Anesthetic Management of an Adolescent With Marfan Syndrome During Anterior Spinal Fusion

Abstract

Marfan syndrome (MFS) is an autosomal dominant disorder resulting from a mutation of the gene encoding fibrillin-1 (FBN1) on chromosome 15. Fibrillin-1 is a matrix glycoprotein essential for the formation of microfibrils that regulate the formation and repair of connective tissue throughout the body. This primary cellular defect results in a multisystem disorder of connective tissue. Given the multisystem involvement of MFS, anesthetic care is frequently required during surgical or orthopedic procedures. We present a 15-year-old adolescent who required anesthetic care during spinal fusion. The perioperative implications of MFS are reviewed and options for anesthetic care presented.




J Med Cases. 2018;9(11):366-370
doi: https://doi.org/10.14740/jmc3181