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.