CTICasesMarfan Syndrome
Imaging findings
The radiograph demonstrates a long thorax and a thickened visceral pleura, suggestive of previous pneumothoraces or underlying pleural abnormalities. CT imaging reveals apical blebs and potential tracheal diverticula. Skeletal abnormalities, such as an unusual thoracic shape with anterior bowing, are also observable.
Key takeaways
Connective tissue abnormalities in Marfan syndrome increase the risk of forming apical blebs, which can lead to pneumothorax. Similar pulmonary manifestations can be seen in Ehlers-Danlos syndrome. Management for recurrent pneumothorax may include surgical intervention such as a blebectomy.
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