First line medical management:
Start oral prednisolone or IV methylprednisolone 2mg/kg/day plus sirolimus 0.8mg/m2/day twice daily (target trough 8-12)
Begin to taper dose once KMP resolves (platelets >100K, fibrinogen >100) to off or to lowest tolerable dose
Add aspirin 10 mg/kg/day and/or ticlopidine 10 mg/kg/day
Figure 1 Laboratory trend for Case 1 showing fibrinogen and d-dimer over time and in relation to treatments. Also depicted are transfusions of platelets (*) and cryoprecipitate (#).
Figure 2 Extensive venous malformation of the anterior hip (left) and posterior back (right).
Figure 3 Institutional guideline for perioperative management of slow-flow vascular malformations. This is not consensus-driven or based on high-quality clinical trial data.