Yes, intubating the trachea of a patient with hemophilia could potentially cause bleeding complications. However, with appropriate preoperative factor replacement therapy and hemostatic precautions during the procedure, endotracheal intubation can be performed safely in a patient with hemophilia.
Some special considerations include:
- Ensure adequate factor VIII levels prior to intubation (aim for >50% of normal)
- Use a well-lubricated, soft endotracheal tube to minimize trauma
- Use gentle technique and avoid multiple attempts during laryngoscopy and intubation
- Apply cricoid pressure during laryngoscopy to protect the airway
- Suction carefully after intubation
2. CASE PRESENTATION
• 4 years old boy
• Known case of
– Hemophilia A (diagnosed since born, on
occasional factor VIII transfusion , last transfusion
was in April 2011)
– HbE/ Beta- thalasemia trait (last blood transfusion
was in April 2011)
– Under Blood Bank HSNZ follow up
3. • Positive family history of thalasemia and
hemophilia.(His brother also had been diagnosed
to have hemophilia and thalasemia + mother is
thalasemia trait and hemophilia trait)
• Previously he had multiple admission to hospital
due to UGIB and recurrent adenotonsillitis with
no obstructive symptoms.
• He was electively plan for adenotonsillectomy for
recurrent adenotonsillitis by ORL team after they
seen him in the clinic.
4. • Patient was seen at anaesthetic clinic for
preoperative assessment.
• Patient was alert, comfortable. Lungs and CVS
examination revealed no abnormality. Liver and
spleen was just palpable. His airway was normal.
He was classified as ASA 2.
• The investigation results were:
HB 8.8
TWBC 9
PLATELET 365
APTT 106.6
INR 1.13
PT 13.5
5. • The anaesthetic plan were:
– Technique of anaesthesia: GA with IPPV
– To repeat FBC and coagulation profile on
admission
– To confirm factor VIII prior op with blood bank
– To discuss with blood bank regarding timing for
factor VIII transfusion prior op
– Aim hb>8 g/dl
6. • The day before operation, he was seen again by anaest
MO for preoperative evaluation. No new complaint. His
investigation results were:
HB 8.1
TWBC 7.5
PLATELET 302
APTT NULL
INR 0.99
PT 13.5
• Case was noted to the specialist incharge. Syrup
phenergen 5mls ON and when OT call was ordered for
premedication. Blood bank was also informed .
7. • On the day of op, patient was alert and
comfortable.
• BP was 104/71 with pulse rate 110.
• Patient was induced with
– IV fentanyl 15mcg
– Iv propofol 40mg
– IV rocuronium 8mg
• Intubation done using ETT size 4.5mm. CL grade 2
and it was uneventful.
8. • 2 vial of Factor VIII was transfused 30min prior to op.
• Intraoperative finding was:
– Adenoid enlarged – curettage done
– Tonsil 2+/2+ - removal done
– Estimated blood loss was minimal
• Able to extubate well. Breathing effort was good. No
stridor noted post extubation.
• After 30min observed in recovery room , patient was
discharged to ward.
• Another 1 vial of Factor VIII was transfused post
operatively and was planned for 8 hourly transfusion
for 3 days.
9. • D2 post adenotonsillectomy, patient developed
stridor and occasional tachypnea. On
examination revealed huge blood clot in his
mouth and patient was unable to expel it.
• Emergency exploration under GA was planned.
Case was notified to GA oncall and discussion
with blood bank was done.
• Factor VIII infusion 250IU was given 2 vials preop
with expected coverage 70%-100%.
• Random FVIII assay was send.
• He was planned for another factor VIII infusion
and iv tranexemic acid post operatively.
10. • Preoperative assessment was done in OT. On
examination, patient was alert and conscious.
Not in respiratory distress .There was old blood
noted from both nostrils. No active bleeding seen
from oral cavity. Lungs examination showed good
and equal air entry. Hemodynamically stable.
• Case was reviewed in OT with specialist on call
and planned to proceed op with ICU back up post
op.
11. • Investigation results were:
Hemoglobin 11.0
TWBC 12.0
Platelet 199
Factor VIII assay 20%
• GSH was converted to GXM.
12. • Patient was induced with
– IV fentanyl 15mcg
– Iv propofol 30mg
– IV scoline 15mg
• Intubation done using ETT size 4.5mm anchored
at 14cm and it was uneventful.
• Intraoperatively, hemodynamically stable.
• Findings were:
– Big blood clot at superior pole of right tonsil bed
– Blood clot at lower pole of left tonsil bed
– Both obstructed the airway
– Removal of blood clot done – minimal bleeding
present and was secured.
– EBL: minimal
13. • Post operatively, able to extubate patient. No
active bleeding from mouth.
• Post extubation, noted noisy breathing. Decided
to admit ICU for close observation.
• Factor VIII infusion 250 IU was given immediately
post op (as planned before)
• Then, he was admitted to ICU for close
observation. Oxygen therapy given at 3L/min.
Patient was kept nil by mouth with IVD
maintenance at 48mls/H. Planned for
reintubation if patient developed worsening
respiratory distress.
14. • In ICU, factor VIII infusion 250 IU was continued
as planned (given 8 hourly). IV tranexemic acid
was given for 1 day.
• Factor VIII assay was repeated and the result was
60%.
• After 1 day in ICU, condition improved and he
was transferred to general ward .
• Day 3 post exploration, blood clot evacuation and
hemostasis ; patient again had bleeding from
nose and oral cavity.
15. • Factor VIII infusion 2 vial was given stat and
continued for 6 hourly. Factor VIII assay and
PT/APPT mixing (for inhibitor screening) was sent
immediately.
• Re-exploration was done as emergency. Novo
seven was standby and planned to wean in ICU
post operatively. Parents were informed
regarding difficult intubation and post op
ventilation in ICU.
• During intubation , noted huge blood clot
obscuring the vocal cord and at peritonsillar area.
However, intubation was uneventful.
16. • Intra-operative findings was:
– Both tonsilar bed full with blood clots and debris ;
removed with forcep
– Multiple bleeding sites from raw areas
– EBL : 50mls
– Novoseven 1 vial was given intra-operatively.
• Post op, patient was transferred to ICU for weaning as
planned. Infusion factor VIII was continued 6 hourly.
• Since hemostasis not secured despite good factor VIII
coverage , development of fVIII inhibitor is suspected.
APTT/mixing test, factor VIII assay and inhibitory assay
were sent immediately.
17. • The results were:
Investigations 5/6/11 6/6/11
Hb 9.8 5.7
Twbc 12.3 6.7
Platelet 361 249
APTT-mixing test 43.2
Factor VIII assay 1% 1%
Inhibitory assay 8.5 bethesda
unit
• Impression was : Hemophilia A with inhibitor
18. • Day 2 post re-exploration, EUA done.
• Findings:
• IV Novoseven given intraoperatively.
• Patient able to extubate post-op. and was sent
to ICU for close observation.
• His condition improved and T/O to general
ward after 8 hours observation.
21. Problem – based learning
• Medical disease (basic)
• Preoperative evaluation and preparation
• Intraoperative Management
• Postoperative Management
22. Medical disease (basic)
1. What are the differences between hemophilia A, B, and
C?
2. Describe the physiologic events that occur following
endothelial interruption in the blood vessel.
3. What prevents the extension of a clot beyond the site of
injury?
4. Describe extrinsic, intrinsic, and common coagulation
cascade.
23. Preoperative evaluation and
preparation
1. What should you ask in history taking?
2. Describe the various laboratory tests that evaluate the
coagulation cascade and the specific components
measured by each.
3. Describe the levels of factor VIII necessary for hemostasis.
4. What is meant by one unit of factor VIII clotting activity
and how much does one unit of factor VIII clotting activity
per kilogram of body weight increase factor VIII
concentration?
5. How much factor VIII activity is present in fresh frozen
plasma (FFP)? What are the risks associated with
administration of FFP?
24. 6. How is cryoprecipitate prepared? What are the
components in cryoprecipitate? How much factor VIII
activity is present in cryoprecipitate? What are the
indications for cryoprecipitate?
7. What is the role of desmopressin (DDAVP) for
hemostatic management in hemophiliac patient?
8. What is the role of tranxenamic acid in the
perioperative period for the hemophiliac patient?
25. Intraoperative Management
1. Is it safe to administer an intramuscular (IM) injection to
patient before surgery?
2. Would it be safe to intubate the patient's trachea?
3. What special considerations should be taken in choosing
anesthetic drugs for this patient?
4. Does giving blood intraoperatively increase the potential for
the development of factor VIII inhibitors?
5. Can factor VIII be safely administered to patients who have
developed circulating inhibitors?
6. Is it appropriate to suction the endotracheal tube and
oropharynx of this patient before extubation?
26. Postoperative Management
1. What special consideration should be given to
postoperative pain management for this
patient?
2. What is recommendation for plasma factor
levels and duration of replacement to prevent
post op bleeding?
28. Hemophilia A
• factor VIII deficiency
• the most common form, accounting for 85% of all patients
with hemophilia.
• It is an X-linked, recessive disorder
Hemophilia B
• deficient or defective factor IX
• this entity represents 14% of hemophilia patients.
• This X-linked, genetic disorder
Hemophilia C
• deficiency in factor XI
• an autosomal disorder
29. The severity of bleeding manifestations in hemophilia is
generally correlated with the
clotting factor level as shown in the following table.
30. exposes platelets to
glycoprotein receptors
subendothelial
on the platelets to
endothelial break structures (collagens
rapidly adhere to
and other activating
these substances.
proteins)
shape of the platelet is
changed and the
Formation of platelet stimulate platelet
contents of the
plug aggregation
cytoplasmic granule
are released (ADP)
interaction with both Activation of factor
fibrin and thrombin XIII produces cross
fuse the platelet plug polymerization of the
for continued loose fibrin to produce
hemostasis a firm clot.
31. • Localization of coagulation and control of primary hemostasis are
controlled by many factors, including the dynamic tension between
two prostaglandins, thromboxane A2 and prostacyclin.
• Thromboxane A2 is released at the site of vascular injury and
stimulates vasoconstriction, ADP release, and platelet aggregation.
• In contrast, prostacyclin is produced by intact endothelial cells and
prevents platelet aggregation and clot formation.
• In addition, clot localization is maintained by the dilution of
procoagulants flowing in the blood, the removal of activated factors
by the liver, the action of circulating procoagulant inhibitors such as
antithrombin III and protein C, and the release of the serine
protease tissue plasminogen activator (TPA).
• TPA digests fibrinogen as well as factors V and VIII, initiating the
physiologic process of fibrinolysis and resulting in fibrin degradation
(split) products, which are removed by the mononuclear phagocyte
system.
32.
33. Elicit the age of onset and symptoms of coagulation disorder
Obtain a family history of bleeding disorders
Determine hemostatic reponses to previous procedures;any
excessive bleeding and need for blood transfusion
Drug history
Associated medical conditions
34. Specific Components Measured by Different Coagulation Tests
LABORATORY TESTS COMPONENTS MEASURED
Bleeding time Platelet count, vascular integrity
Prothrombin time I, II, V, VII, and X (extrinsic pathway)
Partial thromboplastin time (PTT) I, II, V, VII, IX, X, XI, and XII (intrinsic
pathway)
Thrombin time I, II
35. • The levels of factor VIII necessary for hemostasis are
described in the table below. When life-threatening airway or
neurologic bleeding has occurred, factor VIII level of 100%
should be achieved. Similarly when major surgery is
anticipated, correction to 100% factor VIII level should be
done preoperatively and maintained postoperatively
The Levels of Factor VIII Necessary for Hemostasis
CLINICAL PRESENTATION FACTOR VIII CONCENTRATION (%
OF NORMAL)
Spontaneous hemorrhage 1–3%
Moderate trauma 4–8%
Hemarthrosis and deep skeletal
muscle hemorrhage 10–15%
Major surgery Greater than 30%
36. One unit of factor VIII:C clotting activity is defined as
the amount present in 1 mL of fresh normal, pooled
plasma.
A single unit of factor VIII clotting activity per kilogram
of body weight will increase plasma factor VIII levels
approximately 2%.
Prescribe the factor VIII activity necessary to correct a
70-kg hemophilia patient with 5% factor VIII activity to
95% of normal
37. FFP contains all plasma proteins, including factor
VIII
Factor VIII activity : 0.7 to 0.9 unit of clotting
activity per mL FFP
risks associated with the transfusion of FFP
• similar to any other single-donor blood
product i.e HIV or hepatitis viral transmission
38. Cryoprecipitate is the fraction of plasma that
precipitates when FFP is thawed.
Contains: factor VIII, factor VIII: von Willebrand's
factor, factor XIII, and fibrinogen
Factor VIII activity : 5 and 13 units of factor VIII
clotting activity per mL cryoprecipitate
Indication : hemophilia, von Willebrand's disease,
hypofibrinogenemia, uremic platelet dysfunction
39. • This synthetic analog of the antidiuretic hormone is
used to prepare mild and moderate hemophiliacs for
minor surgery.
• Intravenous DDAVP will rapidly release preformed
factor VIII complex, which leads to a two- to threefold
increase in circulating factor VIII within 30 to 60
minutes of administration. In addition, factor VIII:C and
von Willebrand's factor are released from the
endothelial cells.
• In a patient with mild or moderate hemophilia A or von
Willebrand's disease, this influx of factor VIII may
provide adequate hemostasis for minor elective
surgical procedures.
• The half time of this released factor is approximately
12 hours and repeated administration of DDAVP will
deplete the storage capacity in the endothelial cells.
40. antifibrinolytic agent that competitively
inhibits the activation of plasminogen to
plasmin.
promotes clot stability and is useful as
adjunctive therapy in hemophilia and some
other bleeding disorders.
41. • IM injections can be safely administered to
any patient with a factor VIII activity greater
than 30%.
• Therefore, if appropriate factor VIII correction
has occurred before surgery, an IM injection
would be considered safe.
• However, it is best to avoid and would be
prudent to use an intravenous route.
42. unique challenge for the anesthesiologist because of the
risk of hemorrhage in the tongue or neck, which could
completely compromise the upper airway of the patient
manipulation of the airway during intubation should not
be performed until appropriate replacement factors
have been administered.
Care should be used in placing the mask on the patient
to avoid trauma to the lips, tongue, or face
laryngoscopy should only be attempted following
preoperative factor correction and achievement of
complete muscle relaxation
nasal intubation should be avoided
43. • Coexisting liver disease is a common complication
in the hemophiliac patient because of hepatitis
acquired from previous blood or factor
transfusions.
• As a result, drugs that are metabolized by the
liver should be used with caution in the
hemophiliac patient.
• A balanced intravenous technique might be
preferable to an inhaled anesthetic because of
the reduced hepatic blood flow observed in an
inhaled technique.
44. • Although 10% to 15% of all hemophiliac patients will
develop a circulating inhibitor of factor VIII, there is
no evidence that the development of such an
inhibitor is related to the number of transfusions that
the patient receives.
• As a result, the administration of blood products in
this setting should not be withheld for fear of
inducing an inhibitor response
45. • Occasionally hemophiliac patients develop IgG
antibodies to the deficient factor.
• The inhibitors may be of low titer and transient or of
extremely high titer and very persistent.
• The Bethesda unit of inhibition is defined as the
amount of inhibitory activity in 1 mL of plasma that
decreases the factor VIII level in 1 mL of normal plasma
from 1 to 0.5 units.
• It is almost impossible to overpower a high-titer
inhibitor, but, when life-threatening hemorrhage
occurs, massive doses of factor VIII concentrates or
plasmaphoresis with replacement of factor VIII should
be given and may be of temporary benefit.
• Replacement with large amounts of factor VIII
concentrate may provide temporary hemostasis but
will stimulate an increase in the antibody titer.
46. • Porcine factor VIII is effective in hemophilia A patients with
inhibitors. The porcine factor VIII provides adequate factor
VIII activity in patients with less than 50 Bethesda units of
inhibitor.
• Porcine factor VIII may provide hemostasis because of its
distinct antigenicity, even in the presence of circulating
inhibitor.
• Immunosuppressive therapy is of no value.
• Alternative approaches to therapy of the hemophilic patient
with inhibitors involve the use of other agents such as
activated prothrombin complex concentrated (APCC), which
contain activated vitamin K dependent enzymes as well as
recombined factor VIIa.
• These activated coagulants enter the coagulation cascade
distal to the level of factor VIII and, therefore, bypass the
effects of the inhibitor. Thrombosis is a possible
complication.
47. • Removal of secretions that might be aspirated is
essential before the extubation of any patient.
• However, suctioning of the oropharynx of
hemophiliac patient can trap mucosa in the
suction catheter and result in the formation of an
oral hematoma.
• In a hemophiliac patient, gentle oral suctioning
under direct vision is appropriate to remove all
secretions
48. Avoid any postoperative pain supplements that might
produce a bleeding diathesis in the hemophiliac patient.
Analgesics containing aspirin or nonsteroidal
antiinflammatory drugs (NSAIDs) should be avoided.
Antihistamines and antitussives can inhibit platelet
aggregation and prolong bleeding time.
Narcotic analgesics or acetaminophen-based products
should be appropriately titrated for postoperative pain
management.
49.
50.
51. PART 2 – ANAESTHESIA AND POST
TONSILLECTOMY BLEEDING
52. CASE SCENARIO
• You have been called to see a 5-year-old child who had a
tonsillectomy six hours previously. The child is bleeding
and needs to go back to theatre for haemostasis. When
you arrive on the ward the child is agitated and says he
feels sick. The postoperative blood-loss is reported to be
minimal by the nursing staff.
• On examination he is pale. His pulse is 125/min,
respiratory rate is 25/min, blood pressure 80/40mmHg
and capillary refill time 4 seconds. Stridor was heard.
Patient is using his accessory muscle to breath. His
saturation maintain at 95-100%.
53. Questions
1. What are the specific problems in this case?
2. What are the causes of postoperative stridor?
3. How do you assess hypovolemia in children?
4. What is the immediate management in the
ward?
5. How would you induce this patient and what
drugs would you use?
6. Upon arrival at GOT, the stridor does not
resolved and there is falling SpO2 ; what should
you do?
54. What are the specific problems in this case?
Hypovolaemia
Difficult intubation (bleeding, laryngeal oedema)
Aspiration (blood/food)
Second general anaesthetic
Management of an anxious child/parents
55. Causes of postoperative stridor
Laryngospasm
Paralysis of one or both vocal cords
Laryngeal edema
Extrinsic or intrinsic compression of the airway
Presence of mass, fluid or blood in the airway
Congenital or acquired airway pathology
Residual effect of anaesthetic agents
56. How would you assess hypovolemia?
• tachycardia
Cardiovascular • low blood pressure
• prolonged (>2secs) capillary refill time
• cold
Skin
• mottled skin
• agitation , confusion
Cerebral • drowsiness
• depressed conscious level
Renal • low or absent urine output.
• Tachypnoea then acidotic sighing
Respiratory
respirations.
• low haemoglobin
Laboratory • metabolic acidosis
• high blood lactate.
57. The immediate management
• Maintain the airway and support ventilation
Aim • to treat hypovolemia without delaying
transfer to theatre for haemostasis
1. High flow oxygen via facemask if the child tolerates (10-15 L/min)
2. Apply standard airway support using chin lift or jaw thrust
3. Assess for respiratory distress
4. Assist ventilation with continuous positive airway pressure
5. Assess volume status
6. Obtain intravenous access
7. Send for full blood count, cross match and coagulation.
8. Resuscitate with intravenous fluids and blood products as needed
9. Prepare for theatre
10. Call for senior help
11. Closely monitor the saturation and review the hemodynamic status
58. Induction
• Post-tonsillectomy hemorrhage patients must be
considered to have a full stomach given an unknown but
potentially large amount of blood may be swallowed.
• Adequate fluid resuscitation prior to induction is critical.
This can be performed in the emergency department, the
surgical holding area, or even in the operating room prior
to anesthesia.
• Anesthetic management should entail a rapid sequence
induction with cricoid pressure followed by evacuation of
gastric contents.
• The choice of induction drug should be based on the
patient’s volume status and hemodynamic stability.
59. • If the volume status is deemed borderline or there is rapid
ongoing blood loss, etomidate is a good choice.
• While ketamine may also be safe, remember that
myocardial depression and hypotension can still be seen in
the hypovolemic patient.
• The adequately resuscitated patient will likely tolerate
propofol or a barbiturate.
• The neuromuscular blockers that give the best intubating
conditions in less than 60-90 seconds would be
succinylcholine or rocuronium, however rocuronium may
result in paralysis outlasting the surgical procedure.
60. • Call for help
• Ask for difficult intubation trolley
• Prepare for emergency re-intubation
• Use a smaller sized ETT in case of laryngeal edema
• Remember that LMA may not overcome airway
obstruction if it occurs at the glottic or subglottic level
• If tracheal intubation is difficult and SpO2 remains
low , perform an immediate cricothyrotomy and
begin transtracheal jet ventilation.