Protrusio Acetabuli in Marfan Syndrome

Abstract

The authors reviewed all patients in Shriners Hospital for Children, Honolulu, diagnosed with hyperlaxity of the joints (131 in total) for Marfan syndrome. The revised diagnostic criteria for Marfan syndrome were used for inclusion in this study. In the patients diagnosed with Marfan syndrome (n = 13), the incidence (n = 9) and indication for surgical intervention (n = 4) of protrusio acetabuli were evaluated by retrospective radiographic review, clinical findings, and family history. For the identification of protrusio acetabuli, the presence of an abnormal acetabular line position (>1 mm medial to the ilioischial line in boys, >3 mm in girls), a center-edge angle of Wiberg of 40 degrees or more, or crossing of the teardrop by the ilioischial line was used. The authors conclude that serial radiographic investigations, reinforced with a well-documented family history of symptomatic protrusio acetabuli, are needed to rule out progression of the deformity. When progression of the deformity is suggested in a skeletally immature Marfan patient, surgical intervention by closure of the triradiate cartilage is indicated, regardless of the absence of hip complaints or limitation of range of motion.