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CASE REPORT
Year : 2022  |  Volume : 8  |  Issue : 2  |  Page : 180-182

Anesthesia Concerns and Management in Case of Severe Hemophilia A: A Case Report and Review of Literature


Department of Anaesthesiology, Plastic Surgery and Medical Oncology/Haematology, All India Institute of Medical Sciences, Rishikesh, India

Date of Submission18-Oct-2021
Date of Decision18-Nov-2021
Date of Acceptance13-May-2022
Date of Web Publication03-Aug-2022

Correspondence Address:
Sanjay Agrawal
Professor & Head, Department of Anaesthesiology and Critical Care, AIIMS, Rishikesh, Uttarakhand, India, 249203
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mamcjms.mamcjms_115_21

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  Abstract 


Abstract Hemophilia is a genetic disease presenting with increased chances of perioperative bleeding. Preoperative recognition, assessment of risk, and careful titration of factor VIII transfusion are important for anesthesia management and decrease of morbidity. Here we present a case of severe hemophilia with an open wound of the knee scheduled for flap rotation and cover.

Keywords: Anesthesia, factor VIII, hemophilia A


How to cite this article:
Jamgade D, Kantha M, Agrawal S, Chattopadhyay D, Nath UK. Anesthesia Concerns and Management in Case of Severe Hemophilia A: A Case Report and Review of Literature. MAMC J Med Sci 2022;8:180-2

How to cite this URL:
Jamgade D, Kantha M, Agrawal S, Chattopadhyay D, Nath UK. Anesthesia Concerns and Management in Case of Severe Hemophilia A: A Case Report and Review of Literature. MAMC J Med Sci [serial online] 2022 [cited 2022 Sep 25];8:180-2. Available from: https://www.mamcjms.in/text.asp?2022/8/2/180/354395




  Introduction Top


Hemophilia is a genetic disease affecting exclusively males, being an X-linked recessive disease, caused due to factor VIII deficiency. Genetically hemophilia is associated with inversion or deletion of major portions of the X chromosome genome or a missense mutation resulting in factor VIII activity below <1% of normal. Clinical severity of hemophilia A correlates with factor VIII activity level, and activity below <1% of normal is classified as severe hemophilia and such patient presents in childhood with frequent spontaneous hemorrhage into joints, muscles, and vital organs. Here we present anesthetic management of an adult male, known case of severe hemophilia on factor VIII therapy, who presented to the hospital for flap rotation and coverage of knee wound.


  Case Report Top


A 44-year-old male patient presented to the emergency department with chief complaints of the raw area over the left knee [Figure 1] for 1 month. He was diagnosed with septic arthritis of the left knee and underwent arthrotomy and drainage of pus. He underwent surgeries twice and needed ICU admission following the second surgery on knee. The patient was diagnosed with severe hemophilia (factor VIII level <1%) at 5 years of age and was receiving factor VIII concentrates on demand. He was a diagnosed case of type 2 diabetes mellitus for the past 2 years and is on treatment with tab Metformin 500mg twice daily and tab Glimepiride 2 mg once daily. He was also hypertensive for 5 years but was not compliant with medication. Past history of cerebrovascular accident 5 years ago was also elicited and managed conservatively with residual weakness in the right upper limb. He is a known alcoholic and smoker for the past 16 years.
Figure 1 Septic arthritis of left knee with raw area and pus.

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After admission, the patient was monitored by clinical hematology and advised factor VIII 3000 units administration before induction of anesthesia. Wound examination revealed septic arthritis of the knee joint [Figure 1] and was scheduled for debridement with gastrocnemius flap rotation and coverage of the knee [Figure 2].
Figure 2 Gastrocnemius flap rotation and coverage of the knee.

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Biochemical investigations were within normal limits. He was advised nil per oral for 6 hours for solids and 2 hours for clear fluids and was premedicated with tablet ranitidine 150 mg, tablet alprazolam 0.25 mg night before, and on day of surgery with a sip of water. 3000 units of factor VIII concentrates were administered intravenously 30 minutes before induction of anesthesia (500 units every 2–5 minutes). The patient was wheeled inside the operating room and ASA monitors were attached (ECG, NIBP, and Spo2). Induction of anesthesia was achieved with IV fentanyl 2 mcg/kg and IV propofol 2 mg/kg , muscle relaxation was achieved with IV vecuronium 0.1 mg/kg and intubated nontraumatically with an 8.0 mm cuffed endotracheal tube. Ventilation was maintained to achieve an end-tidal carbon dioxide level of 35 to 37 mmHg. Maintenance of anesthesia is achieved with 60% oxygen in the air, sevoflurane (MAC 0.8–1.0), and intermittent boluses of IV vecuronium and IV fentanyl. Injection tranexamic acid 1 g was administered intravenously after induction of anesthesia. As the patient required long-term iv access for fluid and factor VIII concentrate, left internal jugular vein cannulation with triple lumen catheter under ultrasound guidance was done. Peroperatively the patient had a blood loss of about 2 L and was resuscitated with ringer lactate 2000 mL, 3 packed red cells . IV paracetamol 15 mg/kg was administered 15 minutes before the end of surgery. The patient was hemodynamically stable and extubated on the table after administering inj neostigmine and glycopyrrolate and shifted to the postoperative anesthesia care unit.

Postoperatively, the patient received factor VIII 2000 units intravenously every 12 hours for two doses followed by 1500 units intravenously 12 hourly for 3 days once hemostasis was confirmed. The patient was discharged from the hospital in satisfactory condition.


  Discussion Top


Patients with hemophilia are a challenge to the surgeon and anesthesiologist, challenges ranging from securing intravenous access to managing the bleeding and coagulation perioperatively. These groups of patients require a thorough assessment and management from physician/clinical hematologists. Preoperative optimization reduces the risks and complications of a major surgery significantly.

Coagulation studies in patients with hemophilia A reveal high activated partial thromboplastin time but normal platelet count, bleeding time, and prothrombin time. Intracranial hemorrhage accounts for the major cause of mortality and morbidity in such patients. Factor VIII activity is reported in units, where 1 U/mL corresponds to 100% of factor VIII found in 1 mL of normal plasma. Normal plasma activity usually ranges between 0.5 and 1.5 U/mL (50–150%).[1],[2] Severely affected patients have <1% factor VIII activity, 1% to 4% in moderate diseases while mild diseases have 5% to 50% of normal factor VIII levels.

The replacement of recombinant FVIII concentrate must occur before or in parallel with any intervention in a patient with hemophilia. As per the recommendations of the Australian Haemophilia Centre, 1995[3] it is essential that the factor VIII activity in the blood is maintained at 80% to 100% for the first 3 postoperative days. The levels should be 60% to 80% from the 4th to 6th postoperative days, followed by levels of 40% to 60% for the next 7 days.

The dose of FVIII concentrate is calculated according to the World Federation of Hemophilia 2012 (WFH) guidelines – Patient’s body weight (in kg) × desired factor level (IU/dL) × 0.5 or weight (in pound)/4.4 × desired factor level (IU/dL). Our patient was transfused 3000 IU of factor VIII based on these guidelines.[4]

Desmopressin, a synthetic vasopressin analog, also increases plasma FVIII and Von Willebrand factor in mild to moderate hemophilia but not in severe hemophilia A or patients with high FVIII antibody titers and hence was not used in our patient. Tranexamic acid and ε-aminocaproic acid promote clot stability owing to antifibrinolytic activity and reduce bleeding from mucosal surfaces and are useful in hemophilia.

Vascular access is an important step in the management of hemophilia patients. We took utmost care by first administering FVIII and tranexamic acid 1 gm and then proceeding with USG guided left IJV cannulation. Succinylcholine was avoided to prevent muscle fasciculation, which may worsen muscle and joint hemorrhagic state. Smooth induction, gentle laryngoscopy, and minimal manipulation during airway intubation are required. Greasing the tubes (nasal, feeding, endotracheal) and probes (temperature) with lubricants reduces the chances of airway bleeding. Padding the pressure points with cotton reduces the chances of hematomas and hemarthrosis.

If factor VIII is not available, then cryoprecipitate is the next choice of blood product in the management of hemophilia A. It provides 80 units of factor VIII per bag. Presence of fibrinogen in cryoprecipitate warrants serum fibrinogen monitoring as it increases the risk of bleeding despite normal amounts of factor VIII if excessively transfused.[5] Despite adequate preparations and optimizing factor VIII levels as per guidelines, our patient required PRBC transfusion to augment the oxygen-carrying capacity of blood.

Postoperatively analgesics such as aspirin and nonsteroidal analgesics (NSAIDs) are avoided as they predispose patient to gastrointestinal hemorrhage. Intravenous patient-controlled analgesia is a safe and effective alternative to intramuscular injections.

In conclusion, we state that through preoperative assessment and optimization, administration of factor VIII concentrate, and utmost care during anesthesia with avoidance of drugs interfering with coagulation help in successfully managing the case.


  Conclusions Top


In conclusion, we state that through preoperative assessment and optimization, administration of factor VIII concentrate, and utmost care during anesthesia with avoidance of drugs interfering with coagulation help in successfully managing the case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Batorova A, Martinowitz U. Continuous infusion of coagulation factors. Haemophilia 2002;8:44–51.  Back to cited text no. 1
    
2.
Cahill MR, Colvin BT. Hemophilia. Postgrad Med J 1997;73:201–6.  Back to cited text no. 2
    
3.
Sethi M, Gurha P. Perioperative management of a patient with haemophilia-A for major abdominal surgery. Indian J Anaesth 2017:61:354–355. https://doi.org/10.4103/ija.IJA_96_17  Back to cited text no. 3
    
4.
Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, Ludlam CA, Mahlangu JN, Mulder K, Poon MC, Street A; Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia. 2013 19:e1–47. doi: 10.1111/j.1365-2516.2012.02909.x.  Back to cited text no. 4
    
5.
Hathaway WE, Mahasondana C, Clarke S, Humbertr JR. Paradoxical bleeding in intensively transfused hemophiliacs. Transfusion 1973;13:6–12.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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