Transcript Emergency surgery in Haemophilia sufferers

Emergency surgery in Haemophilia sufferers - Factors
and considerations.
Dr Stephen Smith, Dr Dermot Kelly, Dr Muhammed Mukhtar.
Royal Victoria Eye and Ear Hospital, Dublin.
Introducion.
A 53 year old man with a mallampati score of 2 and
haemophilia type A, presented for emergency vitrectomy.
General anaesthesia was complicated by an unanticipated
difficult airway and coagulation problems.
Surgery & the postoperative course were uncomplicated. Fluid intake
was restricted to 1.5L/24 hours to minimise the risk of hyponatraemia.
NSAID’s were avoided.
Conclusion
Haemophilia A is an X linked recessive disorder and is the commonest
inherited coagulation disorder in Ireland.
Aside from Type A haemophilia (diagnosed following excess
bleeding after a tonsillectomy), the patient had no significant
medical problems. Airway assessment revealed a mallampati
score of 2 and a thyromental distance of 6cm with good
mouth opening, dentition and range of neck movement.
Coagulation Management.
Management was conducted in liaison with the National
coagulation laboratory at St James’ Hospital.
Factor VIII and fibrinogen levels were measured pre and post
infusion of DDAVP at a dose of 0.3mcg/kg. This was infused over
30 mins in 50 mls of normal saline. Pre infusion factor VIII level was
approximately 5% of normal and rose to 57% post infusion of
DDAVP.
Surgery is safe to proceed with minimal bleeding risk when factor
VIII levels are greater than 50 % normal. U/E for serum sodium was
checked pre infusion and again 1 day post op as hyponatraemia
may develop due to DDAVP administration.
In case of bleeding, transexamic acid 1g iv TDS was to be given,
which acts by inhibiting fibrinolysis, while awaiting recombinant
factor VIII from laboratory in St James’. A fall in serum sodium from
138 to 131, pre and post infusion respectively was observed.
Factor VIII deficiency results in fibrin deficient clots, prolonged
coagulation and clot instability.¹ The disease is classified by factor VIII
levels into mild, moderate & severe.
DDAVP corrects the coagulation abnormality by stimulating release of
factor VIII from endothelial cells, but can alter serum electrolyte levels –
potentially inducing hyponatraemia. It is used in mild & moderate cases
(and also in von Willebrand’s disease) but severe Haemophilia A requires
factor VIII administration (pooled or recombinant). Response diminishes
with successive doses of DDAVP so continuous factor VIII level monitoring
is required. With repeated transfusions, antibodies may develop which
can render factor VIII inactive. In this situation, a recombinant factor
seven may be administered.
Neuraxial anaesthesia (spinal & epidurals) are probably best avoided,but
can be performed in Haemophilia A with corrected factor VIII levels.²
With corrected levels, other nerve blocks may also be undertaken.
Caution is needed with intramuscular injections.
Major haemorrhage, whilst rare, should be treated by correction of the
factor VIII levels using factor VIII infusions and guided by APTT and factor
VIII levels.
Haemophilia A
• X linked recessive - affects 1 in 5,000 in Ireland
• Classified on VIII levels: Mild > 5%, Moderate >1%, Severe 1%
• APTT , normal PT & bleeding time.
• Treatment  factor VIII levels with DDAVP &/or factor VIII
Airway and Anaesthesia.
Following standard induction (including atracurium), laryngoscopy
revealed a grade IV Cormack and Lehane view. Despite McCoy
laryngoscope use, blinded intubation attempts with a bougie proved
unsuccessful. A size 4 LMA was inserted and ventilation was possible.
A fibreoptic bronchoscope with mounted Aintree catheter was passed
through the mask and vocal cords. The scope and LMA were removed
and an ETT ‘railroaded’ over the catheter. Despite minimal trauma,
generalised oozing from the airway was evident. Satisfactory tube
placement was confirmed & the patient was ventilated easily.
• Surgery safe if factor VIII levels > 50% normal
• Beware: Spinals, Epidurals, NSAID’s, IM injections, ↓ Na+
References:
1)Manucci PM, Tuddenham EG -The haemophilias- from royal genes to gene therapy. NEngJMed
2001; 344, 1782-4.
2)Dhor, Abamovitz, DiMichele,Gibb, Gadalla. Management of pregnancy in patient with severe
haemophilia. BrJAnaesth 2003; 91:432-45.
Acknowledgements:
Dr James O’ Donnell, Consultant haematologist, St james’ Hospita, Dublin.