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Protrusio acetabuli in Marfan's syndrome
S R Yule1, E E Hobson, J C Dean
1Department of Radiology, Aberdeen Royal Infirmary, Foresterhill, UK.
Clinical Radiology
|March 2, 1999
Summary
Marfan syndrome, a connective tissue disorder, often involves skeletal issues like protrusio acetabuli. Pelvic X-rays are recommended only when protrusio acetabuli findings might change a Marfan syndrome diagnosis.
Area of Science:
- Medical Genetics
- Radiology
- Orthopedics
Background:
- Marfan syndrome is an autosomal dominant connective tissue disorder impacting cardiovascular, ocular, and skeletal systems.
- Revised diagnostic criteria emphasize skeletal involvement, including protrusio acetabuli, as a major indicator.
Purpose of the Study:
- To evaluate the diagnostic utility of protrusio acetabuli in Marfan syndrome using pelvic X-rays.
- To determine if routine pelvic X-rays are necessary for diagnosing Marfan syndrome based on protrusio acetabuli.
Main Methods:
- Analysis of pelvic X-rays from 15 Marfan syndrome patients and 15 controls using Kulman's method.
- Assessment of protrusio acetabuli prevalence and its impact on diagnosis under revised criteria.
Main Results:
- Protrusio acetabuli was identified in 47% of Marfan syndrome patients versus 7% of controls (P = 0.035).
- The presence of protrusio acetabuli would alter the diagnosis in only one of 15 Marfan syndrome patients.
- Acetabular line assessment offers a simple and reliable radiological method for detecting protrusio.
Conclusions:
- Pelvic X-rays for protrusio acetabuli should be reserved for cases where findings may alter the Marfan syndrome diagnosis.
- Acetabular line assessment is a reliable radiological technique for evaluating protrusio acetabuli.