A 27-year-old man is referred for preoperative evaluation prior to elective craniotomy to remove a benign brain tumor. He was diagnosed with type 1 von Willebrand disease at age 17 and has intermittent epistaxis and easy bruising. Last year he had a prolonged and severe episode of epistaxis that required cautery and nasal packing. He has no other medical problems or surgeries. The patient has no known drug allergies.
Vital signs are within normal limits. Skin examination shows an area of ecchymosis on the left thigh. Neurologic examination is normal.
Laboratory results are as follows:
The patient has blood type A, and von Willebrand factor (VWF) antigen and activity level are pending.
Which of the following is the most effective strategy for decreasing this patient's risk of perioperative bleeding?

Question

A 27-year-old man is referred for preoperative evaluation prior to elective craniotomy to remove a benign brain tumor.  He was diagnosed with type 1 von Willebrand disease at age 17 and has intermittent epistaxis and easy bruising.  Last year he had a prolonged and severe episode of epistaxis that required cautery and nasal packing.  He has no other medical problems or surgeries.  The patient has no known drug allergies.
Vital signs are within normal limits.  Skin examination shows an area of ecchymosis on the left thigh.  Neurologic examination is normal.
Laboratory results are as follows:
 The patient has blood type A, and von Willebrand factor (VWF) antigen and activity level are pending.
Which of the following is the most effective strategy for decreasing this patient's risk of perioperative bleeding?

Quizplus · Accepted Answer

11ec193a_9836_84ed_853d_a9ef96e7f7fa_MD0002_00 Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Type 1 VWD accounts for 75% of cases and is marked by a quantitive deficiency of von Willebrand factor (VWF), leading to low VWF antigen, VWF activity, and factor VIII levels. Patients with blood type O have VWF levels that are up to 30% lower than those with blood types A, B, or AB. Most patients (99%) with VWD have mild or asymptomatic disease and never seek clinical attention. However, clinically significant skin and mucosal bleeding can occur. Treatment of bleeding complications is typically achieved with: Desmopressin (DDAVP) - an antidiuretic hormone analogue that increases VWF and factor VIII levels and used primarily for mild bleeding episodes or prior to minor procedures. DDAVP is effective for mild/moderate type 1 VWD, but those with severe bleeding (such as this patient who required cauterization) often do not respond sufficiently (Choice C).VWF preparations - used in severe bleeding episodes in those who do not respond to desmopressin and perioperatively for invasive surgeries (such as neurosurgery). Several different preparations, including intermediate-purity factor VIII concentrates, high-purity VWF concentrate, and recombinant VWF, are available. Desmopressin (DDAVP) - an antidiuretic hormone analogue that increases VWF and factor VIII levels and used primarily for mild bleeding episodes or prior to minor procedures. DDAVP is effective for mild/moderate type 1 VWD, but those with severe bleeding (such as this patient who required cauterization) often do not respond sufficiently (Choice C). Desmopressin (DDAVP) - an antidiuretic hormone analogue that increases VWF and factor VIII levels and used primarily for mild bleeding episodes or prior to minor procedures. DDAVP is effective for mild/moderate type 1 VWD, but those with severe bleeding (such as this patient who required cauterization) often do not respond sufficiently (Choice C). VWF preparations - used in severe bleeding episodes in those who do not respond to desmopressin and perioperatively for invasive surgeries (such as neurosurgery). Several different preparations, including intermediate-purity factor VIII concentrates, high-purity VWF concentrate, and recombinant VWF, are available. VWF preparations - used in severe bleeding episodes in those who do not respond to desmopressin and perioperatively for invasive surgeries (such as neurosurgery). Several different preparations, including intermediate-purity factor VIII concentrates, high-purity VWF concentrate, and recombinant VWF, are available. In this patient who has had severe bleeding and is preparing for neurosurgery, administration of VWF preparations will likely lead to VWF activity levels close to 100% and is the most effective strategy to prevent perioperative bleeding. (Choice A) Aminocaproic acid is an anti-fibrinolytic agent that is sometimes used for patients with VWD who have menorrhagia or mild mucosal bleeding; it is usually used in combination with other therapies. (Choice B) Cryoprecipitate is not routinely used for VWD due to the risk of viral transmission; VWF preparations are the preferred therapy for more serious bleeding or prior to a major procedure. (Choice D) Platelets contain a small proportion of total blood VWF and can be used in patients who have refractory bleeding with VWF concentrates. Educational objective: Type 1 von Willebrand disease (VWD) is a quantitative disorder of von Willebrand factor (VWF) and can range from mild to severe disease. VWF concentrates are used perioperatively in severe VWD to decrease bleeding risk in both major and minor surgeries. Levels should be checked during this period to ensure adequate factor replacement. __________ References: How I treat von Willebrand disease. (http://www.ncbi.nlm.nih.gov/pubmed/19474451) von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). (http://www.ncbi.nlm.nih.gov/pubmed/18315614)

A 27-Year-Old Man Is Referred for Preoperative Evaluation Prior to Elective