| Autor: |
Wang, C S, Steinbach, L S, Campbell, J B, Hayashi, G, Yoon, S T, Johnston, J O |
| Zdroj: |
Skeletal Radiology; Jul1999, Vol. 28 Issue 6, p390-395, 6p |
| Abstrakt: |
Objective: Fibrogenesis imperfecta ossium is an extremely rare disorder that can easily be misdiagnosed. We retrospectively reviewed the clinical and imaging data of three confirmed cases of fibrogenesis imperfecta.Design and Patients: The patients consisted of two men and one woman, ranging in age from 40 to 53 years. Radiography was performed in all the patients. One patient had a 3-year follow-up of the thoracolumbar spine with conventional radiography and thoracolumbar magnetic resonance (MR) imaging. Open biopsy was performed in all cases, confirming the diagnosis of fibrogenesis imperfecta ossium.Results: All our cases demonstrated "fishnet" trabecular pattern by conventional radiographs, and a pelvis radiograph of one patient showed an equivocal sclerosis pattern. Multiple fractures were noted in two patients. A pseudoexostosis was present in the ilium in one patient. Thoracolumbar MR imaging demonstrated diffuse low signal intensity within the medullary space on both T1-weighed and T2-weighted images, except for a region of increased signal intensity in the L1 and L2 vertebral bodies on T2-weighed images due to edema from acute collapse.Conclusions: Although uncommon, fibrogenesis imperfecta ossium should be considered in a previously healthy patient with a combination of progressive bone pain, unexplained fractures, a radiologic pattern of fishnet osteopenia and MR imaging of low signal intensity bone marrow on both T1-weighted and T2-weighted images. [ABSTRACT FROM AUTHOR] |
| Databáze: |
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