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.