3.2 | Case 2: Pain and VTE risk in complex vascular malformations
Patient 2 is a 15-year-old male with extensive Klippel-Trenaunay syndrome of the left lower extremity. Her vascular malformation includes a significant ectatic slow-flow vessel. Growth, infections and pain have been fairly controlled since starting sirolimus 3 years ago. Over the past year, she has become more active in sports. She presented with localized left leg pain with palpable knots and an elevated D-dimer (4 times ULN) consistent with LIC. A short 2 week treatment with low-molecular-weight heparin (LMWH) was initiated which resulted decreased pain, resolution of palpable knots, and decreased D-dimer. However, within a couple of weeks of completing the course of anticoagulation, her pain and D-dimer elevation recurred. A left lower extremity recurrent thrombus was confirmed on ultrasound. After a 12 week course of anticoagulation, risks and benefits were discussed and she was transitioned to rivaroxaban. Anticoagulation was continued due to patient’s recurrent VTE and increased risk of thrombosis due to the extensive ectatic slow-flow vessel. She is currently managed on sirolimus and rivaroxaban with plans to decrease her VTE risk by obliterating the ectatic slow-flow vessel through mechanicochemical ablation with ClariVein.