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CASE PRESENTATION

            BY; DR NUR AIZA IDRIS
     MODERATOR: DR NORASLAWATI
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
• 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.
• 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
• 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
• 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 .
• 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.
• 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.
• 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.
• 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.
• Investigation results were:

          Hemoglobin            11.0
            TWBC                12.0
            Platelet            199
        Factor VIII assay       20%


• GSH was converted to GXM.
• 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
• 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.
• 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.
• 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.
• 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.
• 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
• 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.
DISCUSSION
PART 1 – ANAESTHESIA IN
HEMOPHILIAC PATIENT
Problem – based learning
•   Medical disease (basic)
•   Preoperative evaluation and preparation
•   Intraoperative Management
•   Postoperative Management
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.
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?
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?
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?
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?
ANSWERS
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
The severity of bleeding manifestations in hemophilia is
              generally correlated with the
  clotting factor level as shown in the following table.
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.
• 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.
Hemophilia
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
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
• 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%
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
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
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
• 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.
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.
• 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.
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
• 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.
• 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
• 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.
• 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.
• 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
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.
Hemophilia
Hemophilia
PART 2 – ANAESTHESIA AND POST
   TONSILLECTOMY BLEEDING
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%.
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?
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
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
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.
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
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.
• 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.
• 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.
THANK YOU !!!

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Hemophilia

  • 1. CASE PRESENTATION BY; DR NUR AIZA IDRIS MODERATOR: DR NORASLAWATI
  • 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.
  • 20. PART 1 – ANAESTHESIA IN HEMOPHILIAC PATIENT
  • 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.
  • 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.
  • 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.