[Corrective surgery for pectus excavatum in patients with Marfan syndrome].
OBJECTIVE
Among 1,967 patients who underwent corrective surgery for pectus excavatum in our department between January 1980 and December 2001, 33 patients diagnosed as having Marfan syndrome were evaluated. We evaluated the clinical problems and outcome of the 33 patients.


RESULTS
The outcome of corrective surgery for pectus excavatum in the 26 patients who underwent a single technique surgery was generally good. Pectus excavatum was complicated by a total of 25 cases of cardiovascular disorder in 14 of 33 (42%) patients. Six patients underwent corrective surgery for pectus excavatum and cardiovascular disorders simultaneously. Three patients also had disorders of lung, which included spontaneous pneumothorax in 2 patients and progressive emphysematous cyst in 1 patient. The 2 patients with spontaneous pneumothorax underwent resection of bullae together with corrective surgery of pectus excavatum, while the patient with progressive emphysematous cyst underwent resection of bullae in a separate surgery after correction of pectus excavatum. Cardiovascular disorders were aggravated in 6 of the 20 patients who could be follow up. A total of 4 (20%) patients died long after surgery.


CONCLUSIONS
We corrected pectus excavatum in 33 patients with Marfan syndrome, and obtained favorable outcomes. The postsurgical outcome depends on progression of lesions of the heart and large vessels, and it is essential to monitor such lesions carefully.
Oyama
K
K
Department of Surgery I, Tokyo Women's Medical University, School of Medicine, Japan.
Adachi
T
T
Kanzaki
M
M
Sasano
S
S
Kuwata
H
H
Yoshida
T
T
Ohnuki
T
T
D000293:Adolescent; D000328:Adult; D002318:Cardiovascular Diseases / Q000209:etiology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005660:Funnel Chest / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000601:surgery*; D019616:Thoracic Surgical Procedures; D016896:Treatment Outcome