Bleeding perioperative bleeding prevention and treatment in patients with congenital bleeding disorders

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

2013 ESA Guidelines for the Management of Severe Perioperative Bleeding (DO NOT EDIT)[1]

Patients with Congenital Bleeding Disorders

von Willebrand Disease

Class 1
"1. We recommend the use of bleeding assessment tools for predicting the perioperative risk of bleeding. (Level of Evidence: C)"
"2. We recommend that patients with vWD be managed perioperatively in collaboration with a hematologist. (Level of Evidence: C)"
"3. We recommend desmopressin as a first-line treatment for minor bleeding/surgery in patients with vWD, after a trial testing. The regimen is specified by published guidelines. (Level of Evidence: C)"
"4. We recommend replacement of VWF with plasma-derived products for major bleeding/surgery. Treatment regimens are specified by published guidelines. (Level of Evidence: C)"
Class 2
"1. We suggest that if vWD is suspected preoperatively, the patient be referred to a hematologist for assessment and planning of the intervention. (Level of Evidence: C)"
"2. We suggest that antifibrinolytic drugs be used as hemostatic adjuncts. Treatment regimens are specified by published guidelines. (Level of Evidence: C)"
"3. We suggest that platelet transfusion may be used only in case of failure of other treatments. (Level of Evidence: C)"

Platelet Defects

Class 1
"1. We recommend the use of a bleeding assessment tool for predicting the perioperative risk of bleeding. (Level of Evidence: C)"
"2. We recommend that patients with severe inherited platelet disorders should be managed perioperatively in collaboration with a hematologist. (Level of Evidence: C)"
"3. We recommend that rFVIIa treatment should be considered in patients with Glanzmann thrombasthenia undergoing surgery. (Level of Evidence: C)"
"4. We recommend against routine platelet transfusion in patients with inherited platelet disorders. (Level of Evidence: C)"
Class 2
"1. We suggest referring the patient to a hematologist for assessment and planning of the intervention if inherited platelet defects are suspected preoperatively. (Level of Evidence: C)"
"2. We suggest preoperative hemostatic correction in patients with inherited platelet disorders. (Level of Evidence: C)"
"3. We suggest desmopressin be used to prevent/control perioperative bleeding in patients with inherited platelet defects. (Level of Evidence: C)"
"4. We suggest antifibrinolytic drugs be used as hemostatic adjuncts in procedures involving patients with inherited platelet defects. (Level of Evidence: C)"

Hemophilia A and B

Class 1
"1. We recommend that hemophilia patients should be referred preoperatively to a hematologist for assessment/intervention. (Level of Evidence: C)"
"2. We recommend that surgery in hemophilia patients should be performed in specialised centres with expertise in coagulation disorders. (Level of Evidence: C)"
"3. We recommend adequate perioperative replacement therapy to ensure safe surgery in hemophilia patients. (Level of Evidence: C)"
"4. We recommend either recombinant products or plasma-derived concentrates for perioperative replacement therapy in hemophilia patients. (Level of Evidence: C)"
Class 2
"1. We suggest that perioperative replacement therapy (target factor level and duration) in hemophilia patients follows published guidelines. (Level of Evidence: C)"
"2. We suggest that coagulation factors be given perioperatively by continuous infusion. (Level of Evidence: C)"
"3. We suggest either rFVIIa or activated PCCs for hemophilia patients with inhibitors. (Level of Evidence: C)"
"4. We suggest antifibrinolytic drugs as perioperative adjunct therapy in hemophilia patients. (Level of Evidence: C)"
"5. We suggest individualized perioperative thromboprophylaxis in hemophilia patients. (Level of Evidence: C)"

Factor VII Deficiency

Class 2
"1. We suggest that rFVIIa be used in perioperative bleeding due to inherited FVII deficiency. (Level of Evidence: C)"
"2. If rFVIIa is given to control perioperative bleeding in inherited FVII deficiency, we suggest lower doses than in hemophilia patients. (Level of Evidence: C)"

Rare Bleeding Disorders

Class 1
"1. We recommend that patients with rare bleeding disorders should be referred preoperatively to a hematologist for assessment/intervention. (Level of Evidence: C)"
"2. We recommend that surgery in patients with rare bleeding disorders should be carried out in consultation with a hematologist with experience in factor deficiencies. (Level of Evidence: C)"

Sources

  • 2013 ESA Guidelines for the Management of Severe Perioperative Bleeding[1]

References

  1. 1.0 1.1 Kozek-Langenecker, SA.; Afshari, A.; Albaladejo, P.; Santullano, CA.; De Robertis, E.; Filipescu, DC.; Fries, D.; Görlinger, K.; Haas, T. (2013). "Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology.". Eur J Anaesthesiol. 30 (6): 270–382. PMID 23656742. doi:10.1097/EJA.0b013e32835f4d5b. 

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