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Giant carotid pseudoaneurysm amenable to pipeline stenting in a patient with ehlers-danlos type IV
Abstract
Ehlers-Danlos type IV primarily affects collagen synthesis in the vasculature, increasing the risk of these patients to have dissection and pseudoaneurysm formation. Due to friable vessels, antiplatelet or anticoagulation has been the treatment of choice. However, newer intravascular surgical devices may be promising for future management. Case Description: A 24-year-old man with a history of Ehlers-Danlos type IV with multiple vascular and bleeding complications presented after recurrent, unprovoked presyncopal episodes. Patient was found to have dissection of bilateral internal carotid arteries (ICA) and right vertebral artery. Left ICA pseudoaneurysm was found in the proximal cervical segment. Patient was stabilized as an inpatient and discharged with outpatient follow-up with neurointerventional surgery. Follow-up imaging showed growth of the left ICA aneurysm. Patient elected to have pipeline stenting of the left ICA pseudoaneurysm. The procedure was performed without complication. Patient was discharged on dual antiplatelet therapy. At 7-month follow-up appointment, patient noted no neurological deficits. Follow-up digital subtraction angiogram at 7 months documented near-complete resolution of the pseudoaneurysm secondary to pipeline stenting. Conclusion: Pipeline stent implantation may be a viable corrective surgical option for patients with connective tissue disorders (specifically Ehlers-Danlos type IV) who present with pseudoaneurysm formation.
... Another endovascular option, stenting, is an adjunctive technique for vertebral artery PA repair [28, [40][41][42][43]. It is a technique in which a self-expanding mesh tube is placed within the vessel to open the stricture. ...
... Although they do have a high aneurysm occlusion rate, the process can take weeks, leaving the patient at an increased risk of rebleeding during this time [31, 46]. Senay et al. treated a patient presenting with a left internal carotid pseudoaneurysm with a pipeline embolization device [42]. The patient demonstrated near complete occlusion of the PA at 7-month follow up. ...
Background: Dissecting vertebral artery pseudoaneurysms represent a unique clinical challenge with careful appreciation for location of the posterior inferior cerebellar artery. Limited data is available in terms of outcomes regarding the various treatment modalities.
Methods: 11 patients with dissecting pseudoaneurysms were identified from 2013-2021. Pseudoaneurysm size and morphology, clinical presentation, and treatment approach was collected. Success of treatment was recorded based on post-operative imaging as well as documented overall patient outcomes. Three primary treatment modalities emerged: coil embolization, stent assisted coiling, and flow diversion.
Results: Of the 11 patients, 5 were female and 6 were male with an age from 36 to 69.7. 7 had ruptured pseudoaneurysms at time of treatment. Size of pseudoaneurysm ranged from 3 to 6 mm. 8 were on the right and 3 were on the left vertebral artery. 8 were proximal to PICA and 3 were distal. Co-dominance of vertebral filling was seen in 5 patients, 5 with dominance through right vertebral artery, and 1 with dominance through left vertebral artery. Variability existed in treatment approaches with 4 patients undergoing coil occlusion, 5 patients undergoing flow diversion stenting, and 2 patients undergoing flow diversion stenting with jailed coiling. 1 patient had enlargement of pseudoaneurysm while inpatient and required a second flow diversion device. 1 patient had two flow diversion devices placed initially at time of treatment due to morphology of PA. 6 patients had repeat angiograms between 6 to 9 months with complete occlusion. 3 had CTA or MRA with complete occlusion for those that had flow diversion, they were transitioned from aspirin and clopidogrel to aspirin monotherapy after first repeat angiogram. 6 patients required shunt placement for hydrocephalus. 1 patient died prior to discharge due to sepsis. 2 patients died post discharge: 1 with myocardial infarction and the 2nd due to urosepsis.
Dissecting vertebral pseudoaneurysm has high morbidity and mortality if rupture occurs. Location of PICA origin influences treatment approach. Patients with poor Hunt/Hess scores upon arrival had increased risk for systemic infection and mortality.
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