Reports of operative mortality ranging from 30% to 60% have long been a deterrent to surgery in the hemophiliac. From these reports arose a concept of limited surgery, and then only in urgent cases. Since 1952, however, a better understanding of the pathophysiology of hemophilia has been made possible through the work of Biggs,1 Fraenke,2 Alexander,3 MacFarlane,4 Brink-hous,5 and others. Their experimental and clinical findings have led to an effective approach to the management of hemophiliac patients who are undergoing major surgery. As a result, operative mortality has been dramatically reduced.
This report presents an illustrative case of hemophilia and discusses principles of preoperative and postoperative management. The proven cases of classical hemophilia (hemophilia A or AHG deficiency) are reviewed to evaluate current therapy. Excluded from this discussion are diseases characterized by a deficiency of other coagulation factors (e.g., hemophilia B and C).