Perioperative Management of a Patient with Hereditary Angioedema undergoing Lumbar Interbody Arthrodesis - Abstract
Hereditary angioedema (HAE) is a rare genetic disorder resulting from an inherited deficiency or dysfunction of the C1-esterase inhibitor. It is characterized by recurrent and self-limiting angioedema episodes, most often affecting the skin or the mucosal tissues of the upper respiratory and gastrointestinal tracts.
Androgens and fresh frozen plasma (FFP) are frequently used as treatment despite multiple, significant side effects. Newer therapies include C1-inhibitor concentrate (C1-INH) and contact system modulators such as ecallantide and icatibant. In addition, C1-INH can also be used for prophylaxis.
We present the case of a 47-year-old male with a previous diagnosis of HAE, underwent elective lumbar interbody arthrodesis under general anesthesia. Following prophylaxis with danazol and C1-INH, surgery was carried out uneventfully with an unremarkable postoperative course. Perioperative prophylaxis to avoid acute attacks and anesthetic management are discussed.