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Is major surgery in hemophiliac patients safe?

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Abstract

In the last 20 years, surgery in patients with hemophilia has been carried out with progressively less mortality and morbidity. Even extensive and major surgical operations can be carried out provided that the underlying coagulopathy is corrected and normal hemostasis restored.

We report the results of 196 operations performed in 147 hemophiliacs during the period 1961–1985 in the Department of Surgery, Warsaw Institute of Hematology. Our patients experienced, in this 25-year period, 23% of hemorrhagic complications in hemophilia A and 12.5% in hemophilia B. In the last 5 years, however, the incidence of hemorrhagic complications was reduced to 5.2% in hemophilia A and 8.3% in hemophilia B. This correlates with the discovery of cryoprecipitate and with the development of concentrates of factor VIII and IX, respectively.

In all patients, deficient factor levels were determined before the operation. The replacement therapy was based on the policy of raising the level of factor VIII or IX above 60% of the normal value and maintaining this level above 30% in the postoperative period until final wound healing.

Problems discussed in this article include the principles of replacement therapy, its advantages and disadvantages, and the organization and degree of cooperation among surgeon, hematologist, and blood bank staff.

Résumé

Au cours des 20 dernières années, la chirurgie chez l'hémophile est allée de pair avec une diminution de la mortalité et de la morbidité. Des interventions chirurgicales étendues et majeures ont pu Être pratiquées dés lors que la coagulopathie était corrigée et que l'hémostase normale était rétablie.

Les auteurs rapportent les résultats de 196 opérations pratiquées chez 147 hémophiles de 1961 à 1985 dans le département de chirurgie de l'Institut d'hématologie de Varsovie. Pendant cette période de 25 ans, 23% des patients atteints d'hémophilie A et 12.5% des sujets hémophiliques B ont été victimes de complications hémorragiques. Il est à noter qu'au cours des 5 dernières années, le taux des complications hémorragiques s'est réduit à 5.2% chez les sujets atteints d'hémophilie A et à 8.3% chez les malades atteints d'hémophilie B. Ces résultats sont en corrélation avec la découverte de précipités congelés et avec respectivement le développement des concentrés de facteur VIII et de facteur IX.

Chez tous les malades, les taux déficitaires des différents facteurs furent précisés avant l'intervention. La thérapeutique substitutive fut entreprise de manière à élever le taux des facteurs VIII et IX jusqu'à 60% du taux normal et à la maintenir à 30% de ce taux normal en post-opératoire jusqu'au terme de la cicatrisation définitive.

Les problèmes envisagés dans cet article concernent les principes du traitement substitutif, de ses avantages et de ses inconvénients, de l'organisation et du degré de la coopération qui doit exister entre chirurgien, hématologiste, et équipe de la banque du sang.

Resumen

En el curso de los Últimos 20 años se ha podido realizar cirugía en la hemofilia con una progresiva disminución de la mortalidad y morbilidad. AÚn operaciones muy extensas pueden ser prácticas siempre y cuando la coagulopatia subyacente sea corregida y se logre el restablecimiento de la hemostasis normal.

Se informan los resultados de 196 operaciones realizadas en 147 hemofilíacos en el período 1961–1985 en el Departamento de Cirugía del Instituto de Hematología de Varsovia. En este período de 25 años, nuestros pacientes experimentaron un 23% de complicaciones hemorrágicas en la hemofilia A y un 12.5% en la hemofilia B. Sinembargo, en los Últimos 5 años la incidencia de complicaciones hemorrágicas fue reducido a 5.2% en la hemofilia A y a 8.3% en la hemofilia B. Esto se correlaciona con el descubrimiento del crioprecipitado y el desarrollo de los concentrados de factor VIII y IX, respectivamente.

Los niveles del factor deficiente fueron determinados antes de la operación en la totalidad de los pacientes. La terapia de reemplazo se basó en la política de elevar el nivel del factor VIII o IX por encima de 60% del nivel normal y de mantener tal nivel por encima del 30% en el período postoperatorio y hasta la cicatrización definitiva de la herida.

También se discuten en este artículo otros problemas, tales como la terapia de reemplazo, sus ventajas y desventajas, y la organización y grado de cooperación entre el cirujano, et hematólogo, y el personal del banco de sangre.

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Authors and Affiliations

  1. Department of Surgery, Research Institute of Hematology and Blood Transfusion, Chocimska 5, 00-957, Warsaw, Poland

    Witold J. Rudowski M.D. (Director and Head), Romuald Scharf M.D. & Jan M. Ziemski M.D.

  2. National Library of Medicine, Bethesda, Maryland, USA

    Witold J. Rudowski M.D. (Director and Head), Romuald Scharf M.D. & Jan M. Ziemski M.D.

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  1. Witold J. Rudowski M.D.
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  2. Romuald Scharf M.D.
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  3. Jan M. Ziemski M.D.
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Rudowski, W.J., Scharf, R. & Ziemski, J.M. Is major surgery in hemophiliac patients safe?. World J. Surg. 11, 378–385 (1987). https://doi.org/10.1007/BF01658122

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