Surgery of hemophiliacs--20 years' experience

Seventy-seven hemophilic patients of type A or type B were subjected to a total of 108 major surgical procedures mainly in the field of general surgery, orthopedic surgery, or neurosurgery. The principles for the substitution therapy in the different types of procedures and different types of hemophilic diseases are described, as well as the indications for surgery and the surgical technique. The importance of prolonged substitution therapy postoperatively to avoid late hematoma, particularly in patients with severe hemophilia undergoing major surgery, is stressed. With this type of management there has been no increased intraoperative hemorrhage, and very few cases of late hematoma formation. By combining the substitution therapy with immunosuppression, it has been possible to operate also on patients with inhibitors against factor VIII or IX. The rate of complications, particularly the incidence of hepatitis, has been low with the type of substitution given in this series of patients. It is concluded that major surgery can be carried out even in severe hemophilia without significantly increased risk. The handling of the substitution therapy, and the surgical judgment and technique, offers however, special problems, necessitating centralization of elective cases.