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CASE REPORT
Year : 2007  |  Volume : 51  |  Issue : 2  |  Page : 145 Table of Contents     

Anaesthetic management of a patient with haemophilia


1 M.D., DNB Asst. Prof., Department of Anaesthesia, Kasturba Medical College, Mangalore - 575 001, India
2 M.D., Asst. Prof., Department of Anaesthesia, Kasturba Medical College, Mangalore - 575 001, India
3 M.D., Asso. Professor, Department of Anaesthesia, Kasturba Medical College, Mangalore - 575 001, India
4 M.D;D.A Prof. and Head, Department of Anaesthesia, Kasturba Medical College, Mangalore - 575 001, India

Date of Acceptance12-Feb-2007
Date of Web Publication20-Mar-2010

Correspondence Address:
Prashanth Mallya
802, Symphony Apartments, Sturrock Road, Attavar, Mangalore- 575 001
India
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Source of Support: None, Conflict of Interest: None


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Haemophilia A is a bleeding disorder that has a spectrum of manifestations ranging from persistent bleeding after minor trauma to spontaneous haemorrhage. [1] We report a case of a male patient with haemophilia A, who received general anesthesia for exploration of right hernial sac. Shortly before surgery he received 2500 units of factor VIII. There was no excessive blood loss intraoperatively. Postoperatively the patient was supplemented with factor VIII. Rest of the postoperative course 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.

Keywords: Anaesthesia, Coagulation, Haemophilia


How to cite this article:
Mallya P, Kaimar P, Jithesh R, Ranjan R K, Ambareesha M. Anaesthetic management of a patient with haemophilia. Indian J Anaesth 2007;51:145

How to cite this URL:
Mallya P, Kaimar P, Jithesh R, Ranjan R K, Ambareesha M. Anaesthetic management of a patient with haemophilia. Indian J Anaesth [serial online] 2007 [cited 2019 Sep 20];51:145. Available from: http://www.ijaweb.org/text.asp?2007/51/2/145/61133


   Introduction Top


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 Top


A 52 year old male weighing 70 kg presented with pain abdomen to the surgery department. The patient gave history of swelling in right inguinal region for two years. At the age of fifteen the patient had history of tooth extraction following which he had prolonged and excessive bleeding lasting 10 - 12 hours for which four units of blood were transfused. The patient was investigated and diagnosed with haemophilia A. The patient also gave history of bleeding in the right knee joint four years back which was treated in a hospital. There was no history of factor VIII transfusion at that time. Family history of the patient revealed his first degree relative diagnosed as haemophilia A. There was no other significant history of any medical disorder or exposure to anaesthesia or surgical intervention.

On examination, his heart rate was 86/min, regular. Arterial pressure was 130/80 mm of Hg. 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. Preoperative investigations were normal with haemoglobin of 11.6 gm%, prothrombin time was 15/12, INR 1.2, APTT 180 seconds. Factor VIII concentration was found to be about 20%. Test for inhibitors to factor VIII was negative. The coordination of medical , haemotological and surgical care for this patient was carefully planned before his surgery.

The patient was scheduled for exploration of right hernial sac and proceed. An 18 gauge intravenous cannula was inserted. Intramuscular injections were avoided. As per haematologists advice 2500 units of factor VIII were given half an hour prior to surgery intravenously. On table premedication was provided with injection pethidine 75 mg and injection promethazine 35 mg. Following pre-oxygenation for 5 minutes, anaesthesia was induced with thiopentone 300mg. Suxamethonium 1.5mgkg-1 was used to facilitate the insertion of 9.0 mm portex cuffed oral endotracheal tube. Pancuronium was used to provide adequate muscle relaxation and intermittent positive pressure ventilation was established. Systolic blood pressure remained around 140-110 mm of Hg throughout the surgery. The entire operation lasted 2 hours. Reversal of residual neuromuscular blockade was achieved satisfactorily with neostigmine 3.5 mg and atropine 1.2mg. 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 40%. On the seventh day postoperatively the patients Hb was 10.7 gm%. The patient was discharged on the seventh post operative day.


   Discussion Top


Haemophilia A is an X-linked recessive hereditary disorder characterized by a deficient or defective factor VIII coagulant. (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 5000 male live births. [2] 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. Haemophilia severity is classified according to the baseline level of clotting factor activity. Factor VIII activity levels are reported in units, with 1 U ml-1 corresponding to 100% of the factor found in 1 ml of normal plasma. Normal plasma activity levels usually range between 0.5 U ml-1 and 1.5 U ml-1 (50-150%). [3] 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%. [4] Those with < 1% of normal factor VIII level are susceptible to spontaneous bleeding episodes such as haemarthrosis, soft tissue haematoma and intracranial haemorrhage. Intracranial haemorrhage either extra or intracerebral is common in severe disease. [5] All the patients with haemophilia, regardless of the severity of the disease, are at risk of excessive bleeding during surgery.

Our patient complained of an episode of bleeding into knee joint. Levels of 3-5% may adequately protect the patient from spontaneous bleeding, whereas patients with 10-15% of normal activity may be entirely asymptomatic. [6]

The diagnosis of haemophilia is often made from family history, laboratory finding such as greatly reduced factor VIII, and 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. [7] The patient's haemoglobin may be low because of acute or chronic blood loss. When large haematomas are present, the serum bilirubin may be increased. [6] In preparing patients with haemophilia A for surgery, factor VIII levels are routinely raised to approach 100% of normal activity. It should be maintained for the first 3 postoperative days from the 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 Percent activity increase where N is the number of units required. Plasma volume is 40 ml/Kg for adults. [8] 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. [9] The complicating factor in management of haemophilia A is development of antibodies to factor VIII after multiple exposures to factor VIII. [10] Commercially produced factor VIII can be a vector for transmission of hepatitis A and HIV. [11] Surgeons, Haematologists and Anaesthesiologists should carefully plan the perioperative management of the patient with haemophilia.

Intramuscular pre medication should be avoided. Vascular access itself does not cause excessive bleeding and should be appropriate for the proposed procedure. . 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. [12] Our decision to proceed with general anesthesia was based on a risk-benefit analysis, weighing the risk of neuraxial bleeding with a regional anesthetic versus benefits of general anaesthesia. We elected the general anesthetic based on our patient having a Mallampati class 1 airway, a wide mouth opening, a 6.5 cm thyromental distance and a reasonable body mass index. If a more challenging airway had been observed, a regional anesthetic would have been more strongly considered, however, not before adequate factor VIII, and platelet concentrations were obtained. 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. 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. [8] Post operatively analgesics such as aspirin and other NSAIDs should not be given as it can predispose to gastrointestinal haemorrhage. [13] Patient-controlled analgesia is a safe and effective alternative to intramuscular injections. [13] Even for minor procedures like dental extraction the patient should be admitted to the hospital. The patient should be administered plasma (12 mlkg-1), 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. [5] The fibrinolytic inhibitors, epsilon amino-caproic acid (EACA) or tranexamic acid are commonly administered to reduce requirement of factor VIII. The vasopressin analogue DDAVP (Desamino-VIII-Darginine vasopressin), which increases plasma concentration of factor VIII, can be administered intravenously. [5]

 
   References Top

1.P. Dhar, S. Abramovitz, D. DiMichele, C. B. Gibb and F. Gadalla. Management of pregnancy in a patient with severe haemophilia A Br J Anaesth 2003; 91: 432-35   Back to cited text no. 1      
2.Mannucci PM, Tuddenham EG. The hemophilias-from royal genes to gene therapy. N Engl J Med 2001; 344: 1782-84   Back to cited text no. 2      
3.DiMichele D, Neufeld EJ. Hemophilia. A new approach to an old disease. Hematol Oncol Clin North Am 1998;12:1315-44.   Back to cited text no. 3  [PUBMED]    
4.Cahill MR, Colvin BT. Haemophilia. Postgrad Med J 1997; 73: 201-06   Back to cited text no. 4      
5. William ED, D David Glass. Haematological diseases. In : Katz, Benumof, Kadis. Anesthesia and uncommon diseases Philadelphia. 1990; 378-436.   Back to cited text no. 5      
6.Fudeta H, Hashimoto YK, Mori K. Anesthesia in patients with hemophilia A and B. Jpn J Anesth 1976; 25: 718.   Back to cited text no. 6      
7.Brinkhaus KM. The hemophilias. Chapel Hill, University of North Carolina Press. 1964; 69.   Back to cited text no. 7      
8.de Gruchy"s. Clinical haematology in medical practice, Coagulation disorders. Oxford University Press. 1993; 406-36.   Back to cited text no. 8      
9.Hathaway WE, Mahasondana C, Clarke S, Humbertr JR. Paradoxical bleeding in intensively transfused hemophiliacs. Transfusion 1973; 13: 6-12.   Back to cited text no. 9      
10.Roberts HR. Hemophiliacs with inhibitors : Therapeutic considerations. N Engl J Med 1981; 305: 757.   Back to cited text no. 10  [PUBMED]    
11.Curran JW, Lawrence DN, Jaffe H et al. Acquired immunodeficiency syndrome (AIDS) associated with transfusions. N Engl J Med 1984; 69: 310.   Back to cited text no. 11      
12.Abramovitz S, Beilin Y. Thrombocytopenia, low molecular weight heparin, and obstetric anesthesia. Anesth Clin N Am 2003; 21: 99-109.   Back to cited text no. 12      
13.Simon E, Roux C, More J. Drug combinations in the treatment of pain in hemophiliacs. Bibl Haematologica 1966; 26: 78.  Back to cited text no. 13      




 

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