Combined severe pectus excavatum correction and aortic root replacement in Marfan's syndrome. 2006

Kalyana C Javangula, and Timothy J P Batchelor, and Osama Jaber, and Kevin G Watterson, and Kostas Papagiannopoulos
Department of Cardiothoracic Surgery, Leeds General Infirmary, Leeds, United Kingdom. kalyanachakravarthi@hotmail.com

A 23-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia and severe pectus excavatum. A submammary skin incision approach followed by bilateral subperichondrial resection of abnormal costal cartilages was performed. The left intercostal muscles and perichondrial sheaths were divided 2 inches lateral to the sternum in a parasternal fashion to place the retractor. The aortic root was replaced with a 23-mm St. Jude's composite graft (St. Jude Medical, Inc, St. Paul, MN). Chest wall reconstruction was completed with a high sternal osteotomy and support of the sternum was made with Gortex strips (W.L. Gore & Associates, Inc, Flagstaff, AZ). The patient made an uneventful recovery.

UI MeSH Term Description Entries
D008297 Male Males
D008382 Marfan Syndrome An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2. Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D010027 Osteotomy The surgical cutting of a bone. (Dorland, 28th ed) Osteotomies
D004108 Dilatation, Pathologic The condition of an anatomical structure's being dilated beyond normal dimensions. Ectasia,Dilatation, Pathological,Dilatations, Pathologic,Dilatations, Pathological,Pathologic Dilatation,Pathologic Dilatations,Pathological Dilatation,Pathological Dilatations
D005660 Funnel Chest A developmental anomaly in which the lower sternum is posteriorly dislocated and concavely deformed, resulting in a funnel-shaped thorax. Pectus Excavatum,Chest, Funnel,Chests, Funnel,Excavatum, Pectus,Funnel Chests
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001021 Aortic Valve The valve between the left ventricle and the ascending aorta which prevents backflow into the left ventricle. Aortic Valves,Valve, Aortic,Valves, Aortic
D001022 Aortic Valve Insufficiency Pathological condition characterized by the backflow of blood from the ASCENDING AORTA back into the LEFT VENTRICLE, leading to regurgitation. It is caused by diseases of the AORTIC VALVE or its surrounding tissue (aortic root). Aortic Incompetence,Aortic Regurgitation,Aortic Valve Incompetence,Regurgitation, Aortic Valve,Incompetence, Aortic,Incompetence, Aortic Valve,Insufficiency, Aortic Valve,Regurgitation, Aortic
D013249 Sternum A long, narrow, and flat bone commonly known as BREASTBONE occurring in the midsection of the anterior thoracic segment or chest region, which stabilizes the rib cage and serves as the point of origin for several muscles that move the arms, head, and neck.

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