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Endovascular Trapping of a Large Cervical Carotid Pseudoaneurysm in Marfan Syndrome Presenting with Progressive Respiratory Distress.

World Neurosurgery 2018 December 25
BACKGROUND: Isolated cervical pseudoaneurysms of the internal carotid artery (ICA) in patients with Marfan syndrome are extremely rare.

CASE DESCRIPTION: A 73-year-old female with Marfan syndrome and history of multiple aortic aneurysms presented to an outside hospital with dysphagia, a mass in her neck, dizziness, nausea, and vomiting. A computed tomography angiogram (CTA) showed bilateral cervical ICA pseudoaneurysms, more significant on the right side. A laryngoscopy demonstrated edematous vocal cords and significant mass effect on the right side. She suffered from significant respiratory distress and aspiration, for which she had to be intubated and transferred to our institution for definitive care. The patient was taken for angiography for carotid artery test occlusion and endovascular trapping with coils and onyx embolization of the carotid pseudoaneurysm. The hospital course was complicated with femoral closure device failure, leading to groin hematoma, pneumonia and respiratory failure requiring tracheostomy and percutaneous endoscopic gastrostomy (PEG). The patient was discharged to rehabilitation 34 days after admission. Stable exclusion of the large right pseudoaneurysm was observed at 3-months follow up CTA, as well as significant improvement in last clinical follow up obtained 6 months after the procedure.

CONCLUSION: Pseudoaneurysms of the cervical ICA associated with Marfan syndrome are rare. Carotid test occlusion and endovascular permanent embolization are possible in a patient with life-threatening symptoms. Cautious use of vascular closure devices, especially collagen plug-based ones, are required in such patient, given their abnormal extracellular matrix structure.

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