Dynamic Digital Radiography in Ehlers-Danlos Syndrome: Visualizing Diaphragm Motility Impairment and Its Influence on...

A 40-year-old woman with a known diagnosis of Ehlers-Danlos syndrome (EDS) began experiencing progressive shortness of breath and reduced exercise tolerance following her second pregnancy. The patient underwent an unenhanced computed tomography (CT) scan that showed a marked elevation of the left diaphragm. Suspecting diaphragm dysfunction, the patient underwent Dynamic Digital Radiography (DDR) that confirmed a reduction in left diaphragm motility, indicative of impaired diaphragm function. Based on the DDR findings, which demonstrated reduced but preserved diaphragmatic motion without paradoxical movement or complete immobility, the thoracic surgeon decided that surgical intervention, such as plication, was not necessary. Instead, rehabilitation exercises, including breathing techniques and diaphragm strengthening, were recommended. EDS includes connective tissue disorders that vary in severity but are typically characterized by hypermobility of the joints, skin hyper-elasticity, and a predisposition to vascular fragility. One of the complications of EDS is weakened connective tissues, which can affect the diaphragm, impairing the contractility of the muscle and leading to impaired mobility and respiratory symptoms such as shortness of breath. Diaphragm dysfunction can manifest as reduced movement, potentially related to the underlying connective tissue weakness. This case highlights the clinical value of DDR as a non-invasive, low-dose, and dynamic imaging modality in the diagnosis of diaphragmatic dysfunction in EDS patients, enabling individualized treatment planning and potentially avoiding unnecessary surgical interventions.