Cerebral air embolism in vascular Ehlers-Danlos syndrome: a retrospective diagnosis

We describe a case of cerebral air embolism in a man with vascular Ehlers-Danlos syndrome. The key learning point is that cerebral air embolism can cause a florid encephalopathy and MR imaging appearance, yet the causative diagnosis may be made only by carefully scrutinising the CT scan, rather than the MRI. This patient was incorrectly diagnosed with posterior reversible encephalopathy syndrome before subsequent closer inspection of the CT scan at presentation disclosed the true diagnosis. Other diagnostic clues were the close temporal proximity to events that might introduce air into the systemic circulation (eg, thoracic procedures) or syndromes that predispose people to cerebral air embolism. The likely cause in this case was a transient bronchovenous fistula associated with vascular Ehlers-Danlos syndrome. Prompt identification of cerebral air embolism allows supportive measures to be started to prevent potentially fatal sequelae.