| Marfan syndrome. | |
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MedLine Citation:
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PMID: 19726741 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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Marfan syndrome is a variable autosomal dominant disorder; most cases result from mutations of fibrillin-1. Diagnosis is guided by the Ghent nosology. The condition may manifest in the cardiovascular and ocular systems. Musculoskeletal manifestations include scoliosis, dural ectasia, protrusio acetabuli, and ligamentous laxity. Compared with patients with idiopathic scoliosis, patients with Marfan syndrome tend to have scoliosis that progresses at a faster rate and is more resistant to bracing; undergo scoliosis surgery complicated by greater blood loss, pseudarthrosis, and additional curvature; and have more frequent occurrences of dural ectasia, which may cause headaches, leg pain, or perineal pain. Protrusio acetabuli may result in hip joint arthritis and may require valgus osteotomy or total hip arthroplasty. |
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Authors:
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Eric D Shirley; Paul D Sponseller |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The Journal of the American Academy of Orthopaedic Surgeons Volume: 17 ISSN: 1067-151X ISO Abbreviation: - Publication Date: 2009 Sep |
Date Detail:
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Created Date: 2009-09-03 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9417468 Medline TA: J Am Acad Orthop Surg Country: United States |
Other Details:
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Languages: eng Pagination: 572-81 Citation Subset: IM |
Affiliation:
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Naval Medical Center Portsmouth, VA, USA. |
Export Citation:
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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