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

Yoshikazu Yamaguchi, Walter Samora, Jan E. Klamar, Dmitry Tumin, Joseph D. Tobias


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


Spinal fusion; Kyphoscoliosis; Marfan syndrome; Anticoagulation

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