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Surgery in the Hemophiliac Patient

Surgery in the Hemophiliac Patient Abstract 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). Report of References 1. "Briefly the thromboplastin generation test consists of mixing aluminum-hydroxide-treated plasma, serum, a platelet suspension, M/40 CaCl2, and incubating at 37 C. At 1-min. intervals samples are removed and the clotting time measured when added to citrated normal plasma. The patient may provide either plasma, serum, or platelets." Macfarlane, R. G., and Biggs, R.: Diagnosis and Treatment of Hemophilia and Its Related Conditions, Medical Research Council Memorandum No. 32, London, Her Majesty's Stationery Office, 1955, copyrighted by the Crown. † "The partial thromboplastin time is the clotting time of a mixture of oxalated plasma, 0.3 ml. cephalin suspension, and CaCl2 solution." Brown, I. W.; Smith, W. W., and House, R. M.: Surgical Management in Hemophilia and the Hemophilioid Diseases, Ann. Surg. 149:721 (May) 1959. 2. Biggs, R.: Assay of Antihemophilic Globulin in Treatment of Hemophilic Patients , Lancet 2:311 ( (Aug.) ) 1957.Crossref 3. Fraenkel, G. J.: Surgery in Hemophilia , J. Roy. Coll. Surgeons, Edinburgh 3:54 ( (Sept.) ) 1957. 4. Alexander, B.: Medical Progress: Coagulation. Hemorrhage and Thrombosis , New England J. Med. 252:218 ( (Jan.) ) 1955.Crossref 5. MacFarlane, R. G.; Mallam, P. C.; Witts, L. G.; Bidwell, E.; Biggs, R.; Fraenkel, G. J.; Honey, G. E., and Taylor, K. B.: Surgery in Hemophilia: The Use of Animal Antihemophilic Globulin and Human Plasma in 13 Cases , Lancet 2:251 ( (Aug.) ) 1957.Crossref 6. Brinkhous, K. M.; Penick, G. D.; Langdell, R. D.; Wagner, R. H., and Graham, J. B.: Physiologic Basis of Transfusion Therapy in Hemophilia , A.M.A. Arch. Path. 61:6 ( (Jan.) ) 1956. 7. Craddock, C. G.; Fenninger, L. D., and Simmons, B.: Hemophilia: Problem of Surgical Intervention for Accessory Disease; Review of a Case , Ann. Surg. 128:888-903 ( (Nov.) ) 1948.Crossref 8. Langdell, R. D.; Wagner, R. H., and Brink-hous, K. M.: Antihemophilic Factor (AHF) Levels Following Transfusions of Blood, Plasma, and Plasma Fractions , Proc. Soc. Exper. Biol. & Med. 88:212-215 ( (Feb.) ) 1955. 9. Walker, W.: Peptic Ulcer in Hemophiliac Treated by Gastrectomy , Lancet 1:749 ( (April) ) 1955. 10. Gross, J. D.; Hartmann, R. C., and Graham, J. B.: Splenectomy in Hemophilia , Bull. Johns Hopkins Hosp. 100:223 ( (May) ) 1957. 11. Young, J. D., and Sacks, M. S.: Prostatectomy in a Hemophiliac , J. Urol. 78:644 ( (Nov.) ) 1957. 12. Pitney, W. K.: The Management of the Patient with a Hemostatic Defect During the Period of Surgery , M.J. Australia 1:65-68 ( (Jan.) ) 1959. 13. Adams, J. F.; Timbury, G. C., and Mellon, J. P.: Appendicitis in a Hemophilic , Scottish M.J. 2:328 ( (Aug.) ) 1957. 14. Westlin, W. F., Jr.; Mills, S. D., and Owen, C. A., Jr.: Current Status of Hemophilia Problem , Minnesota Med. 41:705 ( (Oct.) ) 1958. 15. Parks, A. G., and Wass, S. H.: Hemophilia with Small Bowel Obstruction Due to an Ileal Hematoma , Proc. Roy. Soc. Med. 149:933 ( (Nov.) ) 1956. 16. Desmond, A. M.: Hemophilia: Gastric Ulcer; Hematemesis; Emergency Gastrectomy , Proc. Roy. Soc. Med. 48:523 ( (July) ) 1955. 17. Israels, M. C. G.; Lempert, H., and Gilbertson, E.: Hemophilia in the Female , Lancet 1:1375 ( (June) ) 1951. 18. Hartmann, J. R., and Diamond, L. K.: Hemophilia and Related Hemorrhagic Disorders , Practitioner 178:179 ( (Feb.) ) 1957. 19. van Creveld, S.; Hoorweg, P. G., and Stijn, R. W.: Surgical Intervention in Cases of Hemorrhagic Diathesis , Arch. chir. neerl. 9:263-273, 1957. 20. deBruijne, J. I.; van Creveld, S., and Stiendijk, R.: Surgical Treatment of Pyloric Stenosis in an Infant with Hemophilia A , J. Pediat. 51: 698-703 ( (Dec.) ) 1957. 21. Pieper, G. R.; Perry, S., and Burroughs, J.: Surgical Intervention in Hemophilia , J.A.M.A. 170:33 ( (May) ) 1959. 22. Brown, I. W.; Smith, W. W., and House, R. M.: Surgical Management in Hemophilia and the Hemophilioid Diseases , Ann. Surg. 149:721 ( (May) ) 1959.Crossref 23. Meynell, M. J., and Tudor, R. W.: Successful Partial Resection of the Small Bowel in the Case of Hemophilia , Brit. J. Surg. 44:182 ( (Sept.) ) 1956.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1960 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1960.01300060010003
Publisher site
See Article on Publisher Site

Abstract

Abstract 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). Report of References 1. "Briefly the thromboplastin generation test consists of mixing aluminum-hydroxide-treated plasma, serum, a platelet suspension, M/40 CaCl2, and incubating at 37 C. At 1-min. intervals samples are removed and the clotting time measured when added to citrated normal plasma. The patient may provide either plasma, serum, or platelets." Macfarlane, R. G., and Biggs, R.: Diagnosis and Treatment of Hemophilia and Its Related Conditions, Medical Research Council Memorandum No. 32, London, Her Majesty's Stationery Office, 1955, copyrighted by the Crown. † "The partial thromboplastin time is the clotting time of a mixture of oxalated plasma, 0.3 ml. cephalin suspension, and CaCl2 solution." Brown, I. W.; Smith, W. W., and House, R. M.: Surgical Management in Hemophilia and the Hemophilioid Diseases, Ann. Surg. 149:721 (May) 1959. 2. Biggs, R.: Assay of Antihemophilic Globulin in Treatment of Hemophilic Patients , Lancet 2:311 ( (Aug.) ) 1957.Crossref 3. Fraenkel, G. J.: Surgery in Hemophilia , J. Roy. Coll. Surgeons, Edinburgh 3:54 ( (Sept.) ) 1957. 4. Alexander, B.: Medical Progress: Coagulation. Hemorrhage and Thrombosis , New England J. Med. 252:218 ( (Jan.) ) 1955.Crossref 5. MacFarlane, R. G.; Mallam, P. C.; Witts, L. G.; Bidwell, E.; Biggs, R.; Fraenkel, G. J.; Honey, G. E., and Taylor, K. B.: Surgery in Hemophilia: The Use of Animal Antihemophilic Globulin and Human Plasma in 13 Cases , Lancet 2:251 ( (Aug.) ) 1957.Crossref 6. Brinkhous, K. M.; Penick, G. D.; Langdell, R. D.; Wagner, R. H., and Graham, J. B.: Physiologic Basis of Transfusion Therapy in Hemophilia , A.M.A. Arch. Path. 61:6 ( (Jan.) ) 1956. 7. Craddock, C. G.; Fenninger, L. D., and Simmons, B.: Hemophilia: Problem of Surgical Intervention for Accessory Disease; Review of a Case , Ann. Surg. 128:888-903 ( (Nov.) ) 1948.Crossref 8. Langdell, R. D.; Wagner, R. H., and Brink-hous, K. M.: Antihemophilic Factor (AHF) Levels Following Transfusions of Blood, Plasma, and Plasma Fractions , Proc. Soc. Exper. Biol. & Med. 88:212-215 ( (Feb.) ) 1955. 9. Walker, W.: Peptic Ulcer in Hemophiliac Treated by Gastrectomy , Lancet 1:749 ( (April) ) 1955. 10. Gross, J. D.; Hartmann, R. C., and Graham, J. B.: Splenectomy in Hemophilia , Bull. Johns Hopkins Hosp. 100:223 ( (May) ) 1957. 11. Young, J. D., and Sacks, M. S.: Prostatectomy in a Hemophiliac , J. Urol. 78:644 ( (Nov.) ) 1957. 12. Pitney, W. K.: The Management of the Patient with a Hemostatic Defect During the Period of Surgery , M.J. Australia 1:65-68 ( (Jan.) ) 1959. 13. Adams, J. F.; Timbury, G. C., and Mellon, J. P.: Appendicitis in a Hemophilic , Scottish M.J. 2:328 ( (Aug.) ) 1957. 14. Westlin, W. F., Jr.; Mills, S. D., and Owen, C. A., Jr.: Current Status of Hemophilia Problem , Minnesota Med. 41:705 ( (Oct.) ) 1958. 15. Parks, A. G., and Wass, S. H.: Hemophilia with Small Bowel Obstruction Due to an Ileal Hematoma , Proc. Roy. Soc. Med. 149:933 ( (Nov.) ) 1956. 16. Desmond, A. M.: Hemophilia: Gastric Ulcer; Hematemesis; Emergency Gastrectomy , Proc. Roy. Soc. Med. 48:523 ( (July) ) 1955. 17. Israels, M. C. G.; Lempert, H., and Gilbertson, E.: Hemophilia in the Female , Lancet 1:1375 ( (June) ) 1951. 18. Hartmann, J. R., and Diamond, L. K.: Hemophilia and Related Hemorrhagic Disorders , Practitioner 178:179 ( (Feb.) ) 1957. 19. van Creveld, S.; Hoorweg, P. G., and Stijn, R. W.: Surgical Intervention in Cases of Hemorrhagic Diathesis , Arch. chir. neerl. 9:263-273, 1957. 20. deBruijne, J. I.; van Creveld, S., and Stiendijk, R.: Surgical Treatment of Pyloric Stenosis in an Infant with Hemophilia A , J. Pediat. 51: 698-703 ( (Dec.) ) 1957. 21. Pieper, G. R.; Perry, S., and Burroughs, J.: Surgical Intervention in Hemophilia , J.A.M.A. 170:33 ( (May) ) 1959. 22. Brown, I. W.; Smith, W. W., and House, R. M.: Surgical Management in Hemophilia and the Hemophilioid Diseases , Ann. Surg. 149:721 ( (May) ) 1959.Crossref 23. Meynell, M. J., and Tudor, R. W.: Successful Partial Resection of the Small Bowel in the Case of Hemophilia , Brit. J. Surg. 44:182 ( (Sept.) ) 1956.Crossref

Journal

Archives of SurgeryAmerican Medical Association

Published: Dec 1, 1960

References