|Year : 2013 | Volume
| Issue : 4 | Page : 156-159
Perioperative management of a patient with hemophilia A with acute subdural hematoma
Virender Belekar1, Vaibhav Jain1, Priyanka Deshmukh1, Sandeep Iratwar2
1 Department of Anesthesia, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India
2 Department of Neurosurgery, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India
|Date of Web Publication||26-Feb-2014|
Incharge, Surgical Intensive Care Unit, Jawaharlal Nehru Medical College, Wardha - 442 002, Maharashtra
Source of Support: None, Conflict of Interest: None
The hallmark of hemophilia A is prolonged bleeding and re-bleeding. We report a case of 28-year-old male with hemophilia A who after road accident presented with acute subdural hematoma which after evacuation re-bled to extradural hematoma. Prompt diagnosis and treatment with Factor VIII infusions perioperatively and meticulous hemostasis are the key to the management of such cases.
Keywords: Anesthesia, factor VIII, hemophilia, intra-cerebral hemorrhage
|How to cite this article:|
Belekar V, Jain V, Deshmukh P, Iratwar S. Perioperative management of a patient with hemophilia A with acute subdural hematoma. J Appl Hematol 2013;4:156-9
|How to cite this URL:|
Belekar V, Jain V, Deshmukh P, Iratwar S. Perioperative management of a patient with hemophilia A with acute subdural hematoma. J Appl Hematol [serial online] 2013 [cited 2020 Aug 7];4:156-9. Available from: http://www.jahjournal.org/text.asp?2013/4/4/156/127904
| Introduction|| |
Hemophilia A is rare X-linked recessive hereditary hemorrhagic disease caused deficiency of functional plasma clotting factor VIII, which may be inherited or arise from spontaneous mutation. Hemophilia A represent 80% of hemophilia cases; , other less common forms are hemophilia B and C. Incidence of hemophilia A is about 1 in 5000 male births.  Prolonged bleeding and re-bleeding are diagnostic symptoms of hemophilia.
Intracranial hemorrhage (ICH) is a life threatening complication of hemophilia. The site of bleeding is about equally distributed between subdural hematoma, intracerebral and subarachnoid hemorrhage.  Isolated ICH alone has a very high mortality rate. Incidence of mortality with hemophilia is still higher. 
We report a perioperative case management of hemophilia A with subdural hematoma after road accident and subsequent re-bleeding leading to extradural hematoma.
| Case Report|| |
A 28-year-old male, 60 kg, was admitted to emergency department with history of road accident 2 days back. He was admitted to some other center where he was conscious, Glasgow coma score (GCS) 15/15, and the computed tomogram of brain was normal; hence was discharged within 24 hours. After 48 hours of RTA, he became unconscious, had vomiting and convulsions. The patient was a known case of hemophilia A having previous history of excessive bleeding after minor cuts in his childhood for which he received factor VIII transfusions.
On admission, heart rate was 88/min, blood pressure 160/90 mm Hg, Resp. Rate 28/min, Glasgow coma scale was E 1 M 2 V 1 . Pupils were bilaterally dilated, not reactive to light. Computerized Tomography Scan showed 'Large acute subdural hematoma in right fronto-parietal temporal region with significant midline shift and brain herniation' [Figure 1]. He was immediately intubated with IV fentanyl 2 ug/kg, Inj. propofol 100 mg, Inj. rocuronium 60 mg and Inj. lignocaine 100 mg.
Patient was mechanically ventilated in Surgical Intensive Care Unit (SICU) on volume control mode. All cerebro-protective measures were initiated including 45° propped up position, Inj. mannitol 1 gm/kg, Inj. phenytoin 15 mg/kg loading, hyperventilation to reduce PaCo 2 <32 mm hg.
Patient's Blood Investigations were Normal Except Prolonged APTT
Patient was planned for Emergency Craniotomy and clot evacuation. Hematology consultation was taken and Factor VIII (IMMUNATE, Baxter Pharmaceuticals) 1500 IU was administered preoperatively and infusion was continued in intraoperative period (1000 IU). Inside operation theater 2, wide bore 18G cannula were secured. Right subclavian vein was cannulated with double lumen central venous catheter for CVP monitoring and fluid management. Patient was monitored intraoperatively with pulse oximetry, ECG, non-invasive blood pressure, end tidal CO 2 , temperature measurement, and urine output and CVP monitoring. Anesthesia was maintained with O 2 : Air (FiO 2 : 0.5) and sevoflurane. Analgesia was maintained with Inj. fentanyl intermittent doses of 1 μg/kg And Inj. vecuronium (loading 0.1 mg/kg and maintenance 0.02 mg/kg every 25 minutes) for muscle relaxation.
Patient received tranexemic acid (10 mg/kg loading and maintenance 1/mg/kg/hr). Intra-operative blood loss was around 1200 ml. Patient received 1 unit packed red blood cells (PRBC). Patient remained hemodynamically stable throughout the surgery. Patient was shifted to SICU for elective mechanically ventilation. Patient was sedated and paralyzed with Inj. midazolam and Inj. vecuronium. Post op analgesia was maintained with fentanyl patch (50 μg/hr) and paracetamol infusion. ABG analysis revealed no major acid-base imbalance. Serum electrolytes were within normal limits.
Patient received 1 vial of IMMUNTE (250 IU) every 2 hourly on post-op day 1 along with Inj. mannitol, Inj. phenytoin, acid prophylaxis and antibiotics. Sedation and paralyzing agents were stopped in the morning for neurological assessment. His GCS scale was E 3 M 6 V t . Patient was weaned from the ventilator and kept on T-piece with oxygen. Patient was extubated in the evening. Factor VIII assay report was 40%. Factor VIII infusion rate was reduced to 1 vial every 4 hourly. Patient was hemodynamically stable, and GCS was E 3 M 6 V 3 .
On 2 nd postoperative day, GCS deteriorated to E 1 M 3 V 1 with dilated pupils, with Left-sided weakness. Immediate computed tomogram was done which was suggestive of 'large extradural hematoma on right fronto-parietal region with cerebral edema and bony defect consistent with post-operative history' [Figure 2]. Emergency craniotomy for evacuation of clot and hemostasis was scheduled.
Patient was loaded with IMMUNATE 6 vials (1500 IU) and shifted to operation theater. Patient was administered general anesthesia with endotracheal intubation with muscle relaxant. Intraoperatively besides infusion of Factor VIII (750 IU), patient also received cryoprecipitate transfusion 6 units. Patient received 1 unit PRBC. Patient was shifted to SICU postoperatively for elective mechanical ventilation with sedation and muscle relaxant. Factor VIII transfusion were continued at rate of 1 vial every 2 hourly.
On post-op day 3, patient was weaned off from mechanical ventilation and was extubated. Post-extubation GCS was E 3 M 6 V 3 with left-sided weakness. Patient also received 6 bags of cryoprecipitate following extubation. Other laboratory investigations including ABG, KFT, CBC, coagulation profile were within normal limits.
Patient received IMMUNATE transfusion till post op Day 15. Factor VIII assay were 70% on post-op day 7 and 50% on post-op day 13. There were no other complications in post-op period. GCS improved to E 4 M 6 V 5 on post-op day 20 with no residual neurological deficit. Patient was discharged on post-operative day 30.
| Discussion|| |
Hemophilia A is a X-linked, recessive disorder caused by deficiency of functional plasma clotting factor VIII. Incidence of hemophilia A is about 1 in 5000 male births. Affected persons have a bleeding tendency that is inversely proportional to factor VIII levels in the body. Hemophilia severity is classified according to baseline level of clotting factor activity  [Table 1]. Factor VIII activity levels are reported in units with 1 U/ml corresponding to 100% of factor found in 1 ml of normal plasma. Levels of normal plasma activity usually range between 0.5 U/ml and 1.5 U/ml (50-150%).
Hemophiliacs do not bleed more intensely compared with a normal person but can bleed for much longer period. In severe hemophiliacs, even minor injuries could result in blood loss lasting for days, weeks or not ever healing completely. In areas such as brain or inside the joint, this can be fatal or permanently debilitating. 
One International Unit (IU) factor VIII per kg body weight raises the plasma factor VIII activity by 1.5% to 2%, with half-life of 10-12 hours. The required dosage is determined using the following formula. 
Required units = body weight (kg) × desired factor VIII rise (%) × 0.5
Factor VIII should be infused slowly at a rate not more than 2 ml/minute. Besides determination of factor VIII levels, the amount to be administered and the frequency of administration should always be oriented to the clinical effectiveness in the individual case.
Recommended factor VIII levels are illustrated in [Table 2].
As emergency factor VIII levels were not available, we transfused factor VIII as advised by the hematologist and clinical effectiveness in pre and intraoperative period.
Apart from factor VIII, cryoprecipitate and fresh frozen plasma can be used. These blood components can be used as an alternative if factor concentrates are not available. 30-40 ml of cryoprecipitate provide 80 U of Factor VIII. 1 ml of FFP will provide one unit of Factor VIII activity.
For patients with mild hemophilia desmopressin can also be administered either intravenously or intranasally. Fibrinolytic inhibitors, such as ε-aminocaproic acid (EACA) and tranexamic acid, can be given as adjunctive therapy for bleeding from mucous membranes and are particularly useful for dental procedures. 
Surgery in hemophiliacs is a major challenge to all perioperative clinicians. Routine procedures such as IV cannulatons, intramuscular injections, tracheal intubations, tracheostomies, oropharyngeal suction should be done with caution and meticulous care so as to avoid bleeding in such patients. , Central venous catheters should ideally be placed under ultrasound guidance, if available.
Perioperative hemodynamics of such patients should be tightly controlled so as to avoid excessive bleeding due to raised blood pressure and tachycardia. Special care should be taken to achieve hemostasis while operating so as to avoid re-bleeding in postoperative period. Hemophilia lowers the blood plasma clotting factor levels of the coagulation factors needed for the normal clotting process. Thus when a blood vessel is injured, a temporary scab does forms, but the missing coagulation factors prevent fibrin formation which is necessary to maintain the blood clot. Hence hemophiliacs are more prone for re-bleeding, inspite of meticulous hemostasis.
To conclude, all the patients with hemophilia A should be closely monitored for 48-72 hours as these patients tend to bleed for longer period of time. Factor VIII assays are not available in emergency situations, so factor VIII replacement should be done as per the clinical scenario and surgical procedure. These patients are more likely to re-bleed, hence close monitoring of factor VIII levels, GCS and prompt diagnosis and management is required in these patients for the better outcome in this population.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]