J Korean Med Sci.  2015 Sep;30(9):1260-1265. 10.3346/jkms.2015.30.9.1260.

Analysis of Protrusio Acetabuli Using a CT-based Diagnostic Method in Korean Patients with Marfan Syndrome: Prevalence and Association with Other Manifestations

• Chun KJ
• Yang JH
• Jang SY
• Lee SH
• Gwag HB
• Chung TY
• Huh J
• Ki CS
• Sung K
• Choi SH
• Kim SM
• Choe YH
• Kim DK

• KMID: 2344150
• DOI: http://doi.org/10.3346/jkms.2015.30.9.1260

Abstract

A new CT-based diagnostic method of protrusio acetabuli (PA) was introduced. However, prevalence of PA by this method and correlation between PA and other manifestations of Marfan syndrome (MFS) is unknown in Korean MFS patients. This study aimed to investigate the prevalence of PA diagnosed by a CT-based method in Korean patients with MFS, the association of PA with other manifestations of MFS, and the contribution of PA to MFS diagnosis. We retrospectively reviewed the records of 146 MFS patients with the presence of a causative FBN1 mutation and 146 age- and sex-matched controls from a single tertiary care center. All MFS patients underwent a complete assessment of criteria based on the revised Ghent nosology. PA was assessed quantitatively using a CT-based circle-wall distance (CWD) method. PA was diagnosed in 77.4% of patients in the MFS group and in 11.0% of the control group. CWD was significantly different between the two groups (1.50 mm vs. -0.64 mm, P<0.001). The presence of PA did not correlate with the presence of ectopia lentis, aortic root diameter, or history of aortic dissection. The presence of PA did not have a significant impact on the final diagnosis of MFS. Even though the presence of PA does not related to the cardinal clinical features of MFS or influence MFS diagnosis, its presence may be helpful for the suspicion of MFS when aortic dissection or aneurysm is found on CT angiography of the aorta because of the high frequency of PA in MFS patients.

Keyword

Protrusio Acetabuli; Marfan Syndrome; FBN1 Mutation; Computed Tomography

MeSH Terms

Acetabulum/*abnormalities/radiography
Adolescent
Adult
Aged
Aged, 80 and over
Aortic Aneurysm/*epidemiology/radiography
Comorbidity
Female
Humans
Male
Marfan Syndrome/*epidemiology/*radiography
Middle Aged
Prevalence
Reproducibility of Results
Republic of Korea/epidemiology
Risk Factors
Sensitivity and Specificity
Tomography, X-Ray Computed/*methods/*statistics & numerical data
Young Adult

Figure

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  Fig. 1 Diagnostic methods for protrusio acetabuli using plain anteroposterior radiographs of the pelvis and CT images. (A) The center-edge angle (CEA) is formed by a vertical line drawn through the center of the femoral head and a line drawn from the center through the lateral edge of the acetabular roof. A CEA of >50° is considered indicative of protrusio acetabuli. (B) The acetabular-ilioischial distance represents the transverse distance between the ilioischial line (a) and the acetabular line (b). Crossing of the ilioischial line by the acetabular line by > 3 mm medially in men or > 6 mm in women is considered indicative of protrusio acetabuli. (C) Radiographic changes of the teardrop figure in protrusio acetabuli. a, opened; b, closed; c, crossed; d, reversed. (D) CT-based circle-wall distance (CWD) method. A 10 cm radius circle is adapted to the inner acetabular wall of the pelvis. The distance between the line of the circle and the medial most point of the inner pelvic wall of the acetabular fossa was measured. Measurement of CWD is indicated. CT, computed tomography.

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  Fig. 2 Scattergram of CWD in patients with MFS and normal controls. CWD, circle-wall distance; MFS, Marfan syndrome.

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