Anaesthetic Management of a Patient with Von Willebrand’s Disease with Cerebral Cavernoma

Gauri Sankhe*, Anil Parakh**

Introduction
Anaesthesia management of a rare patient with temporo-occipital cavernoma associated with Von Willebrand’s disease and difficult intubation, undergoing extra cranial temporo-occipital mass excision.

The temporo-occipital cavernoma was extending to the right side of the neck involving posterior auricular region, making the neck movement impossible. The associated restricted mouth opening and protruding teeth made the intubation difficult.

Case Report
A 26 year old male patient weighing 39 kg suffering from Von Willebrand’s disease was posted for excision of temporo-occipital mass had come for preanaesthetic evaluation.

The patient had discharging sinus over the mass and did not have any other significant medical history Pt was diagnosed to be V W disease but was refused surgery by many surgeons.

With the consultation of haematologist the patient was managed with inj vasopressin Tranaxemic acid and was receiving the same drugs and cryoprecipitate.

Pt was poorly built and nourished pulse 64/min, B.P- 110/80.CVS, RS, CNS - Normal
Pt had no active bleeding sites.
Baseline investigations CBC, Platelets count and coagulation studies were done.
Bleeding and Clotting Time- Normal
Prothrombin Time and APTT were prolonged
Chest X-ray NAD
ECG within normal limits.
All medications were continued till morning of surgery
Cryoprecipitate and FFP were kept ready
Patient received Inj Glycopyrrolate I.V as premedication.
Pt was taken in operation room.
BP 120/84; pulse 100/min.

After infusion of 20 bags of Cryoprecipitate, Left Subclavian Vein was catheterized.
As the tumour was extending in Temporo-Occipital area and Postero-lateral part of the neck, it was restricting all the neck movements hence difficult intubation was predicted (MPC Grade- 4). We planned to intubate the patient awake, with the help of the Fibre-Optic Laryngoscope. Xylocaine viscous gargles and xylocaine spray was done for awake fibreoptic intubation

Emergency tracheostomy kit/ suction / blood/ cryoprecipitate /FFP kept ready

Awake fibreoptic intubation nasally was performed

Anaesthesia was induced with IV Inj fentanyl 80 µg / Thiopentone 250 mg and vecuronium 4 mg.

Anaesthesia maintenance- Air + Oxygen 2:2, Isoflurane - 0.4-1%

Surgery was carried out in supine position with head up reverse trendelenburg tilt continuous infusion of atracurium and fentanyl was used intraoperatively. Surgery lasted for 6 hrs

IV fluids were administered judiciously

IV Fluids 1.5 L colloid 2.5 lit lactated ringers, 2.5 lits normal saline - BLOOD- 2 Units, FFP- 4 Bags

As soon as surgical incision given – there was sudden loss of blood and subsequent hypotension, which was responded to crystalloids / Blood and Inj Dopamine 5 ug/kg/min infusion given.

After haemodynamic stability surgery proceeded and BP was maintained by above measures.
At the end of the operation, patient was shifted to Neurosurgical -ICU, elective ventilation with SERVO 900-C Ventilator on volume control for 12 hrs.

After ABG and withdrawal of inotropic support, Patient was extubated next morning.

After 1 hr of extubation saturation started falling rapidly, emergency intubation by conventional method was tried but failed.

mergency tracheostomy was performed in the operation theatre by surgical team.

Post procedure patient received Cryoprecipitate / FFP-6 BAGS.

BT, CT and PT were performed 12 hourly. HB 10 gm/dl.

Diagnosis was suggested by patient history and demonstration of prolonged PT despite normal platelet counts.

Diagnosis was suggested by patient history and demonstration of prolonged PT despite normal platelet counts.

Discussion
Von-Willebrand’s disease (vWD) is the most common inherited bleeding disorder, inherited as an autosomal dominant trait.1,5-7

Types 1 and 3 vWD are associated with relative or absolute quantitative defects in the protein, respectively. Type 1 vWD accounts for 70% of all cases and is likely to temporarily improve in parturients as a result of an increase in vWF and factor VIII with pregnancy.2 Type 2 vWD has qualitative abnormalities and comprises 20-30% of all vWD diagnoses. A unique subtype, type 2B, accounts for less than 20% of all type 2 vWD and is characterized by an increased affinity of vWF for platelet glycoprotein Ib, resulting in spontaneous binding and clearance of both vWF and platelets.3,4

This coagulation disorder is caused by deficit or defective amount is of Von Willebrand’s factor (vWF) in the patients plasma.1,2 The function of Von Willebrand’s factor is to facilitate platelet adhesion to exposed endothelium and to act as a carrier for factorVIII.1 The normal plasma vWF level is 10 mg/ml. In mild cases bleeding occurs only after surgery or trauma. More severely affected patients have spontaneous epistaxis or oral mucosal, gastrointestinal or genitourinary bleeding.5-8 There are three major types of the vWD. Patients with type I disease, the most common abnormality, have a mild to moderate decrease in plasma vWF upto 50% activity or 5 mg/ml. The variant form of vWD (type II disease ) is much less common and is characterized by normal or near normal levels of the dysfunctional proteins. This forms intravascular platelet aggregates, which are rapidly cleared from the circulation causing mild cyclic thrombocytopenia. Levels of vWF antigen and factor VIII usually remain normal. Type III disease patients have severe mucosal bleeding, no detectable vWF antigen or activity and may have sufficiently low factor VIII level to have occasional haemoarthrosis like haemophiliacs.8,9

The most diagnostic laboratory finding in vWD is the combination of (1) prolonged bleeding time, (2) a reduced plasma concentration of vWF, (3) a parallel reduction in ristocetin co factor activity and (4) reduced factor VIII activity.5-8,11

The appropriate treatment of vWD consists of two therapeutic options. The first option involves the use of Cryoprecipitate, which is a plasma fraction enriched in vWF and is an appropriate treatment for vWD. The dose of Cryoprecipitate is 1 bag/ 10 kg daily.5-7,9,11 Each bag of Cryoprecipitate contains 30 ml and 4-6 bags are required to raise the factor VIII by 3%.12 During surgery or major trauma patient should receive 10 bags of Cryoprecipitate. This should be continued twice daily for 48-72 hours to ensure optimum haemostasis.

The second option is 1-desamino-8-D arginine, Vasopressin (dDAVP), a vasopressin analogue, having minimal blood pressure elevating and fluid retaining properties and it raises the plasma vWF level in normal individual and patient with mild vWD.13 The usual regimen is 0.3 mg/kg. IV in 50 ml of isotonic saline over 15-20 minutes. Side effects are more prominent if the total dose exceeds 24 mg. The dDAVP acts on vasopressin receptors and mediate water intoxication hence should not be used in acute renal failure.20 Although desmopressin, a vasopressin analogue that raises plasma vWF, is a common therapy for vWD, in type 2B vWD it can increase the abnormal vWF, resulting in further binding of platelets, depletion of high molecular weight multimers, and potentially greater bleeding.10,14-16 As such, the preferred therapy for this specific vWD subtype is factor VIII plasma concentrate (FVIII) (Humate-Por Alphanate; Grifols, Los Angeles, CA), which, although FDA approved for haemophilia and not vWD, provides the following corrective components: 2.5 IU vWF:RCo, 1 IU of FVIII, and a near-normal count of high molecular weight multimers.1,14,17,18

In the pre-operative preparation of vWD disease, therapy should begin 24 hours before the surgery in order that “newly” produced factor VIII may raise the factor endogenously. However they must be tested for an adequate response prior to surgery and vWF levels must be closely monitored during therapy. The Desmopressin is the treatment of choice in most of the patients with type I and type II vWD, who accounts for 60-70% of cases. In the severe cases plasma concentrate containing factor VIII and vWF should be given, But this does not correct the Bleeding time defect and Desmopressin or Platelet concentrates can be used as an adjuvant treatment.21

In patients not responding to Desmopressin, replacement with plasma –derived concentrates is the treatment of choice.22 In developing countries like ours Cryoprecipitate is the safe alternative. It is safe and effective alternative to the use of blood products in vWD.23,24

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