Importance of early closure of the marginal venous system in Klippel-Trenauny and CLOVES syndromes: anatomical and technical considerations

Purpose To describe i.various configurations of the marginal venous system (MVS) in Klippel-Trenauny (KTS) and CLOVES syndromes, ii.the importance of early intervention and iii.report technical considerations. Materials and Methods Patients with KTS and CLOVES underwent venous mapping using direct and diversion venographyfollowed by embolization of anomalous veins. Results In KTS, a persistent sciatic vein was the most consistent and largest part of the MVS. Networks of veins, multiple perforators and communicators were common. In CLOVES, the leg marginal veins communicated with a truncal vein draining into the axillary/subclavian vein. Embolization and endovenous laser caudal to the communication with the iliac veins were performed. Use of “stopper wire/needle,” coil scaffolding or anchoring techniques, pull-back glue, Amplatzer obstructive device, and various combinations were utilized. Prior to implementing this approach, several patients had pulmonary embolism (1 fatality). Conclusion The marginal venous systems in KTS and CLOVES syndromes should be mapped and closed early in life.