von Willebrand's disease (vWD) is an inherited bleeding disorder due to deficiency or abnormality of von Willebrand factor (vWF).
The majority of patients with vWD tend to have clinical defects that are only associated with superficial bleeding. There is no specific age of onset of the disease, and a history of cutaneous or mucosal bleeding which includes epistaxis, menorrhagis, easy bruising or failure to clot after dental extraction
DDAVP (Deamino-8-D-arginine vasopressin), a synthetic analogue of anti-diuretic hormone, is the specific agent for the treatment of vWD and is effective to increase the excretion of the intrinsic factor from vascular endotherial cell.
A 46 year-old-male with vWD underwent total thyroidectomy and radical neck disection for cancer of thyroid under general anesthesia.
Before skin incision, 0.3μg/kg/ml of DDAVP was infused. Increase of vWF and VIII factors to the level of twice of preoperative value were obtained 20 minutes later.
During operation, urinary output decreased as expected, however, diuretics was not given to avoid, electolytes imbalance. Careful measurements of CVP, electolytes, CBC, and blood gases were required to maintain fluid balance and to control hemor-rhage. During 8 hours anesthesia, blood loss was 473ml, urinary volume was 151 ml, and fluid given was 1, 500ml.
Postoperative course was uneventful except transient blood pressure falling treated by volume loading. On the second operative day, normal urinary output was resumed.
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