Protusión acetabular en el síndrome de Marfan. (Inglés)

Samuel Van de Velde, MD1, Ramona Fillman, MHA, PT2 and Suzanne Yandow, MD2

1 University Hospital Pellenberg, Weligerveld 1, 3212 Pellenberg, Belgium.

Investigation performed at Shriners Hospitals for Children, Honolulu, Honolulu, Hawaii

The authors did not receive grants or outside funding in support of their research for or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

Marfan syndrome is an autosomal dominant disorder of connective tissue, with ocular, skeletal, and cardiovascular manifestations. Protrusio acetabuli is a criterion for the diagnosis of Marfan syndrome.

Prolonged protrusio acetabuli may result in secondary osteoarthritic changes in the hip joint.

Radiographic criteria for protrusio acetabuli include an abnormally positioned acetabular line, a center-edge angle of Wiberg of >40°, and crossing of the teardrop by the ilioischial line.

In a skeletally immature patient with Marfan syndrome in whom the triradiate physis of the acetabulum is still open, closure of the triradiate physis can interrupt and decrease the progression of the deformity. In older patients, valgus intertrochanteric osteotomy and eventually total hip arthroplasty are the only methods available for correction of the protrusio acetabuli.

The Journal of Bone and Joint Surgery (American). 2006;88:639-646.