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
- Vincristine IV 0.05 mg/kg once weekly may also be considered instead
of sirolimus based on family and provider preferenceSupportive Care:
- Cryoprecipitate for active bleeding, planned invasive procedure and
fibrinogen < 100 mg/dL
- Platelet transfusion ONLY for active bleeding or planned invasive
procedures if < 50,000/mm3
- PRBC transfusion for severe and/or symptomatic anemia (usually Hb
< 8 g/dL)Second line for recurrent or refractory KMP:
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.