Surgery in Patients with Congenital Factor

S URGERY POSES a maximal challenge to the hemostatic mechanism of the individual and may be life-threatening in patients with certain hemostatic defects, unless protection is offered by proper replacement therapy. Whereas considerable experience has been gained in the control of surgical bleeding in such relatively common disorders as hemophilia A and B, and von Willebrand’s disease, experience is scanty in certain other rare coagulation defects, such as congenital Factor VII deficiency ( congenital hypoproconvertinemia ) . Of the latter entity only 46 authentic cases have been pub lished to date.129 Of the patients who underwent surgery, without replacement therapy, some-but not all-surprisingly had no bleeding difficulties. With replacement therapy none of the patients had any excessive bleeding during or after surgery, but detailed documentation regarding infusion therapy and the Factor VII levels achieved is lacking. Knowledge of required replacement therapy in association with surgery is not only important from a practical point of view, but also throws some light on the physiologic role in hemostasis of the deficient clotting factor. In the present report, our recent experience with a previously published case1 of a 17-year-old patient with severe congenital Factor VII deficiency, who required hysterectomy because of intractable menometrorrhagia, is described. With moderate replacement therapy for a brief time, no excessive bleeding was encountered, which is remarkable in view of the short in vivo survival of Factor VII and the modest rises of Factor VII levels achieved in the patient’s circulation. The pertinent literature is reviewed.