Tailored Charles' procedure and vascularized lymph node transfer for advanced stage Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome (KTS) is primarily managed for vascular and soft-tissue abnormalities, while treatment of associated lymphedema remains poorly described. This study outlines our protocol for advanced lower-extremity lymphedema using a combined modified Charles' procedure and vascularized lymph node transfer (VLNT).

KTS patients with International Society of Lymphology (ISL) stage III lower-limb lymphedema treated between 1999 and 2018 were retrospectively reviewed. All underwent a modified Charles' excisional procedure with Homans techniques and VLNT. Outcomes were assessed at least one year postoperatively, focusing on early complications and postoperative hospital admissions for residual disease or recurrent infections.

Twenty-two patients (14 male, 8 female) with a mean age of 21 years (range, 4-41) were included. Average time since KTS diagnosis was 14 years (range, 3-26). VLNT donor sites included groin (18.2%), supraclavicular (31.8%), and gastroepiploic (50%). Mean hospital stay was 16 days (range, 14-39). Follow-up averaged 38 months (range, 27-45). Three minor complications occurred (one wound dehiscence, one infection, one bleeding), and flap survival was 100%. Annual hospital admissions for soft-tissue infections decreased from a preoperative mean of 3.5 to 1.2 postoperatively. Patients required an average of 3.5 additional procedures (range, 2-8) to remove residual hemangiolymphangioma.

Lymphedema in KTS presents unique challenges and differs markedly from typical primary or secondary lymphedema. Effective management requires meticulous assessment and individualized planning. In this population, functional improvement is the main objective, and extensive debulking procedures combined with physiologic reconstruction may provide meaningful clinical benefit despite limited aesthetic outcomes.

Type of Research: Single-center, retrospective, cohort studyTake Home Message: In severe lymphedema of patients diagnosed with KTS, single excisional or physiological surgical treatment is insufficient to fight the totality of pathologic processes at work. A combination of varied techniques that act on different disease's features can achieve significantly better outcomes.