Reporte de Caso

Anaesthetic Management of a Haemophiliac patient with Thrombophilia

Mohamad Ommid*, Arun Kumar Gupta**, Abraq Asma***, Showkat Gurcoo****, Ayaz Farooqi*****, Shigufta Qazii******

* Residente de último ano, Departamento de Anestesiología y Cuidado Crítico, SKIMS Soura,. Srinagar, J&K India, ommid76@yahoo.com
** Profesor Asistente, Colegio Rural Medico, Loni,Maharashtra, India.
*** Profesor auxiliar, Departamento de Anestesiologia y Cuidado Crítico, SKIMS Soura, Srinagar, J&K India.
**** Residente de último ano, Departamento de Anestesiologia y Cuidado Crítico, SKIMS Soura, Srinagar, J&K India.
***** Profesor auxiliar, Departamento de Anestesiologia y Cuidado Crítico, SKIMS Soura, Srinagar, J&K India.
****** Profesor y Jefe del Departamento de Anestesiologia y Cuidado Critico, SKIMS Soura, Srinagar, J&K India.

Recibido: julio 26 de 2010. Enviado para modificaciones: agosto 9 de 2010. Aceptado: octubre 10 de 2010.

SUMMARY

Haemophiliac patient are known to have increased mortality and morbidity in the past due to previous non existence of practice of blood banking. Mainly morbidity increased due to the complications arises such as recurrent bleeding episodes, painful haemarthroses and permanent disability secondary to ankylosed joints. This case enlightens the role and impact of undetected changes in Hemophilia A patient with Buerger's disease, highlighting the importance of vigilance on the part of the anesthesiologist, who must ensure the adequacy of hemostatic monitoring in these patients. The clinical presentation of Haemophilia A disease with Buerger's disease is a rare entity and can be challangeable for practicing anaesthesiology.

INTRODUCTION

Haemophilia A is a hereditatry bleeding disorder which shows a spectrum of manifestations ranging from persistent bleeding after minor trauma to spontaneous haemorrhage because of hereditary deficiency of factor VIII (1). Thrombophilia may be defined as hereditary or acquired conditions which predispose individuals to thromboembolic events. Buergers disease is one of the peripheral vascular diseases involving vasculitis. Today it is well known that the inflammatory process characterizing vasculitides activates coagulation factors, inhibits anticoagulant factors, inhibits fibrinolytic processes, increases platelet activity and production and determines endothelial dysfunction (2).

Thrombophilia in buerger's disease is attributed due to hyperhomocysteinaemia. Anaesthetic management of patients with Haemophilia A & Thrombophilia is very challenging & needs to be planned well. Breathtaking advances like stem cell transplant, bone marrow transplant & replacement therapy will increase life expectancy of such patients exposing them to a variety of problems & coming generation of anesthesiologists are more likely to see greater number of such patients in the days to come.

CASE REPORT

A 50 year old male weighing 72 kg known haemophiliac and known hypertensive since 5 years presented with a history of pain in the lower limbs for last 5 years. The pain used to start with walking and subsided with rest. Since last two years the symptoms restarted and with more intensity. The patient also started with bluish discoloration of the left foot toe since last 1 month.

The patient has a history of prolonged and excessive bleeding following minor cuts and bruises. The patient was investigated and diagnosed as a case of Haemophilia A. There was no history of any surgical intervention or factor VIII transfusion. The patient was also diagnosed with hypertension 4 years back and was started on ACE inhibitors.

On examination his heart rate was 74 bpm (beats per minute), regular. Arterial pressure was 150/96 mmhg. Heart sounds were found to be normal on auscultation. Airway was adequate with mallampatti class 1, thyromental distance more than 6.5 cms, interincisor gap more than 4 cms and reasonable body mass index. The trachea was centrally placed. Chest movements were symmetrical. Local examination of the limb revealed diminished to absent pulsations in the Dorsalis pedis, Posterior tibial and popliteal arteries, with gangrenous changes setting in the left little toe. Preoperative investigations revealed hemoglobin of 17.2 g/dl, TLC 15,000 platelet count of 423,000/ microlitre. Coagulogram showed Prothrombin time was 15/12 seconds, INR 1.08, PTI 93.3 % and aPTT of 35/24 seconds. Factor VIII concentration was found to be 17 % of NPP.Test for inhibitors of factor VII was negative. Determinations of antithrombin, protein C, protein S, and plasminogen activities were performed as additional tests for studying the cause of thrombophilia and revealed no deficiency of antithrombin III, protein C, protein S, or plasminogen. After it we have also studied the an-tiphospholipid antibodies which also suggests negative. Homocysteine levels were studied and found to be normal.

Echocardiography revealed Grade I diastolic dysfunction with normal systolic function. Doppler was suggestive of peripheral vascular disease with vessels showing diffuse mild thickening with bi to monophasic flow. Magnetic resonance Angiography showed multifocal bilateral arterial disease with collaterals and showing abrupt cutoff with left popliteal artery (Figure 1) with functioning collaterals. Cardiology call was sought and additional antihypertensives were started. The coordination of medical, haemotological and surgical care for this patient was carefully planned before his surgery.

The patient was scheduled for Femoropopliteal bypass. A 16 gauge intravenous cannulae was inserted. As per haemotologists advice 2500 units of Factor VIII were given half an hour prior to surgery intravenously. On table premedication was provided with injection midazolam 2mg. Following preoxygenation with oxygen for 5 minutes, anaesthesia was induced with thiopentone 400 mg. Vecuronium 6 mg was used to facilitate the insertion of 8.5 mm portex cuffed oral endotracheal tube. Vecuronium was used to provide adequate muscle relaxation and intermittent positive pressure ventilation was established. Systolic blood pressure remained around 110-140 mmHg throughout the surgery. The entire operation lasted 4 hours. Intraoperative findings were lots of periarterial adhesions with absent flow in lower one thirds of thigh. 5 cm of femoral artery had atherosclerotic debris. Sudeen cutoff at the lower end of popliteal artery was observed. Popliteal artery was dissected, Fogarty catheter introduced and the intraluminal debris extruded. Saphenous vein was harvested and a femoropopliteal bypass performed.

Reversal of residual neuromuscular blockade was achieved satisfactorily with neostigmine 3.5 mg and atropine 1.2 mg. Spontaneous ventilation was quickly established. After extubation of the trachea the patient was given oxygen by facemask for 10 minutes in the operation theatre. Estimated blood loss during surgery was minimal. Patient was monitored with cardioscope and pulse oximeter. All vital parameters were maintained well throughout the procedure. Postoperatively analgesia was provided by injection tramadol 100 mg IV twice a day. Postoperatively the patient was given factor VIII 12 hourly for 2 days on the third postoperative day the patient's factor VIII activity was 92 %. A repeat coagulogram showed the following findings PT (14 sec), PTI (100 %), INR of 1 and aPTT of 30. Postoperative Anticoagulant Treatment was given in the form of Low molecular weight Heparin on 1st Postoperative day. On the seventh day postoperatively the patients Hb was 12 gm/dl. The patient was discharged on the sixth postoperative day.

DISCUSSION

Haemophilia A is a hereditary deficiency of factor VIII and is transmitted as an X-linked trait with variable expressio being the most common and serious hereditary disorder of coagulation. It is carried by females who are unaffected so it is a disease of males. The incidence is 1 in 5000 male live births (3). 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 vasculaintegrity.

Whereas Buerger's disease or thrombangiitis obliterans (TAO) is non artherosclerotic vascular disease that most commonly affects the small and medium-sized peripheral arteries and veins. Although a strong association with tobacco use has been established, the cause of the disease remains unknown. Possible pathogenic factors include autoimmune phenomena, haemostatic factors abnormalities and hyperhomocystynemia. In a study conducted by Hus et al it is shown in patients with Buerger's disease there were significantly higher levels of prothrombotic factors as compared to healthy control (4). But in our case of Buerger's disease with Haemophilia A the concentration of factor VIII were found to be less.

Tendency to bleed in Haemophilia A patients is inversely proportional to factor VIII levels in the body. Normal plasma activity (5) levels usually range between 0.5 Uml-1 and 1.5 Uml-1 (50-150 %). Severely affected patients have < 1 % of normal factor levels, while those with moderate disease have 1-4 %, and the patients with mild disease have 5-50 % (6). Intracra-nial haemorrhage either extra or intracerebral is common in severe disease (7). Our patient complained of pain in the lower limbs while walking for more than a 100 feet and onset of bluish discoloration of little toe of left foot. Levels of 3-5 % may adequately protect the patient from spontaneous bleeding, whereas patients with 10-15 % of normal activity may be entirely asymptomatic (8).

The diagnosis is often made on, laboratory-finding such as greatly reduced factor VIII, elevated PTT. Tests which are not dependent upon factor VIII (9) i.e. Prothrombin Time, bleeding time, platelet count and clot retraction will be normal in these patients. 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 (10). Since half-life of factor VIII is about 12 hours, it must be administered twice daily.

If Factor VIII is not available then 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 (11). Complicating factor in management of haemophilia A is development of antibodies to factor VIII after multiple exposures to factor VIII (12). There is always a chance of transmission of hepatitis A and HIV (13) in commercially produced factor VIII. 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. During manipulation or intubation of the airway extra care is necessary 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. If the decision is made to proceed with neuraxial anaesthesia, a subarachnoid block using a small gauge spinal needle may be preferable to epidural anaesthesia (14). Post operatively analgesics such as aspirin and other NSAIDs should not be given as it can predispose patients to gastrointestinal haemorrhage (15). Patient-controlled analgesia is a safe and effective alternative to intramuscular injections (3).

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, and intravenous sedation. For closure of the wound absorbable suture material like catgut should be used and local pressure should be applied (7). The fibrinolytic inhibitors, E amino-caprice acid (EACA) or tranexamic acid are commonly administered to reduce requirement of factor VIII. The va-sopressin analogue DDAVP (Desamino-VIII-D-arginine vasopressin), which increases plasma concentration of factor VIII, can be administered intravenously (7).

So in patients of Haemophilia A with Thrombophilia, both anesthesiologist and surgeons should be aware of the expected complications as these patients are known to have increased morbidity.

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