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PREOPERATIVE PREPARATION ANDANAESTHETIC MANAGEMENT OFA PATIENT WITH HAEMOPHILIA A

CHANDRAKANT P GOSAVI*, LS CHAUDHARI**,RASHMI PODDAR***
*Registrar, KB Bhabha Hospital, Kurla - 400 070; **Prof. in Anaesthesia, KEM Hospital, Parel, 400 012; ***Anaesthetist, Lotus Eye Hospital, VN Desai Hospital, Mumbai.

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This report describes successful management of 27 year old haemophiliac patient who presented with right shaft femur fracture. Shortly before surgery he received 2500 units of factor VIII. There was no excessive blood loss intraoperatively. Postoperatively patient was supplemented with factor VIII. On 15 postoperative day, test for inhibitors to factor VIII was positive. Patient was started on cryoprecipitate. Seven days later on patient developed wound haematoma. Assay of factor VIII showed 16% of normal activity and no inhibitors to factor VIII were found. Rest of the postoperative course in the ward was uneventful. The literature on the management of patient with Haemophilia A is reviewed and considerations are presented concerning preoperative preparation and anaesthetic management of haemophiliac patient for minor and major surgery.

INTRODUCTION

Prior to the age of modern blood banking, mortality and morbidity of haemophiliac patient was much higher. Complications like recurrent bleeding episodes, painful haemarthroses, and permanent disability secondary to ankylosed joints increased the morbidity. Surgery was impossible, as even dental extractions were life-threatening events. The development of factor VIII concentrates has greatly revolutionized the management of haemophiliac patients.

CASE REPORT

A 27 year old male patient presented with right shaft femur fracture to orthopaedic department. Patient gave history of fall six months back, following which he developed right shaft femur fracture. At the age of five years patient had history of spontaneous haemarthroses for which he was not investigated. Three years back he bled profusely, for which two units of blood were transfused. Patient was investigated and found to be haemophiliac. Patient also gives history of severe headache, which was followed by dysarthria. There was no history of factor VIII transfusion at that time. Family history of the patient revealed his first degree relative to be haemophiliac. After admission to this institute his factor VIII assay was done which showed activity less than 1%. There was no other significant history of any medical disorder or surgical intervention.

On examination patient’s height was 5 feet VIII inches. Weight was 52 kg; his heart rate was 84/min, regular. Arterial pressure was 110/70 mm of Hg. Heart sounds were found to be normal on auscultation. The trachea was centrally placed chest movements were symmetrical. Preoperative investigations were normal with haemoglobin of 11.2 gm% prothrombin time was 14/13, APTT 89/37, mixed APTT 46/37. Test for inhibitors to factor VIII was negative. Patient was posted for open reduction and internal fixation of the fracture.

An 18 gauge intravenous cannula was inserted. Intramuscular injection were avoided. Premedication consisted of atropine 0.6 mg intravenously. As per haematologists advise 2500 units of factor VIII were given half an hour prior to surgery intravenously. Following pre-oxygenation for 5 minutes, anaesthesia was induced with thiopentone 5 mg/kg body weight. Suxamethonium 2 mg/kg was used to facilitate the insertion of 9.5 mm portex cuffed endotracheal tube. Anaesthesia and sedation was provided by injection pentazocine 0.3 mg/kg and injection midazolam 0.03 mg/kg IV. Vecuronium was used to provide adequate muscle relaxation and intermittent positive pressure ventilation was established. Arterial pressure remained around 100-130 mm of Hg throughout the surgery. The entire operation lasted for 4 hours. The total vecuronium administered was 16 mg. Reversal of residual neuromuscular blockade was achieved satisfactorily with neostigmine 0.05 mg/kg and atropine 0.02 mg/kg. Spontaneous ventilation was quickly established. Afterextubation of the trachea the patient was given oxygen by facemask for 10 minutes in the operation theatre. Estimated blood loss during surgery was 700 ml which was replaced by 2 units of fresh blood. Patient was monitored with cardioscope and pulse oximeter. Patient maintained all vital parameters well through out the procedure.

Postoperatively analgesia was provided by injection Tramadol 50 mg IV twice a day. Patient was given chest physiotherapy to avoid respiratory complications. Postoperatively patient was given factor VIII every 12 hourly. Seventh postoperative day patient’s factor VIII activity was 16%. Fifteenth day postoperatively patients Hb was 5.7 gm%. Patient was given four units of blood transfusion to treat anaemia. Test for inhibitors to factor VIII was positive. After seven days patient developed a large haematoma at the site of wound. Factor VIII assay done at that time showed activity of 16% and no inhibitors to factor VIII were detected. Patient was started on 20 units of cryoprecipitate on alternate days.

DISCUSSION

Haemophilia A is a hereditary deficiency of factor VIII (factor VIII:C or antihaemophilic factor) and is the most common and most serious hereditary disorder of coagulation. It is transmitted as an X-linked trait with variable expression; it is ordinarily carried by females who are unaffected. Thus it is a disease of males. The incidence is 1 in 10000 to 25000. [1]

Since the intrinsic limb of the coagulation system is disabled, haemostasis depends upon vascular and extrinsic mechanisms. As a result, bleeding from larger rather than small vessels poses the most serious problem. Furthermore delayed bleeding is a common phenomenon, occurring after an early period of apparent haemostasis, whereupon an inadequately reinforced clot is unable to maintain vascular integrity.

Affected persons have a bleeding tendency that is inversely proportional to factor VIII levels in the body. Patients with factor activity less than 1% are at the risk of spontaneous haemorrhage, most commonly occurring in large joint, nose, soft tissues and urinary tract. Intracranial haemorrhage either extra - or intracerebral is common in severe disease. [2] Our patient complained of an episode of severe headache followed by dysarthria, which could be suggestive of intracranial bleed. Levels of 3-5% may adequately protect the patient from spontaneous bleeding, whereas patients with 10-15% of normal activity may be entirely asymptomatic. [3]

The diagnosis of haemophilia is often made from family history, laboratory-finding such as greatly reduced factor VIII, elevated PTT. The PT, bleeding time, platelet count and clot retraction will be normal in these patients, since none of these tests are dependent upon factor VIII. [4] Patients haemoglobin may be low because of acute or chronic blood loss. When large haematomas are present, the serum bilirubin may be increased. [2] In preparing patients with haemophilia A for surgery, factor VIII levels are routinely raised to approach 100% of normal activity. Same should be maintained for first 3 postoperative days. Day 4 onwards it should be maintained at 80%, from 7th day onwards it is allowed to decline to 40% of normal activity. The formula used to calculate the factor VIII dose is - N = plasma volume (ml/Kg) X weight (Kg) X per cent activity increase where N is the number of units required. Plasma volume is 40 ml/Kg for adults and 45 ml/Kg. [1] Since half-life of factor VIII is about 12 hours, it must be administered twice daily. Cryoprecipitate is next choice of blood product in the management of haemophilia A, which provides 80 units of factor VIII per bag. But as cryoprecipitate contains fibrinogen, serum levels of fibrinogen may rise and increase the risk of bleeding inspite of normal amounts of factor VIII if excessively transfused. [5] Complicating factor in management of haemophilia A is development of antibodies to factor VIII after multiple exposures to factor VIII. [6] Commercially produced factor VIII can be a vector for transmission of hepatitis A and HIV. [7] Surgeons, haematologist and anaesthesiologists should carefully plan the perioperative management of patient with haemophilia.

Intramuscular pre medication is unnecessary and should be avoided. Vascular access itself doesnot cause excessive bleeding and should be appropriate for the proposed procedure. After induction of anaesthesia, extra care should be taken in manipulation or intubation of the airway as it can cause submucosal haemorrhages, which can prove life threatening condition. Nasal intubation should be avoided, as it can prove traumatic and bleeding from the site can lead to aspiration. Care should be taken during positioning of the extremities and pressure points should be padded to prevent intramuscular haematomas or haemarthrosis. [1] Post operatively analgesics such as aspirin and other NSAIDs should not be given as it can predispose patients to gastrointestinal haemorrhage. [8] Patient-controlled analgesia is a safe and effective alternative to intramuscular injections. [1]

Even for minor procedures like dental extraction patient should be admitted in the hospital. Patient should be administered plasma (12 ml/kg), cryoprecipitate 600-800 units, local anaesthesia, intravenous sedation. For closure of the wound absorbable suture material like catgut should be used and local pressure should be applied. [2] The fibrinolytic inhibitors, E amino-caprice acid (EACA) or tranexamic acid are commonly administered to reduce requirement of factor VIII. The vasopressin analogue DDAVP (Desamino-VIII-D-arginine vasopressin), which increases plasma concentration of factor VIII, can be administered intravenously. [2]

REFERENCES

1.de Gruchy’s. Clinical haematology in medical practice, Coagulation disorders. In : Frank Firkin, Colin Chesterman, David Pennington, Bryan Rush. 5th ed. New Delhi : Oxford University Press. 1993; 406-36.

2.William ED, D David Glass. Haematological diseases. In : Katz, Benumof, kadis. Anesthesia and uncommon diseases. Philadelphia 1990; 378-436.

3.Fudeta H, Hashimoto YK, Mori K. Anesthesia in patients with hemophilia A and B. Jpn J Anesth 1976; 25 : 718.

4.Brinkhaus KM. The hemophilias. Chapel Hill, University of North Carolina Press. 1964; 69.

5.Hathaway WE, Mahasondana C, Clarke S, Humbertr JR. Paradoxical bleeding in intensively transfused hemophiliacs. Transfusion 1973; 13 : 6-12.

6.Roberts HR. Hemophiliacs with inhibitors : Therapeutic considerations. N Engl J Med 1981; 305 : 757.

7.Curran JW, Lawrence DN, Jaffe H, et al. Acquired immunodeficiency syndrome (AIDS) associated with transfusions. N Engl J Med 1984; 69 : 310.

8.Simon E, Roux C, More J. Drug combinations in the treatment of pain in hemophiliacs. Bibl Haematologica 1966; 26 : 78.

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