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Surgery in Bleeding disorders- A challenging problem to all surgeons


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Surgery in patients with bleeding disorders like hemophilia is a nightmare to any surgeon. They must have an working knowledge of how to deal these patients in this challenging situation.

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Surgery in Bleeding disorders- A challenging problem to all surgeons

  1. 1. Surgery in Bleeding Diathesis
  3. 3. Surgery In Bleeding Diathesis Plan Introduction Physiology Of Hemostasis Preop Evaluation Cases Managed Common Bleeding Disorders Surgery In Hemophiliacs Clinical Decision Making
  5. 5. Physiology of Hemostasis Platelet Activities Vessel wall Platelet Fibrinogen GP1b Receptor Gpi ib -iii a Complex Receptor VWBF Endothelium
  6. 6. Platelet membrane phospholipids inhibits Activates Phospholipase A2 Arachiodonic Acid Fatty acid Cyclooxygenase Cyclic Endoperoxides:- PGG2 PGH 2 Thromboxane-A2 C AMP Thrombin Collagen or ADP Aspirin Thromboxane Synthetase inhibits
  7. 7. Coagulation factors I. Fibrinogen II. Prothrombin III.Tissue factor IV.Ionised calcium V. Proaccelerin VII.Proconvertin VIII.Antihemophilic factor(AHF) IX.Christmas factor X. Stuart prower factor XI.Plasma thrombo plastin antecedent XII.Hageman factor XIII.Fibrin stabilising factor
  8. 8. INTRINSIC SYSTEM HMWK XII XII a XI XI a EXTRINSIC SYSTEM IX IX a+VIII X Xa +V VII Ca TF Prothrombin Thrombin Fibrinogen Fibrin XIII XIIa Stable fibrin clot
  9. 9. Plasminogen Stretokinase Plasminogen Damaged cells Tissue plasminogen Activator Prourokinase Urokinase Contact activation slow XII XIIa t-PA + Fibrin Plasmin Proactivator Activator t-PA Inhibitor Rapid Enhancement Fibrinogen Fibrin Factors V VIII Fibrinogen degradation products Fibrin degradation products Destruction by Proteolysis EACA Alpha2-antiplasmin
  10. 10. PRE OP EVALUATION Thorough personal medical history, family history drug ingestion history PHYSICAL EXAM: SKIN Petechiae thrombocytopenia Ecchymoses Hematomas platelet dysfunction JOINTS Hemarthrosis severe coagulation defect ORGANO MEGALY Hepatomegaly coagulopathy of cirrhosis Spleenomegaly possible thrombocytopenia
  11. 11. LAB SCREENIG TESTS Test Normal range Abnormalities Platelet Count 150-250,000/dl Thrombocytopenia Thrombocytopenia Thrombocytopathy Extrinsic system, factor VII Intrinsic system,factors VIII,IX,XI,XII Hypofibrinogenemia, heparin Bleeding Time 2- 8 minutes Prothrombin Time 12-14 seconds Partial Thromboplastin 30-40 seconds Time Thrombin time 12-20 seconds Fibrin Degradation 103gm/ml DIC,fibrinolysis product assay
  12. 12. CCAASSEE NNOO::11 2 Yrs old 1st born male child Was brought with obstructed RIH H/O Epistaxiseasy bruisability Ear boring ceremony resulted in prolonged bleeding Emergencyso no extensive workup was done
  13. 13. CASE NO:1 Basic Screening Tests Hb-----11Gms Platelets----2 Lakhs/cmm Bleeding Time--- 15 mts Clotting Time----- 13 mts Prothrombin Time--- 12 sec PTT------------------- 39 Sec Blood group------B+ve
  14. 14. CASE NO:1 Contd Only B.T. was prolonged Provisional Diagnosis:Functional Platelet problem---------Thrombocytopathy Emergency Herniotomy with 200 ml of fresh whole blood Readmitted after 10 days for prolonged bleeding from a small cut to upper lip Managed by multiple WB transfusions
  15. 15. CASE NO:1 Contd Hematological Workup • Hb--------11.5Gms • PCV------36% • Platelets-----2.00.000/cmm • TC------12.000/Cmm • DC------N 33 L 43 E 18 M 4 • Bleeding Time----15 mts
  16. 16. CASE NO:1 Contd Hematological Workup Clotting Time--------13 mts Clot Retraction--------Nil Clot Lysis--------------Nil Plasma Fibrinogen---280 mgms Factor xiii activity----normal PT---------12 Sec PTT-------40Sec
  17. 17. CASE NO:1 Contd • PLATELET FUNCTION TESTS -Invivo Platelet Adhesion—10.8% -ADP Aggregation------Absent -Aggregometer Studies -ADP-------No Aggregation -Collagen-----No Aggregation • Thrombin Time---------8sec • Blood group-----------B+ve
  18. 18. CCAASSEE NNOO::11 CCoonnttdd •• NNoorrmmaall PPllaatteelleett ccoouunnttmmoorrpphhoollooggyy •• NNoorrmmaall CClloottttiinngg tteessttss •• PPrroolloonnggeedd BBlleeeeddiinngg TTiimmee •• AAbbsseenntt PPllaatteelleett AAggggrreeggaattiioonn wwiitthh AADDPP aanndd CCoollllaaggeenn •• AAbbsseenntt CClloott RReettrraaccttiioonn •• GGLLAANNZZMMAANN``SS TTHHRROOMMBBAASSTTHHEENNIIAA
  19. 19. CASE NO:2 11Yrs old boy , no previous H/O bleeding underwent Interval Appendicectomy Excessive bleeding from OP site immediate postop B.T.---prolonged,C.T.----Normal, Platelet count--------Normal Since no Platelet concentrate was available,was treated with multiple WB transfusions
  20. 20. CASE NO:3 • 4Yrs old boy came with feverpain abdomen • O/E Abdomen---Generalised tenderness and rigidity • AXR-Erect---Gas under diaphragm + • Patient known Hemophiliac • Laparotomyileal perforation closure was done with 1 unit of AHF • On 2nd POD –oozing from the wound,one more unit of AHF Was given
  21. 21. CASE NO:4 5Yrs old boy with H/O fall over cycle bar reported with retention of urine Perineal HematomaExtravasation of urine + With difficulty catheter was passed into the bladder Multiple Perineal incisions were made
  22. 22. CASE NO:4 Contd Next day –profuse bleeding from the perineal incision sites On subsequent interrogation parents came out with H/O previous bleeding episodes Child was a Hemophiliac He was treated with AHF repeated fresh WB transfusions
  23. 23. Bleeding Disorders Commonly Encounter By Surgeons Thrombocytopenia Thrombocytopathy Hemophilia- A Hemophilia- B- Christmas disease Von Willebrand Disease Disseminated Intravascular Coagulation Liver Disease– Factor vii x deficiency
  24. 24. THROMBOCYTOPENIA Low Platelet Count Acquired or Inherited Purpura Ecchymosis----- superficial bleeding Labs:B.T P.C PT/PTT- N Treatment--- Platelet Concentrates
  25. 25. THROMBOCYTOPATHY Functional Platelet Abnormality Acquired due to drugs like Aspirin and Uremia Inherited-----Bernard Soulier Syndrome due to defective Platelet adhesion, Glanzman`s Thrombasthenia due to defective Platelet aggregation
  26. 26. THROMBOCYTOPATHY EcchymosisEpistaxis------ Superficial Hemorrhage Labs:B.T P.C—N, PT/PTT---N Treatment---Platelet Concentrates
  27. 27. HEMOPHILIA----A Factor viii procoagulant deficiency Sex linked recessive inheritance Deep tissue bleedhemarthrosis Labs:B.T,P.C,P.T---N;PTT-- Specific Factor Assays Treatment:AHF 1U/Kg raise the level by 2%.For 5Kg child 250u for 100% raise
  28. 28. Hemophilia-B—Christmas Disease Factor ix deficiency Sex linked recessive inheritance Deep tissue bleedHemarthrosis Labs:B.T,P.C,P.T—N,PTT-- Specific Factor Assays Treatment:Factor ix Concentrates or FFP
  29. 29. Von Willebrand`s Disease Factor viii vwf deficiency Autosomal Dominant Inheritance Epistaxis,easy bruising prolonged bleeding from dental extraction Labs:B.T P.C—N,P.T—N,PTT-- Specific Factor Assays Treatment: FFP or Cryoprecipitate
  30. 30. Disseminated Intravascular Coagulation—(D.I.C) Thrombohemorrhagic disorder Acquired:Abruptio placenta,Septic abortion,Toxemia,Gram-ve sepsis,snake bite,Massive tissue injury etc Activation of Coagulation Cascade— Microthrombi throughout microcirculation Consumption of Coagulation factors,Platelets and Fibrin Activation of Fibrinolytic System
  31. 31. Disseminated Intravascular Coagulation--------contd Mucosal bleed,Ecchymosis,oozing from wound sites Labs:B.T P.C PT/PTT T.T F.S.P Fragmented RBCs + Treat the underlying cause FFPPlatelet Concentrates Role of Heparin is controversial
  32. 32. LLIIVVEERR DDIISSEEAASSEE Acquired Factor vii x deficiency Epistaxis,MenorrhagiaHemarthrosis Labs:B.T—N,P.C—N, Factor x—PT/PTT Factor vii----P.T P.T.T----N.,Specific Factors Assay Treatment-------FFP
  33. 33. Surgery In Hemophiliacs Minor Surgery Give dose calculated to bring patient’s plasma level to 100% 1 hour prior to surgery(50 units/kg q12h) Maintain plasma level above 60% for 4 days Maintain plasma level above 20% for subsequent 4 days Assay daily prior to dose
  34. 34. Surgery In Hemophiliacs Major Surgery Give dose calculated to bring patient’s plasma level to 100% 1 hour prior to surgery(50 units/kg q12h) Maintain plasma level above 60% for 4 days Maintain plasma level above 40% for subsequent 4 days or until all drains sutures are removed Assay daily prior to dose
  35. 35. Surgery IInn HHeemmoopphhiilliiaaccss OOrrtthhooppeeddiicc SSuurrggiiccaall PPrroocceedduurreess •• GGiivvee ddoossee ccaallccuullaatteedd ttoo bbrriinngg ppaattiieenntt’’ss ppllaassmmaa lleevveell ttoo 110000%% 11 hhoouurr pprriioorr ttoo pprroocceedduurree ((5500 uunniittss//kkgg qq1122hh)) •• MMaaiinnttaaiinn ppllaassmmaa lleevveell 8800%% ffoorr 44 ddaayyss •• AAssssaayy ddaaiillyy pprriioorr ttoo ddoossee •• MMaaiinnttaaiinn ppllaassmmaa lleevveell 4400%% ffoorr 44 ddaayyss
  36. 36. Surgery In Hemophiliacs Orthopedic Surgical Procedures If patient is casted, discontinue replacement until rehabilitation program is begun If not casted, maintain above 20 % for ambulation For rehabilitation program ,maintain 10% for three weeks
  37. 37. Clinical DDeecciissiioonn MMaakkiinngg CCaassee NNoo::1 40yrs old man c/o bleeding PR Colonoscopy revealed single polyp in descending colon Colonoscopic snare polypectomy was done Postop profuse bleeding+ Labs:only B.T Diagnosis Treatment ?
  38. 38. Clinical Decision Making Case No:2 3 months old congenital Biliary Atresia baby Was brought with purpuric skin lesionsoozing from gums Labs:PTPTT Diagnosis Treatment ?
  39. 39. Clinical Decision Making Case No:3 3 days old baby was brought with H/O hemetemesis melena O/E Well baby Labs: Hb –N;B.T,P.C,PT,PTT----N. Diagnosis--?
  40. 40. Clinical Decision Making Case No:4 • 60 yrs old man a case of ESRD on chronic hemodialysis • Called to see him for profuse oozing from puncture site @ AV fistula • Labs:B.T,P.C,P.T---N,PTTTT • Diagnosis and Treatment?
  41. 41. Clinical Decision Making Jump to first page Case No:5 12 yrs old girl on chronic hemodialysis for ESRD Uremia developed because of temporary interruption of dialysis Developed brisk epistaxis Labs:BUN-164 mgms, BT20mts Diagnosis Treatment?
  42. 42. Clinical Decision Making Case No:6 50 yrs old lady with severe burns injury became febrile toxic Epistaxis, Hemetemesis melena Wound Swab--Gram –ve sepsis Labs:BT PC PTPTT TT Diagnosis Treatment?