Lymphovenous Anastomosis and Secondary Resection for Noonan Syndrome with Vulvar Lymphangiectasia.

In this case report we describe the use of a 2-stage approach to treat severe recurrent vulvar lymphangiectasia in a patient with Noonan syndrome. First, 3 functional lymphatic vessels were identified and anastomosed to venules in an end-to-end fashion. Then, in a second surgical procedure, the vulvar lesions were resected as much as possible and the vulva was reconstructed. By the 12-month follow-up the patient had recovered well. Although there were still some small vesicles on the left labia there was no more ooze, itch, and pain. Lymphatic mapping using indocyanine green showed improvement of the edema of her vulva region and patent LVA. In addition to the demonstration of this 2-stage approach, this case report also demonstrates the benefits of preemptive LVA before performing surgery that may be at high risk for postoperative lymph edema.