is a syndrome consisting of vascular angiomata, congenital varicosities, and trophic changes of the soft tissue and the skeleton. Frequently referred to as Klippel-Trenaunay or Klippel-Trenaunay-Weber syndrome, it rarely affects the female genitalia. An 18-year-old woman underwent evaluation and treatment for Klippel-Trenaunay syndrome with vulvar involvement. Preoperative evaluation included consultation with pediatric surgeons, gynecologic surgeons, and an interventional radiologist as well as individual and family psychological counseling. Attention to intraoperative detail resulted in minimal operative blood loss and preservation of normal anatomy. A postoperative hematoma was treated aggressively with surgical evacuation and drainage. Six-month follow-up revealed functionally and cosmetically normal vulvar anatomy.