3.3 | Case 3: Perioperative management in patient with LIC
Patient 3 is a 25-year-old male with a history of multifocal venous malformation of the chest wall, back, retroperitoneum, abdomen, scrotum, buttocks and right lower extremity. [Figure 2] He had a history of significant bleeding after a knee surgery (synovectomy) at age 15 years complicated by a report of disseminated intravascular coagulopathy (DIC). Upon presentation to hematology 4 years ago he had the following labs: Platelet count 121,000/mm3, PT 13.6 sec, aPTT 27 sec, fibrinogen <80 mg/dl, d-dimer 24.22 mg/L FEU. He had significant pain and hard nodules in his malformation and was started on rivaroxaban 10mg daily and titrated up to 20 mg daily. Sirolimus was subsequently added and pain and coagulation labs improved some. He underwent a surgical debulking of a lesion on his back and developed a significant hematoma at the surgical site while on LMWH 1mg/kg/dose twice daily. Due to the persistent pain he was scheduled for glue embolization and resection followed by sclerotherapy of several of the malformations on his back and chest. Prior to this procedure he had the following labs: platelet count 164,000/mm3, fibrinogen 148 mg/dl, d-dimer 6.37 mg/L FEU. He was switched to LMWH 40mg once daily for the procedure given his previous history of bleeding complication. He tolerated procedure without bleeding complications but labs after procedure showed d-dimer >30mg/L FEU, fibrinogen <80 mg/dl and platelets 77,000/mm3. LMWH was increased to therapeutic dosing and he received several transfusions of cryoprecipitate and platelets for some bleeding from the wound and development of a large flank wall hematoma. On therapeutic LMWH his platelets improved to 244,000/mm3, fibrinogen to 260 mg/dl and d-dimer to 8.92 mg/L FEU and the bleeding ceased. He is currently managed on ongoing rivaroxaban 20 mg daily with normal platelet count and fibrinogen level and d-dimer is 0.79 mg/L FEU.