[Anesthesia for a patient with Ehlers-Danlos syndrome and factor XIII deficiency]

Abstract

Emergency anesthesia was carried out for a patient with Ehlers-Danlos syndrome (type IV) and factor XIII deficiency. The operation was a ligation of the bleeding right subclavian artery. One month later, a transverse colectomy and splenectomy was performed for a necrotic perforation of the colon, but the wound did not heal completely. The patient died of another perforation of the intestine two months later. Anesthetic management of a patient with Ehlers-Danlos syndrome and factor XIII deficiency was reviewed. Particular problems concerning a genetic disease were also discussed.