Document Detail


Marfan syndrome.
MedLine Citation:
PMID:  19726741     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
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.
Authors:
Eric D Shirley; Paul D Sponseller
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of the American Academy of Orthopaedic Surgeons     Volume:  17     ISSN:  1067-151X     ISO Abbreviation:  -     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-09-03     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9417468     Medline TA:  J Am Acad Orthop Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  572-81     Citation Subset:  IM    
Affiliation:
Naval Medical Center Portsmouth, VA, USA.
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