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J Am Acad Orthop Surg, Vol 17, No 9, September 2009, 572-581.
© 2009 the American Academy of Orthopaedic Surgeons
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Marfan Syndrome

LCDR Eric D. Shirley, MD, MC, USN and Paul D. Sponseller, MD

Dr. Shirley is Attending Pediatric Orthopaedic Surgeon, Naval Medical Center Portsmouth, Portsmouth, VA. Dr. Sponseller is Professor, Chief of the Pediatric Division, and Vice Chairman, Department of Orthopaedic Surgery, The Johns Hopkins Hospital, Baltimore, MD.

Dr. Sponseller or a member of his immediate family serves as a board member, owner, officer, or committee member of the Pediatric Orthopaedic Society of North America; has received royalties from Globus Medical; serves as a paid consultant to or is an employee of DePuy; and has received research or institutional support from DePuy. Neither Dr. Shirley nor a member of his immediate family has received anything of value from or owns stock in a commercial company or institution related directly or indirectly to the subject of this article.

The views expressed in this article are the views of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the US Government.

Reprint requests: Dr. Sponseller, c/o Elaine P. Henze, Department of Orthopaedic Surgery, Johns Hopkins Bayview Medical Center, #A665, 4940 Eastern Avenue, Baltimore, MD 21224-2780.

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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Copyright © 2009 by the American Academy of Orthopaedic Surgeons.