Case Presentation: 46 year-old-female with past medical history of hypertension, Factor VII(FVII) deficiency, morbid obesity, thoracic spine stenosis presented with bilateral lower extremity weakness. Computed Tomography (CT) myelogram showed severe canal stenosis with spinal cord compression at Thoracic(T)10-11 and T11-12 levels and the spine team was planning surgery. Pre-operative labs showed prothrombin time(PT) of 21.2 seconds (normal 12 to 14.7), INR of 1.90 (normal 0.85 to 1.17) and normal activated partial thromboplastin time (aPTT). She is not on any blood thinners. No personal or family history of bleeding disorder. Mixing study done showed correction of PT. FVII level was low at 24%(normal 72- 205). Hematology recommended to give recombinant Factor VII (RFVII) immediately before surgery and 6 to 8 hours after surgery. Patient underwent T10- 12 revision decompression with posterior instrumented fusion. RFVII 2 milligram(mg) was given prior to surgery and repeat dose of RFVII 1 mg was given 8 hours after surgery. Repeat FVII level was normal at 89%. Patient tolerated surgery well without any bleeding complications and patient was subsequently discharged to rehabilitation facility.
Discussion: Factor VII (FVII) deficiency is a rare bleeding disorder and is also called as Alexander’s disease. Clinical severity of FVII deficiency can vary from no symptoms to severe life threatening bleeding. Interestingly, there is no direct correlation between bleeding manifestation and FVII level. There are environmental and inherited modifier components that modulate the manifestation of FVII deficiency. FVII deficiency can inherited or acquired. Inherited Factor VII deficiency is an autosomal recessive disorder. Acquired FVII deficiency can result from severe liver disease, vitamin K deficiency or sepsis. Individuals with factor VII deficiency have a normal aPTT and a prolonged PT.Treatment for FVII deficiency is based on the potential benefits and risk on case to case basis. For bleeding issues in FVII deficiency treatments include RVII, prothrombin complex concentrate (PCC) and fresh frozen plasma(FFP). NovoSeven is a genetically engineered recombinant version of FVII used to treat bleeding issues in individuals with FVII deficiency. There is no risk of transmitting blood-borne pathogens as it does not contain human blood or plasma. PCC is a concentrate form of four clotting factors: II, VII, IX and X. Theoretically, factor VII deficiency can be treated with FFP. But in clinical practice because of the requirement of high volume of FFP and the short half-life of FVII it is impractical to manage with FFP.
Conclusions: Pre-operative bleeding risk is assessed based on history and physical examination. If the history and physical examination do not suggest the presence of a bleeding disorder, no additional laboratory testing is recommended(1). If the history or physical examination suggests the presence of a bleeding disorder, appropriate screening tests should be performed, including PT, aPTT, platelet count and thromboelastography (TEG). Abnormalities in above tests may need further investigations including factor levels and may warrant hematology consult. Managing orthopedic patients is common in hospital medicine practice and this case illustrates how me managed an orthopedic spine surgery in a patient with bleeding disorder. Bleeding during spine surgeries can result in increased morbidity and mortality. So it is important to assess bleeding risk pre operatively and manage accordingly.