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CASE REPORT
Year : 2008  |  Volume : 52  |  Issue : 5  |  Page : 573 Table of Contents     

Patient with von Willebrand Disease for Gynaecologic Surgery - Perianaesthetic Concerns


1 Senior Resident, Department of Anaesthesiology and Intensive Care, All India Institute Of Medical Sciences, Ansari Nagar, NewDelhi -110029, India
2 Assistant Professor, Department of Anaesthesiology and Intensive Care, All India Institute Of Medical Sciences, Ansari Nagar, NewDelhi -110029, India

Date of Acceptance27-Jun-2008
Date of Web Publication19-Mar-2010

Correspondence Address:
Mridu Paban Nath
Department of Anaesthesiology and Intensive Care, All India Institute Of Medical Sciences, Ansari Nagar, NewDelhi -110029
India
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Source of Support: None, Conflict of Interest: None


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We report a case of von Willebrand (vWD) disease and highlight the relevance of this condition to the perianaesthetic management.A 23-year-old female, known case of vWD diagnosed at 3 years of age, was posted for laparoscopic ovarian cystectomy. Pre-induction, patient was transfused with cryoprecipitate 10units. Standard tech­nique of anaesthesia was followed with intraoperative cryoprecipitate administration. Surgery was uneventful with adequate hemostasis. Post-operatively analgesia was maintained with morphine patient controlled analgesia(PCA). All intra-muscular injections were avoided. She was transfused with cryoprecipitate/ fresh frozen plasma(FFP) till 5th day and injection tranexamic acid was administered. Postoperative serum coagulation studies were within normal limits.
Patients with vWD do not carry an increased operative risk during elective procedures if appropriate prophylac­tic and corrective therapy is administered. Although the administration of cryoprecipitate and other blood products has traditionally been the cornerstone of treatment for vWD, the recent development of desmopressin(DDAVP) for clinical use may provide an effective alternative to replacement therapy with blood products. Further laparaoscopic procedures, taking care during ryle's tube and foley's catheter insertion, in such patients are the safer alternative for all kind of gynecologic surgeries.

Keywords: von Willebrand disease, von Willebrand factor, Coagulopathy.


How to cite this article:
Garg R, Nath MP, Verma S, Kumar A. Patient with von Willebrand Disease for Gynaecologic Surgery - Perianaesthetic Concerns. Indian J Anaesth 2008;52:573

How to cite this URL:
Garg R, Nath MP, Verma S, Kumar A. Patient with von Willebrand Disease for Gynaecologic Surgery - Perianaesthetic Concerns. Indian J Anaesth [serial online] 2008 [cited 2019 Oct 19];52:573. Available from: http://www.ijaweb.org/text.asp?2008/52/5/573/60678


   Introduction Top


von Willebrand disease (vWD) is a congenital bleeding disorder resulting from a quantitative or quali­tative deficiency of von Willebrand factor (vWF). vWD is clinically characterized by mucocutaneous hemor­rhages and sometimes, postoperative bleeding that can lead to catastrophic surgical outcome [1] . We report a case of vWD and highlight the relevance of this condi­tion to the perianaesthetic management.


   Case report Top


A 23-year-old female who was a known case of vWD diagnosed at 3 years of age, was posted for laparoscopic ovarian cystectomy. She was diagnosed vWD when she was investigated for abnormal dental bleeding. Past history revealed severe menorrhagia at 10 years of age and treated conservatively requiring blood transfusion (haemoglobin-4gm.dL -1 ) and steroid (levonorgestrol) with which she responded. At 17 years of age, she underwent emergency laparotomy for pain abdomen and was diagnosed as ruptured ovarian cyst with haemoperitoneum. Abdominal packs were applied to stop oozing for 2 days and supportive treatment with 15 units of cryoprecipitate was given intraoperatively. Further 10 units twice daily for 5 days and subsequently 5 units daily for next 5 days were administered.

On regular 6 monthly follow-ups, she was diag­nosed now as having ovarian cyst and planned for laparoscopic cystectomy. Systemic examination was within normal limits. Pre-operative investigation re­vealed: Hb-6.5 gm/dL, PCV-31.9%, WBC- 7620 cells/dL, platelet-317000/dL, PT- 14/12 seconds (con­trol/test), aPTT-52.3/30 seconds(control/test), Clot solubility test-normal, Factor VIII assay-6% of nor­mal, Bleeding time>15 sec, PE-3 availability with ADP at 0 min and 20 min were within normal limits (patient/ control - 22/24, 22/17); Platelet aggregation-normal, Ristocetin- absent but corrected with normal plasma. Liver, renal function tests and serum electrolytes were within normal limits. Pre-operatively 2 units of RBC were transfused.

Balanced general anaesthesia with tracheal intu­bation was planned. Pre-induction, patient was trans­fused with cryoprecipitate 10units. In the operating room, monitoring included ECG, pulse oximetry (SpO 2 ), capnography (EtCO 2 ), airway pressure, noninvasive blood pressure (NIBP). Anaesthesia was induced with fentanyl (2mcg.kg -1 ), propofol (2mg.kg -1 ) and vecuronium bromide 6 mg was used to facilitate tra­cheal intubation and maintained with isoflurane and ni­trous oxide in oxygen along with neuromuscular moni­tor guided vecuronium top ups. Analgesia was provided with morphine (100 mcg.kg -1 ). Intra-operative blood loss was approximately 200 ml. Residual neuromuscu­lar blockade was reversed and after return of sponta­neous respiration, trachea extubated.

Patient was shifted to recovery room and NIBP, SpO 2 and ECG monitors were attached. She was ob­served for any blood collection in the drain tube which was within acceptable limits. Post-operatively analge­sia was maintained with morphine PCA (basal infusion of 0.5 mg.hour -1 , bolus of 1.5 mg with lockout interval of 20 minutes). All intra-muscular injections were avoided. She was transfused with cryoprecipitate/FFP till 5th day and injection Tranexamic acid 500 mg 8 hourly was administered. Postoperative serum coagu­lation studies were within normal limits. She was dis­charged on 6 th post-operative day uneventfully.


   Discussion Top


vWD, first described by Erik von Willebrand in 1926, is the most common inherited autosomal domi­nant bleeding disorder in humans with prevalence of 0.1-1% [2] . vWF, a large multimeric glycoprotein syn­thesized in endothelial cells and megakaryocytes medi­ates platelet adhesion and thrombus formation at sites of vascular injury, and serves as a carrier for procoagulant factor VIII (FVIII) [1],[3] .

Clinically vWD is divided into three major types, and provides the basis for appropriate therapy. [4]

Type I, commonest form (90%), is characterized by a quantitative decrease in all vWF multimers. The FVIII may also be decreased but the prothrombin time (PT) and partial thromboplastin time (PTT) are usually normal. Typically, the bleeding time (BT) is prolonged, but it may be normal in some individuals despite a sig­nificant bleeding history. 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 is characterized by either decreased or absent vWF from the plasma.

Type III is the most severe and rarest (<1%) form of vWD, the entire vWF molecule is undetectable. The VIII C is also undetectable and PTT will be consis­tently abnormal.

There is no specific age of onset of the disease 2 . Patients usually have a dual bleeding defect involving superficial (cutaneous and mucous membrane) bleed­ing and deep tissue (muscle and joint) bleeding. Epistaxis (60%), menorrhagia (35%), easy bruising (40%), and gingival bleeding (35%) are cardinal features. Clinical symptoms also include excessive mucocutaneous bleed­ing from post surgical (20%), post dental (50%), bleed­ing from traumatic wounds (35%), postpartum hemor­rhage (25%) and gastrointestinal bleeding (10%) which can be life-threatening or even fatal [5] .

The coagulation screening tests should include complete blood count, platelet count, PT and BT. PTT reflects the VIIIC portion of the Factor VIII complex rather than the vWF.

In deciding upon treatment in vWD it is important to consider tha nature of the bleeding, the FVIII and vWF levels and the vWD subtype, previous bleeding history and response to treatment [6] . 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, which raises the Factor VIIIC level by 15­20%. 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.

Fresh frozen plasma (FFP) corrects the abnor­mality similar to cryoprecipitate, but administration may be associated with circulatory volume overload. Usu­ally 20 ml.kg -1 FFP every eight hours will control clini­cal bleeding.

Transfused platelets do not correct the BT but may be required in addition to cryoprecipitate in a patient who is thrombocytopenic (<50-80 x 109 L-1) secondary to blood loss or plasma dilution during surgery. [6]

Desmopressin (DDAVP) releases vWF and fac­tor VIII coagulant from storage sites [7] . The exact method by which DDAVP reduces the bleeding time in vWD is not known [1] . Excessive perioperative fluids should be avoided, and if fluid retention occurs (synthetic ana­logue of anti-diuretic hormone), low doses of furo­semide (0.25-0.50 mg.kg -1 ) will produce an appropri­ate diuresis. [3] DDAVP is most effective in patients with Type I von Willebrand's disease, produces a variable response in patients with Type II disease, and a poor response in patients with Type III disease. Since the classification of the patient preoperatively is often un­known, it may be necessary to administer DDAVP by slow i.v. infusion of 0.3 mcg.kg -1 over 20 min. and to re-check the coagulation studies for its response. [7] Other potential side effect of DDAVP is hyponatremia. [6]

vWF containing factor VIII concentrates is rec­ommended in vWD that do not respond to DDAVP. [2]

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 [5],[6] . We also considered this therapy in the perioperative management of our patient.

The surgeons and anaesthesiologists who perform procedures in patients with vWD have to keep in mind the armamentarium available to combat perioperative bleeding. Antiplatelet drugs are avoided and fever and/ or infections should be well controlled before surgery. During perioperative period of a major surgery, factor VIII activity and von Willebrand factor (vWF) activity should be kept at adequate levels (factor VIII: 105­150%; vWF: 65-225%) [7] . Intramuscular injections are avoided and rectal medications may be preferred [8] . 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. Rapid bleeding in the cutaneous surgical field is usually due to poor control of severed vessels, whereas disorders of hemostasis tend to mani­fest with recalcitrant slow oozing diffusely within the operative site [5] . Therefore, careful dissection, bipolar electrocautery, or laser/harmonic dissection have been proposed [1] . In our case too, we preferred for minimally invasive procedure in view of previous episode of prob­lem of surgical bleeding. Moreover the risk of nasal/ urethral bleeding during Ryle's tube and foley's catherter insertion should be kept in mind, as it is usually required for most of the laparoscopic surgeries. Oral route for Ryle's tube may preferred.

In summary, patients with vWD do not carry an increased operative risk during elective procedures if appropriate prophylactic and corrective therapy is ad­ministered. Although the administration of cryoprecipi­tate and other blood products has traditionally been the cornerstone of treatment for vWD, the recent de­velopment of DDAVP for clinical use may provide an effective alternative to replacement therapy with blood products. Using a careful multidisciplinary approach, excellent hemostasis can be achieved in patient with vWD. Further laparoscopic procedures in such patients are the safer alternative for all kind of gynecologic surgeries.

 
   References Top

1.Victor JY, Prim MP, et al. Otolaryngologic surgery in children with von Willebrand disease. Arch Otolaryngol Head Neck Surg 2002;128:1365-68.  Back to cited text no. 1      
2.Cameron CB, Kobrinsky N. Perioperative management of patients with von Willebrand's disease. Can J Anesth 1990; 37:341-7.  Back to cited text no. 2      
3.Franchini M, Rossetti G, Tagliaferri A, Pattacini C, Pozzoli D, Lippi G, Manzato F. Efficacy and safety of factor VIII/ von Willebrand factor concentrate (Haemate-P®) in pre­venting bleeding during surgery or invasive procedures in patients with von Willebrand's disease. Haematologica/journal of hematology 2003; 88:1279-82.  Back to cited text no. 3      
4.Jeroen CJ, Eikenboom I, Pemila T, Marion VV, Heering JJ. Acquired von Willebrand syndrome: Diagnostic prob­lems and therapeutic options. American Journal of He­matology 2007;82:55-58.  Back to cited text no. 4      
5.Aimee LL, William CH, Griest A. Perioperative manage­ment of von Willebrand disease in dermatologic surgery. Dermatol Surg 2007;33:403-409.  Back to cited text no. 5      
6.Pasi KJ, Collins PW, et al. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization.. Haemophilia 2004; 10:218-231.  Back to cited text no. 6      
7.Gerling V, Lahpor JR, Buhrel W. Peri-operative manage­ment of an adult patient with type 2N von Willebrand's disease scheduled for coronary artery bypass graft. Ana­esthesia 2007; 62:405-408.  Back to cited text no. 7      
8.Leo TK, Bruce LG, Mathew TP. Orthopedic manifesta­tions and management of patients with von Willebrand Disease. Orthopedics 2008;31:263-68.  Back to cited text no. 8      




 

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