Radiotherapy in the Management of Klippel-Trénaunay-Weber Syndrome: Report of Two Cases

Klippel-Trénaunay-Weber syndrome (KTWS) is characterized by combined vascular malfomations of capillary, venous, and lymphatic types usually observed during infancy or childhood. In this report, we describe two KTWS patients treated with radiotherapy after long-term conservative treatment or multiple surgical interventions. The first patient, a 15-year-old female, presented with vascular masses located on the right upper extremity. She was treated conservatively for 14 years, and amputation was offered at the age of 14 due to cardiac failure. A course of radiotherapy to a total dose of 40 Gy with conventional daily fractionation was administered as an alternative to the mutilating surgery, and prominent improvement both subjectively and objectively was detected 1 year after radiotherapy. The second patient, a 40-year-old male, suffered from huge vascular masses of both the trunk and lower extremities. Despite multiple surgical interventions and conservative treatment, disease progressed and neurological symptoms occurred due to compression of vertebral structures. He was administered a course of 30 Gy radiotherapy with 1.5 Gy daily fractions to the lower thoracic region and whole abdominal cavity. Six months after radiotherapy, subjective relief described by the patient but without any objective response was detected. Though considered as a benign disease, KTWS can cause life-threatening complications. The response of one of our patients is a hopeful finding for considering radiotherapy as an alternative treatment modality for these patients.