A Case Report of Marfan Syndrome under General Anesthesia.
10.4097/kjae.1993.26.5.1055
- Author:
Dae Woo KIM
1
;
Yong Gul LIM
Author Information
1. Department of Anesthesiology, Catholic University Medical College, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Marfan syndrome;
General anesthesia
- MeSH:
Anesthesia, General*;
Cesarean Section;
Collagen Type I;
Connective Tissue;
Female;
Heart Murmurs;
Humans;
Hypertension;
Intubation;
Joints;
Marfan Syndrome*;
Mitral Valve Prolapse;
Pregnancy;
Prevalence
- From:Korean Journal of Anesthesiology
1993;26(5):1055-1058
- CountryRepublic of Korea
- Language:Korean
-
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.
