Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation

Afibrinogenemia is a rare hereditary coagulation disorder characterized by a propensity toward bleeding. A 21-year-old Hispanic woman with afibrinogenemia developed ascites, a distended abdomen, an enlarged liver, scleral icterus, and umbilical vein and abdominal wall vein distension. Computed tomography (CT) scan showed occlusion of the hepatic veins and the infrahepatic vena cava consistent with Budd-Chiari syndrome. These developments led to her evaluation for, and placement on, the liver transplantation waiting list. The patient's unmeasurable international normalized ratio (INR) artifactually increased her Model for End-Stage Liver Disease (MELD) score to 40. Immediately before transplant surgery, an initial thrombelastogram (TEG) showed a flat line, indicating a complete lack of fibrin clot formation. Preoperatively, 20 units of cryoprecipitate were infused. The first intraoperative TEG demonstrated a tracing consistent with normal clot formation. Coagulation studies normalized with the newly functioning liver. The liver transplantation appears to have corrected the fibrinogen deficiency, presumably limiting the chance of recurrent Budd-Chiari syndrome. This case report discusses the essential role of fibrinogen in the coagulation cascade, as visualized by thrombelastography, and exposes the interplay between plasma fibrinogen and thrombin levels, which determine coagulation or fibrinolysis.