Analysis of Protrusio Acetabuli Using a CT-based Diagnostic Method in Korean Patients with Marfan Syndrome: Prevalence and Association with Other Manifestations.
10.3346/jkms.2015.30.9.1260
- Author:
Kwang Jin CHUN
1
;
Jeong Hoon YANG
;
Shin Yi JANG
;
Seung Hwa LEE
;
Hye Bin GWAG
;
Tae Young CHUNG
;
June HUH
;
Chang Seok KI
;
Kiick SUNG
;
Seung Hyuk CHOI
;
Sung Mok KIM
;
Yeon Hyeon CHOE
;
Duk Kyung KIM
Author Information
1. Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. dukkyung.kim@gmail.com
- Publication Type:Original Article ; Research Support, Non-U.S. Gov't
- Keywords:
Protrusio Acetabuli;
Marfan Syndrome;
FBN1 Mutation;
Computed Tomography
- MeSH:
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
- From:Journal of Korean Medical Science
2015;30(9):1260-1265
- CountryRepublic of Korea
- Language:English
-
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.
