Congenital Afibrinogenemia: Anaesthetic Implications of a Rare Inherited Coagulation Disorder

Abstract

Congenital afibrinogenemia is a very rare inherited bleeding disorder that results from fibrinogen deficiency and is associated with bleeding manifestations of varying severity. A 21 Year-old, diagnosed case of congenital afibrinogenemia, was admitted to the hospital with blunt injury to both the eyes. He was posted for right eye enucleation. Main modality of treatment during bleeding episodes is transfusion of fibrinogen. Alternatively, cryoprecipitate can also be used for minor bleeding episodes or to maintain a base line fibrinogen level. Team work involving haematologists, surgeons and anaesthetist is needed for good pre-operative preparation, to form a protocol for intraoperative management and for optimum post-operative care. It is mandatory to arrange blood and blood products even for minor surgeries in view of the possibility of excessive blood loss.

Keywords

• congenital afibrinogenemia
• fibrinogen
• cryoprecipitate
• anesthesia