Surgery in patients with bleeding disorders requires careful evaluation and management to minimize bleeding risks. The document discusses: 1) The physiology of hemostasis including the vessel wall, platelet, and coagulation responses. 2) Evaluation of patients including history, exam, and screening tests to identify bleeding disorders. 3) Four case examples of patients who bled excessively during or after surgery, who were found to have thrombocytopathy, hemophilia, or other disorders. 4) Common bleeding disorders surgeons may encounter including thrombocytopenia, thrombocytopathy, hemophilia A/B, von Willebrand disease, DIC, and liver disease. 5) Guidelines

1. Surgery in Bleeding
Diathesis

2. DR.B.SELVARAJ,MS,Mch,FICS,
NEONATAL & PEDIATRIC
SURGEON
PONDICHERRY INSTITUTE OF
MEDICAL SCIENCES
PONDICHERRY-605014
INDIA

3. Surgery In Bleeding Diathesis
Plan
Introduction
Physiology Of Hemostasis
Preop Evaluation
Cases Managed
Common Bleeding Disorders
Surgery In Hemophiliacs
Clinical Decision Making

6. Physiology Of
Hemostasis
FOUR COMPONENTS
VESSEL WALL RESPONSE
PLATELET ACTIVITIES
COAGULATION CASCADE
FIBRINOLYTIC SYSTEM

8. Physiology of Hemostasis
Platelet Activities
Vessel wall
Platelet
Fibrinogen
GP1b Receptor
Gpi ib -iii a
Complex Receptor
VWBF
Endothelium

9. 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

10. 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

11. 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

12. 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

13. 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

14. 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

16. 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

17. 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

18. 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

19. 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

20. 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

21. 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

22. 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

23. 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

24. 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

25. 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

26. 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

27. 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

28. THROMBOCYTOPENIA
Low Platelet Count
Acquired or Inherited
Purpura Ecchymosis----- superficial
bleeding
Labs:B.T P.C PT/PTT- N
Treatment--- Platelet Concentrates

29. 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

30. THROMBOCYTOPATHY
EcchymosisEpistaxis------ Superficial
Hemorrhage
Labs:B.T P.C—N, PT/PTT---N
Treatment---Platelet Concentrates

31. 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

32. 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

33. 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

34. 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

35. 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

36. 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

38. 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

39. 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

40. 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

41. 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

42. 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 ?

43. 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 ?

44. 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--?

45. 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?

46. 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?

47. 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?

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