The successful use of recombinant factor VIIa in a patient with vascular-type Ehlers-Danlos syndrome.

Abstract

Vascular-type Ehlers-Danlos syndrome is an inherited connective tissue disorder resulting in an increased risk of serious peri-operative bleeding during surgery for spontaneous organ or vessel rupture. The excessive bleeding may result in coagulopathy, and thus compound the difficulty in securing surgical haemostasis. With the advent of recombinant factor VIIa, a new therapy has become available for the management of intractable surgical bleeding.