Korean J Anesthesiol.  1993 Oct;26(5):1055-1058. 10.4097/kjae.1993.26.5.1055.

A Case Report of Marfan Syndrome under General Anesthesia

• Kim DW
• Lim YG

• KMID: 1690066
• DOI: http://doi.org/10.4097/kjae.1993.26.5.1055

Abstract

We experienced a case of Cesarean section of Marfan syndrome under the general anesthesia. Marfan syndrome is an autosomal dominant trait characterized by generalized defect of connective tissue, which is mediated by a structural deficiency in Type 1 collagen. Its prevalence has been estimated at 1 to 5 in 100,000. Expression of the trait is variable but the most common diagnostic signs are disproportionate length of long bones, hypermobility of joints, prolapsed lens, and cardiac murmurs, most often the systolic click-murmur of mitral valve prolapse. The importance in anesthetic management of these patients should be chosen to prevent any hypertension in response to intubation and surgery, and handled very gently, with minimal stress placed on the connective tisues.

Keyword

Marfan syndrome; General anesthesia

MeSH Terms

Anesthesia, General*
Cesarean Section
Collagen Type I
Connective Tissue
Female
Heart Murmurs
Humans
Hypertension
Intubation
Joints
Marfan Syndrome*
Mitral Valve Prolapse
Pregnancy
Prevalence
Collagen Type I