|Year : 2022 | Volume
| Issue : 4 | Page : 243-246
An anesthetic management of patient with hemophilia A undergoing recurrent perianal abscesses: A case report
Oktom Nurumbetova, Özer Tuç, Nedim Çekmen
Department of Anesthesiology, Faculty of Medicine, Baskent University, Ankara, Turkey
|Date of Submission||09-Jul-2022|
|Date of Decision||09-Aug-2022|
|Date of Acceptance||19-Aug-2022|
|Date of Web Publication||31-Oct-2022|
Department of Anesthesiology, Faculty of Medicine, Baskent University, Fevzi Cakmak Caddesi 10, Sokak No: 45 Bahcelievler, 06490 Ankara
Source of Support: None, Conflict of Interest: None
Hemophilia A is an x-linked recessive disorder characterized by an absent or reduced factor VIII (FVIII). It is a genetic deficiency that causes increased bleeding. It can be challenging for anesthesiologists to discover it in the perioperative management. This case report represents the successful management of a 25-year-old male patient having severe hemophilia A. We managed a 25-year-old man, 89 kg, 180 cm, with a recurring history of perianal abscesses. Previously at 13 years old, he experienced prolonged and excessive bleeding after a minor cut and injury. Following clinical and laboratory investigation, he was diagnosed with severe hemophilia A. Ever since, he used to take antihemophilic factor (AHF) VIII transfusion therapy and revealed severe disease with a positive inhibitor. No other coexisting disease was revealed during history-taking. We managed this case with general anesthesia because of the fact that he was hemophilic. We administered 2000 International Unit (IU) of recombinant Factor VIII (rFVIII) preoperatively as per the hematologist’s recommendation. We continued with 2000 IU of AHF FVIII transfusion after the surgery, but unfortunately, he redeveloped the abscess again. Another careful anesthesia and surgery were then conducted with a satisfactory result. The multidisciplinary team approach was provided to a positive outcome in these patients. Perioperative transfusion of AHF FVIII is essential for patients with hemophilia.
Keywords: Anesthesia, factor VIII, general, hemophilia A
|How to cite this article:|
Nurumbetova O, Tuç &, Çekmen N. An anesthetic management of patient with hemophilia A undergoing recurrent perianal abscesses: A case report. Bali J Anaesthesiol 2022;6:243-6
|How to cite this URL:|
Nurumbetova O, Tuç &, Çekmen N. An anesthetic management of patient with hemophilia A undergoing recurrent perianal abscesses: A case report. Bali J Anaesthesiol [serial online] 2022 [cited 2022 Nov 26];6:243-6. Available from: https://www.bjoaonline.com/text.asp?2022/6/4/243/359931
| Introduction|| |
A congenital hemostasis disorder known as hemophilia A, a factor VIII (FVIII) deficiency, is an inherited x-linked recessive pattern. This bleeding disorder is associated with reduced or absent FVIII biological activity. In 80% of cases of hemorrhagic diseases caused by hemophilia A and among the other coagulation diseases, the incidence is approximately one in 5000 male births. All individuals with FVIII deficiency are characterized by excessive bleeding after minor trauma to spontaneous hemorrhage.
The normal plasma concentration is 0.5–1.5 International Unit (IU)/mL or 50%–150%. Depending on plasma levels, it is classified as mild (FVIII level, >5%), moderate (1%–5%), and severe (<1%). An assay of FVIII deficiency is characterized by prolonged activated prothrombin time (APTT), but platelet count (PLT), bleeding time (BT), and prothrombin time (PT) remain normal., Frequently, hemophiliac patients with massive blood loss can be life-threatening and require urgent management. Regardless of the severity of the disease and the type of surgery, the anesthetic management in patients diagnosed with hemophilia A is a challenging and logical multidisciplinary consideration. Herein, in this case, our main goal is to discuss the anesthetic management of a patient with hemophilia A undergoing recurrent perianal abscesses in the literature light.
| Case Report|| |
A 25-years-old man, 89 kg, 180 cm, presented with anal pain. After investigation, he was diagnosed with a perianal abscess and scheduled for surgery. A patient had multiple histories (six times) of perianal abscesses. On all occasions, he had them drained under local anesthesia. At 13 years old, he had prolonged and excessive bleeding after a minor cut and injury. Following clinical and laboratory investigation, he was diagnosed with severe hemophilia A (0.5% serum FVIII activity). Ever since he used to take antihemophilic factor (AHF) VIII transfusion therapy and revealed severe disease with a positive inhibitor. No other coexisting disease was revealed during history-taking.
On preoperative laboratory assay, his APTT was 28 s, PT was 12.9 s, PLT was 188.000/mm3, and hemoglobin level was 17.2 g/dL. Other investigations were otherwise unremarkable, including electrocardiography, transthoracic echocardiography, and chest radiography. The hepatitis C virus and human immunodeficiency virus assays returned as nonreactive. During preanesthetic visits, we collected a standard written and verbal ethical approval from a patient for the possibility of future publication for his case.
We concluded this case as an American Society of Anesthesiologists (ASA) 2 patient. The patient was admitted to the operating room with complete care. Standard ASA monitoring was attached. The peripheral line was secured with a 22-gauge cannula, and an Isolyte solution was initiated. Preoperatively, 2000 IU of recombinant Factor VIII (rFVIII) was infused as per the hematologist’s recommendation. Considering the relative contraindication of spinal anesthesia in severe hemophilia A and the timing of the procedure, we decided to conduct the deep sedation approach. He was preoxygenated with a face mask at 6 L/min. At induction, midazolam 0.5 mg/kg, fentanyl 1 mcg/kg, lidocaine 1 mg/kg, and propofol 2 mg/kg were administered intravenously (IV). Antibiotic prophylaxis was provided with cefazolin 2 g IV. During surgery, the patient was face masked and had an oropharyngeal airway to prevent airway obstruction. Anesthesia was maintained with sevoflurane 2%. Intraoperatively, adequate hemostasis was achieved. The entire surgery lasted 30 min.
Postoperatively, a patient was transferred to the postanesthesia care unit (PACU). He was monitored and oxygenated via nasal prong until fully conscious. Tramadol 50 mg IV was provided for postoperative analgesia. During the postoperative period, his vital signs were typical. On the first day of postoperation, he was transfused 2000 IU of AHF VIII and discharged from the hospital with no complications.
Ten days after the surgery, he redeveloped a recurrent perianal abscess. Another elective surgery was scheduled. Again, we used standard monitoring. Peripheral IV cannulation was secured, and we administered 2000 IU rFVIII. Preoxygenation with a face mask at 6 L/min and induction with midazolam 0.5 mg/kg, fentanyl 1 mcg/kg, lidocaine 1 mg/kg, and propofol 2 mg/kg were administered. IV 2 g of cefazolin was also given. A laryngeal face mask was inserted. Sevoflurane 2% was used to maintain anesthesia. The operation was completed with minimum bleeding and lasted 40 min. After surgery, he was transferred to PACU, monitored, and oxygenated. Postoperatively, he continued to receive rFVIII 2000 IU IV. He was discharged from the hospital on the third postoperative day without complications.
| Discussion|| |
Hemostasis means maintaining normal blood flow and the clotting process to stop bleeding. The blood clotting machinery exists as a highly complex and dynamic system and derives from balancing procoagulant, anticoagulant, and fibrinolytic forces to facilitate hemostasis without causing thrombosis. This coagulation cascade involves two dozen extracellular proteins produced from multiple genes. The affected individuals are male, but female carriers, although female heterozygous can be encountered in approximately 30% with FVIII activity less than 40%.
The clinical symptoms present with spontaneous or trauma hemorrhage in musculoskeletal and joints, most frequently in the ankle, knee, and elbows. The frequency and the age of diagnosis are present regardless of FVIII biological activity. The diagnosis of a disease is made on bleeding manifestations that cannot be explained by trauma and excessive and oozing hemorrhage after surgical or dental procedures that require coagulation studies. Patients have prolonged APTT and normal PT, BT, and PLT.
Congenital bleeding FVIII deficiency disorder is uncommon but performs significant challenges in the perioperative management. In preparing for surgery, patients with mild and severe hemophilia A must be provided with clotting FVIII concentrates before intubation because there is a high risk of bleeding complications such as hematoma and trauma-induced hemorrhage from the upper airway tract.
In the event of the absence of inhibitors, each IU of recombinant AHF VIII level increases the plasma FVIII level by approximately 2 IU/dL depending on body mass index. Before surgery, the patient’s FVIII was 0.5%, and we started to infuse IV 2000 IU slowly in 2 min by the recommendation of the hematologist. Because the half-life of FVIII is approximately 8–12 h, it must be administered twice a day. Because the routine surgical intervention was predicted to have less bleeding volume, the bolus injection method was appropriate for our patient.
When recombinant FVIII is unavailable, cryoprecipitate and fresh frozen plasma (FFP) can be used. Cryoprecipitate contains FVIII, von Willebrand factor (vWF), factor XIII, and fibrinogen. It (30–40 mL) provides 80 U of FVIII. FFP contains 1 U of FVIII activity in 1 mL. In the presence of FVIII antibodies, bypassing agents (rFactor VIIa or Factor VIII inhibitor bypass activity [FEIBA]) are recommended.
Apart from the FVIII, most mild hemophiliac patients do not mandatorily require clotting FVIII concentrations. 1-Desamino-8-D-arginine-vasopressin (DDAPV), a synthetic analog of vasopressin, can increase the FVIII and vWF plasma levels 3–5 times baseline levels by releasing vWF that, in turn, forms a complex with FVIII so that preventing its disintegration. Nonetheless, it is not the choice of treatment in severe hemophilia A. Antifibrinolytic agents such as tranexamic acid and E-aminocaproic acid can be helpful as an adjunctive therapy in controlling bleeding from mucous membranes and in dental procedures in hemophiliac patients.,, Because our patient had minimal blood volume loss, we did not use the adjusted therapy.
Chauhan et al. reported implementing general anesthesia for bilateral total knee prosthesis to a 36-year-old male patient with hemophilia A who was infused with AHF VIII before surgery and reported a successful outcome. Another report described two cases with postoperative complications after total knee and hip arthroplasty. One of them performed with a hematoma on postoperative day 70 despite infusing rFVIII preoperatively and postoperatively. The second patient’s problem was an infection in aspirating samples from the knee joint. They suggested it thus to hematoma in the intra-articular cavity, which was found intraoperatively.
The perioperative management should be carefully planned. Routine procedures such as intravascular access do not cause excessive bleeding, but intramuscular injection arterial punctures should be avoided. Smooth induction under deep anesthesia should be provided. Tracheal intubation, oropharyngeal suction, and any manipulating of the airway should be done with caution and proper care because of submucosal hemorrhage, which can be life-threatening. Traumatic manipulations such as nasal intubation should be avoided so they can lead to bleeding and aspiration. Considering the risk of hematoma, spinal anesthesia is contraindicated. General anesthesia was the choice of anesthesia for our patient, and we avoided tracheal intubation because of the minority of surgery. Pressure points were padded during positioning to prevent hematomas and hemarthrosis in the extremities. Hemodynamic status was maintained near-normal range because hypertension and tachycardia can lead to increased surgical bleeding. Hemostasis in small vessels was achieved with minimal intraoperative blood loss.
Tramadol provided sufficient postoperative analgesia for our case. We avoided nonsteroid anti-inflammatory drugs due to gastrointestinal complications such as ulcers, bleeding, and perforation in hemophiliac patients.
| Conclusion|| |
The patient with classic hemophilia A presenting with a perianal abscess was managed successfully. It is crucial managing patients having FVIII deficiency disorder with a cohesive multidisciplinary team including anesthesiologists, surgeons, and hematologists. In addition, our patient has a history of eight times recurrent perianal abscesses and Ig A negativity, which can be related to several conditions such as immunosuppression and malignancy.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
| References|| |
Berntorp E, Fischer K, Hart DP, Mancuso ME, Stephensen D, Shapiro AD, et al
. Haemophilia. Nat Rev Dis Primers 2021;7:45.
Shah UJ, Narayanan M, Smith JG. Anaesthetic considerations in patients with inherited disorders of coagulation. Cont Educ Anaesthesia Crit Care Pain2015;15:26-31.
Mistry T, Dogra N, Chauhan K, Shahani J. Perioperative considerations in a patient with hemophilia A: A case report and review of literature. Anesth Essays Res 2017;11:243-5.
] [Full text]
Peters R, Harris T. Advances and innovations in haemophilia treatment. Nat Rev Drug Discov 2018;17:493-508.
Konkle BA, Huston H, Nakaya Fletcher S, Hemophilia A. 2000. In: Adam MP, Everman DB, Mirzaa GM , et al
editors. GeneReviews® [Internet]. Seattle, WA; 2017. p. 1-19. PMID: 20301578.
Kulkarni R, Soucie JM, Lusher J, Presley R, Shapiro A, Gill J, et al
; Haemophilia Treatment Center Network Investigators. Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: A report from the Centers for Disease Control and Prevention’s (CDC) Universal Data Collection (UDC) project. Haemophilia 2009;15:1281-90.
Srivastava A. Diagnosis of haemophilia and other inherited bleeding disorders—Is a new paradigm needed? Haemophilia 2021;27 Suppl 3:14-20.
Okamoto A, Yamamoto K, Eguchi G, Kanai Y, Yamaguchi T, Maeda YPerioperative management of hemophilia A using recombinant factor VIII in patients undergoing major or minor surgery. In: Abrol P, editor. Hemophilia - Recent Advances. IntechOpen 2018;1:1-86. DOI: 10.5772/intechopen.81172
Page D. Comprehensive care for hemophilia and other inherited bleeding disorders. Transfus Apher Sci 2019;58:565-8.
Chauhan P, Dhanerwa R, Mahajan HK. Management of hemophilia-A for B/L TKR. J Med Sci Clin Res 2015;3:4372-6.
Tateiwa T, Takahashi Y, Ishida T, Kubo K, Masaoka T, Shishido T, et al
. Perioperative management of hemophilia patients receiving total hip and knee arthroplasty: A complication report of two cases. Ther Clin Risk Manag 2015;11:1383-9.
Kim I, Park M, Lee KE, Lee JH. Dental management under general anesthesia of children with hemophilia A and hemophilia B. J Korean Assoc Disability Oral Health 2018;14:102-5.
Kumar P, Banerjee A, Bansal T, Pirkad A, Govil V. Hemophilia A: An anesthetic challenge. Anaesth Pain Intensive Care 2016;20: 221-22.
Levchenko O. Haemophilia A. Anästh Intensivmed 2022;63: 106-12.