Perioperative Management of a Child with von Willebrand Disease Undergoing Surgical Repair of Craniosynostosis: Looking at Unusual Targets

Abstract

We report the successful management of a craniosynostosis repair in a child with severe Type I von Willebrand disease diagnosed during the preoperative assessment and treated by coagulation factor VIII and ristocetin cofactor. Collaboration among the anesthesiologist, the neurosurgeon, the clinical pathologist, and the pediatric hematologist is important for successful management.

Elevated prothrombin time on routine preoperative laboratory results in a healthy infant undergoing craniosynostosis repair: Diagnosis and perioperative management of congenital factor VII deficiency

“…In the literature to date, there are several syndromes and genetic mutations associated with craniosynostosis but none with an association with Factor VII deficiency or other coagulation disorders. In 2009, a case report by Maquoi and colleagues described the diagnosis of Von Willebrand’s disease as an incidental finding in a child undergoing craniosynostosis repair during the preoperative visit due to a positive family history—no direct association has been reported [6] .…”