Potential Utilization of Lymphoscintigraphy in Patients With Klippel-Trenaunay Syndrome.

PURPOSE: Klippel-Trenaunay syndrome (KTS) is a rare disease that was characterized by vascular malformation. Lymphatic malformation was also commonly associated with KTS. However, the application of lymphoscintigraphy in the management of patients with KTS was rarely described. The purpose of this study is to assess whether the findings of lymphoscintigraphy can aid the management of the patients with KTS.

METHODS: A total of 28 patients with known KTS who underwent Tc-dextran lymphoscintigraphy with lower extremity tracer injection were included in this retrospective analysis. The images from lymphoscintigraphy were reviewed for any abnormalities in the body with the attention on the region of left subclavian-jugular venous angle.

RESULTS: In addition to abnormal activity in the other regions, abnormal activity in the left subclavian-jugular venous angle was visualized in over half of the patients (53.6%, 15/28). Based on the findings of the lymphoscintigraphy, 7 patients with left subclavian-jugular venous angle activity underwent thoracic duct decompression. In 4 patients with postsurgery follow-up, 3 achieved significant, measurable symptomatic relief.

CONCLUSIONS: Lymphoscintigraphy can be used to assess potential candidates for thoracic duct decompression to alleviate the symptoms in patients with KTS.