Introducion.

1. Emergency surgery in Haemophilia sufferers - Factors and considerations. Dr Stephen Smith, Dr Dermot Kelly, Dr MuhammedMukhtar.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. 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. 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. • 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 • Surgery safe if factor VIII levels > 50% normal • Beware: Spinals, Epidurals, NSAID’s, IM injections, ↓ Na+ 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. • References: • Manucci PM, Tuddenham EG -The haemophilias- from royal genes to gene therapy. NEngJMed 2001; 344, 1782-4. • 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.