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Surgery of hemophiliacs—20 years' experience

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Abstract

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.

Résumé

Soixante-dix-sept (77) patients porteurs d'hémophylie A ou B furent soumis à un total de centhuit (108) opérations chirurgicales majeures relevant principalement de la chirurgie générale, de la chirurgie orthopédique ou de la neurochirurgie. L'article décrit les principes guidant le remplacement des facteurs anti-hémophyliques selon les différents types d'hémophylie et d'intervention ainsi que les indications opératoires et la technique chirurgicale. On y souligne l'importance de prolonger la thérapie de substitution durant la période post-opératoire pour éviter la formation d'hématome tardif, en particulier chez les cas d'hémophylie grave soumis à des interventions chirurgicales majeures. Grâce au traitement employé, on a évité les hemorragies peropératoires et quelques rares cas seulement d'hématomes post-opératoires ont été observés. En combinant le remplacement des facteurs anti-hémophyliques et l'immunosuppression, mÊme les patients porteurs d'inhibiteurs des facteurs VIII et IX ont pu Être opérés. Le taux de complication, en particulier l'incidence d'hépatite, est demeuré bas. En conclusion, l'hémophylie mÊme sévère est compatible avec la chirurgie majeure et ce, sans augmentation significative du risque opératoire. Cependant, l'application du traitement de substitution de mÊme que le jugement et la technique chirurgicale posent des problÊmes importants qui nécessitent la centralisation des cas électifs.

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Authors and Affiliations

  1. Coagulation Laboratory, Malmö General Hospital, University of Lund, Malmö, Sweden

    Inga Marie Nilsson M.D., Ulla Hedner M.D., åke Ahlberg M.D., S. Anders Larsson M.D. & Sven -Erik Bergentz M.D.

  2. Department of Orthopedics, Malmö General Hospital, University of Lund, Malmö, Sweden

    Inga Marie Nilsson M.D., Ulla Hedner M.D., åke Ahlberg M.D., S. Anders Larsson M.D. & Sven -Erik Bergentz M.D.

  3. Department of Surgery, Malmö General Hospital, University of Lund, Malmö, Sweden

    Inga Marie Nilsson M.D., Ulla Hedner M.D., åke Ahlberg M.D., S. Anders Larsson M.D. & Sven -Erik Bergentz M.D.

Authors
  1. Inga Marie Nilsson M.D.
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  2. Ulla Hedner M.D.
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  3. åke Ahlberg M.D.
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  4. S. Anders Larsson M.D.
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  5. Sven -Erik Bergentz M.D.
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Additional information

International treatment and training centre of the World Federation of Hemophiliacs.

Supported by grants from the Swedish Medical Research Council (B76-19X-00087-12B), (B76-17X-00759-11B).

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Nilsson, I.M., Hedner, U., Ahlberg, å. et al. Surgery of hemophiliacs—20 years' experience. World J. Surg. 1, 55–66 (1977). https://doi.org/10.1007/BF01654733

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