Tissue expansion in the management of Klippel–Trénaunay syndrome in pediatric patients

Abstract

Summary Klippel–Trenaunay syndrome (KTS) is a rare congenital disorder that consists of mixed vascular malformations. The management of KTS is challenging and multidimensional. Debulking or excisional surgery may be performed in certain patients with KTS. Tissue expansion is a widely used reconstruction technique in plastic surgery and facilitates the reconstruction of skin and soft tissue defects after the excision of large vascular anomalies. In children with lower extremity KTS, large areas of the involved skin and soft tissue can be removed safely and reconstructed by using expanded tissue. In this paper, we report two cases of pediatric lower limb KTS with large cutaneous involvement and substantial soft tissue hypertrophy around the knee. The placement of a tissue expander was planned preoperatively. For 18 weeks postimplantation, the expander was overinflated to 3–4 times its capacity. Partial excision of the malformed tissue around the knee was subsequently performed. The resultant defect was reconstructed using the expanded tissue. Both patients were postoperatively inspected regularly at our outpatient clinic. Neither patient had surgical or tissue expander-related complications. One year after surgery, there was no evidence of local recurrence. Both children retained full range of knee joint motion without limitations in activities of daily living. Tissue expansion is an effective and reliable method of reconstruction. It may be considered in addition to surgical excision in patients with KTS with substantial cutaneous involvement around the joints. Long-term management and close follow ups are necessary for achieving satisfactory outcomes.