CTICasesSubarachnoid-Pleural Fistula in Marfan Syndrome
Imaging findings
Chest radiograph and CT show scoliosis, thoracic dural ectasia with lateral thoracic meningoceles in the posterior mediastinum, and a large recurrent right-sided pleural effusion. Thoracentesis confirmed the fluid was cerebrospinal fluid (CSF), communicating with the meningocele.
Key takeaways
Dural ectasia and lateral meningoceles are known dural anomalies in Marfan syndrome. A lateral meningocele can rarely rupture or fistulize into the pleural space, causing a subarachnoid-pleural fistula. This presents as a recurrent, non-inflammatory pleural effusion consisting of CSF, often accompanied by low-pressure headaches.
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