Ali Sarfraz Siddiqui*, Jamil Ahmed** , Safia Zafar Siddiqui***. Saeeda Haider****
*Senior Anesthesiologist, **Senior Resident, ***Assistant Professor, ****Professor & Chairperson
Department of Anesthesiology, Surgical Intensive Care and Pain Management, Dow Medical College and Civil Hospital Karachi, Dow University of Health Sciences, Karachi (Pakistan).
Correspondence: Dr.Ali Sarfraz Siddiqui, Senior Anesthesiologist, Department of Anesthesiology, Surgical Intensive Care & Pain Management, Dow Medical College and Civil Hospital Karachi, Dow University of Health Sciences, Karachi (Pakistan); E-mail: firstname.lastname@example.org; email@example.com
Perioperative management of patient with severe von Willebrand disease (vWD) especially for major surgery is always a challenging task for the anesthesiologists. We report a case of a 17 year old female, diagnosed as a case of vWD at 8 years of age, who was scheduled for exploratory laparotomy for ruptured ovarian cyst with hemoperitonium. She was successfully managed with factor VIII, fresh frozen plasma (FFP) and tranexamic acid administration. Intraoperatively, more than 2 liters of clotted blood was removed and 1.5 liters of fresh blood was lost, which was replaced by red cell concentrate (RCC), colloid and crystalloid solutions. Post-operatively, cryoprecipitates and RCC were transfused. Postoperative serum coagulation studies remained within normal limits. She was transfused with cryoprecipitates and factor VIII till 5th day.
Patients with vWD do not carry an increased operative risk during elective procedures if appropriate prophylactic and corrective therapy is administered. Apart from the administration of cryoprecipitate and other blood products; factor VIII is needed for patients with severe vWD.
Key words: von Willebrand disease; von Willebrand factor; Bleeding.
Citation: Siddiqui AS, Ahmed J, Siddiqui SZ, Haider S. Perioperative anaesthetic management for a patient with severe von Willebrand disease for major gynaecological surgery. Anaesth Pain & Intensive Care 2009;13(1):19-22
von Willebrand disease (vWD) is a congenital bleeding disorder resulting from a quantitative or qualitative deficiency of von Willebrand factor (vWF)1. It is clinically characterized by mucocutaneous hemorrhages, and sometimes postoperative bleeding that can lead to catastrophic surgical outcome. We report a case of von vWD and highlight the relevance of this condition to the extensive perioperative anesthetic management sometimes required.
A 17 year old female was scheduled for exploratory laparotomy for ruptured ovarian cyst with hemoperitonium. She had been diagnosed to be suffering from vWD on investigation for an unusually severe bleeding from her forehead after a road traffic accident at the age of eight years. Past history revealed menorrhagia, intermittent epistaxis and gum bleeding, necessitating blood transfusion and cryoprecipitate and factor VIII concentrate administration.
She was admitted with the complaints of lower abdominal pain, abdominal distention, vomiting, fever and bleeding per rectum for 10 days. She was diagnosed to suffer from ruptured ovarian cyst with hemoperitonium by clinical history, laboratory evaluation, ultrasound abdomen and CT scan abdomen. She was planned for elective exploratory laparotomy and transfused 4 units of RCC. Pre-operative investigations revealed: Hb11.4 gm/dL, PCV 35 %, WBC count 6300 cells/dL, platelets 443000/dL, BT 6 min, CT 8 min, PT 12/13 seconds (control/ test), INR 1.09, aPTT 26/42 seconds(control/test) and Factor VIII assay to be 10 % of the normal. Plasma inhibitor to factor VIII was not detected. Hepatitis C antigen was positive. Her liver function tests showed; bilirubin total 2.4, bilirubin direct 2.4, SGPT 28, and alkaline phosphatase 196. Renal function tests and serum electrolytes were within normal limits.
Balanced general anesthesia with tracheal intubation was planned. The patient received factor VIII 2000 IU one hour before surgery. After pre-oxygenation, anesthesia was induced with propofol 2mg/kg, nalbuphine 6 mg and atracurium 25 mg. Patient was ventilated for 3 minutes before intubation with 6.5 mm I/D tracheal tube. Anesthesia was maintained with isoflurane and nitrous oxide in oxygen. On laparotomy, more than 2 liters of blood clots were removed from the peritoneum and another 1.5 liters of fresh blood was estimated to be lost intra-operatively which was replaced by RCC, FFPs, colloids and crystalloid solution. Patient was injected inj. tranexamic acid 1 gm IV intraoperatively. On complete recovery after surgery the patient was shifted to recovery room. Blood collection from the drain was 200 ml after 30 min. Post-operatively patient was shifted to SICU, where she was transfused with cryoprecipitate 4 units and RCC 4 units. She remained stable and pain free with good urine output. Factor VIII 1000 IU was repeated after 12 hours postoperatively. Postoperative serum coagulation studies were within normal limits. She was shifted to gynecology ward on the next day where she received factor VIII 1000 IU on day 2, 500 IU on day 3 and 500 IU on day 4; and cryoprecipitate 6 units daily till 5th postoperative day. Her coagulation profile remained within normal limits and factor VIII level was 80 % on 7th day. On 9th day, the peritoneal drain was removed and factor VIII level was measured to be 109%. She was given inj. tranexamic acid 500 mg 8 hourly and estrogen containing hormonal tablets were started. She was discharged on 10th day.
vWD was first described by a Finnish pediatrician Erik von Willebrand in 1926. It is the most common inherited autosomal dominant bleeding disorder in humans with prevalence of 0.1-1%1,2. It arises from a qualitative or quantitative deficiency of vWF – a large multimeric glycoprotein that is synthesized in endothelial cells and megakaryocytes and causes platelet adhesion and primary haemostatic plug formation at sites of vascular injury, and serves as a carrier for procoagulant factor VIII.1,3
Clinically, vWD is divided into three major types that provide the basis for appropriate therapy.1,2 Type I is the commonest form (60 -80%) and is characterized by partial quantitative decrease in all vWF multimers. Typically, the bleeding time is prolonged, but it may be normal in some individuals. Pregnancy, estrogen therapy, liver disease, inflammatory disorders and renal disease may result in an increase in vWF, which may convert a prolonged BT to normal. Type II (20-30%) is a qualitative defect and the bleeding tendency can vary between individuals. There are normal levels of vWF, but the multimers are structurally abnormal, or subgroups of large or small multimers are absent. Type III is the most severe and rare form of vWD (<1 %) in which complete quantitative deficiency of vWF occurs with severe mucosal bleeding and occasional hemarthroses. The aPTT is consistently abnormal. There is no specific age of onset of the disease1. Epistaxis (60%), menorrhagia (35%), easy bruising (40%), and gingival bleeding (35%) are cardinal features. Clinical symptoms also include excessive mucocutaneous bleeding, e.g. from postsurgical (20%), post dental (50%), bleeding from traumatic wounds (35%), postpartum hemorrhage (25%) and gastrointestinal bleeding (10%) which can be life-threatening or even fatal 3.
The coagulation screening tests for this disease include complete blood count, platelet count, PT, BT, aPTT and fibrinogen. Specific tests like Factor VIII level, Factor VIII binding assay, Factor VIII coagulant activity, vWF antigen and vWF activity may be required to identify sub types of vWD and to guide appropriate management. The cornerstone of treatment for vWD is the replacement of the vWF factor. Cryoprecipitate is administered in a dose of 1 unit/5-6 kg body weight, which raises the Factor VIIIC level by 15- 20%. During the intraoperative period, the consumption of vWF is increased, and may require the administration of cryoprecipitate as frequently as every 6-8 hours in contrary to every 8-12 hours postoperatively, after reviewing the bleeding time and the clinical response. FFP may be used but large amount is required. Platelets may be required in a patient who is thrombocytopenic (<50-80 x 109 /L).1,3,6
Desmopressin (1-desamino-8-D-arginine vasopressin; DDAVP) 0.3 μg/Kg IV releases vWF and factor VIII coagulant from storage sites 5,11. DDAVP is most effective in patients with Type I vWD but produces a variable response in patients with Type II disease. Potential side effect of DDAVP is hyponatremia4. vWF containing factor VIII concentrates is recommended in vWD that do not respond to DDAVP. Antifibrinolytic amino acids such as tranexamic acid and aminocaproic acid can be administered alone or in addition to DDAVP and plasma concentrates for adjuvant therapy 3,4,11. The antiplatelet drugs are avoided and fever and infections should be well controlled before surgery. During perioperative period of a major surgery, factor VIII activity and vWF activity should be kept at adequate levels (factor VIII: 105- 150%; vWF: 65-225%) 5. Intramuscular injections are avoided and rectal medications may be preferred 6. Minimally invasive procedure should be planned and during surgery the aim is to improve surgical hemostasis by performing an atraumatic dissection and avoiding incisional bleeding. Moreover the risk of nasal or urethral bleeding during catheter insertion should be kept in mind, as it is usually required for most of the surgeries. Oral route for gastric tube may be preferred 8,11.
Patients with vWD do not carry an increased peri-operative risk during elective procedures if appropriate prophylactic and corrective therapy is administered. Cryoprecipitates, other blood products and factor-VIII; all are needed for patients with severe vWD. Using a careful multidisciplinary approach, excellent hemostasis may be achieved even in patient with severe vWD.
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