CTICasesNeurofibromatosis Type 1 (NF1) with Ribbon Ribs and Dural Ectasia
Imaging findings
Hand radiograph shows a soft tissue mass. Chest radiograph shows scoliosis and severe attenuation/thinnning of the upper ribs ("ribbon ribs") with tracheal deviation and a mediastinal mass. MRI demonstrates severe expansion of the spinal canal and neural foramina with fluid-intensity dural ectasia.
Key takeaways
Mesenchymal dysplasia in NF1 can manifest as characteristically thin, attenuated "ribbon ribs," scoliosis, and extensive dural ectasia mimicking a mediastinal or spinal mass. Alternatively, adjacent intercostal neurofibromas can remodel and narrow the ribs through pressure erosion.
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