Direct puncture of the highest cervical segment of the internal carotid artery for treatment of an iatrogenic carotid cavernous fistula in a patient with Ehlers-Danlos syndrome: Figure 1

Khan, Aslam; Chaudhary, Neeraj; Pandey, Aditya S.; Gemmete, Joseph J.

doi:10.1136/neurintsurg-2011-010075

Cited by 11 publications

(13 citation statements)

References 10 publications

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“…Five (5.2%) patients [ 18 , 40 , 46 , 47 , 50 ] experienced a direct sCCF recurrence on the contralateral ICA 2.5 years, IQR [2.0–3.5] after the first fistula. Of 97 patients, 4 (4.1, 75.0% male) were diagnosed either with a direct CCF following minor trauma [ 7 , 11 , 14 ] or with an iatrogenic direct CCF after placement of a Fogarty balloon into the cavernous segment of the ICA [ 28 ].…”

Section: Resultsmentioning

confidence: 99%

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Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review

Adham

Trystram

Albuisson

et al. 2018

Orphanet J Rare Dis

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BackgroundVascular Ehlers-Danlos syndrome (vEDS) is a rare condition characterized by connective tissue fragility. Direct spontaneous carotid-cavernous fistula (sCCF) is reportedly pathognomonic of vEDS. We conducted this study to understand the possible mechanisms of occurrence of sCCF in this subset of patients.MethodsWe conducted a retrospective analysis of a monocentric vEDS cohort along with a literature review regarding sCCF in this condition.ResultsOf 133 patients regularly followed in our centre between 2000 and 2017, 13 (9.8%) had a diagnosis of direct sCCF (92.3% female, median age 33.0 years, interquartile range (IQR) [26.0–39.5]). There were 7 Glycine missense and 6 splice-site variants but no variant leading to haploinsufficiency. The literature search identified 97 vEDS patients with direct sCCF (79.4% female, 7.2% sex not reported, median age 31.0 years, IQR [24.0–39.0]). Increased carotid circumferential wall stress, higher carotid distensibility and lower carotid intima-media thickness could contribute to a higher risk for direct sCCF in vEDS. There is no predictive factor for the occurrence of sCCF apart from female sex in vEDS.ConclusionsIn vEDS, anatomical and pathophysiological features of the intra-cavernous internal carotid artery make it prone to shunting in the cavernous sinus, due either to a spontaneous rupture or to a spontaneous dissection with pseudoaneurysm formation. Direct sCCF in seemingly healthy young individuals should be highly suggestive of vEDS and prompt further investigation.

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Section: Resultsmentioning

confidence: 99%

“…Of 97 patients, 4 (4.1, 75.0% male) were diagnosed either with a direct CCF following minor trauma [ 7 , 11 , 14 ] or with an iatrogenic direct CCF after placement of a Fogarty balloon into the cavernous segment of the ICA [ 28 ].…”

Section: Resultsmentioning

confidence: 99%

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review

Adham

Trystram

Albuisson

et al. 2018

Orphanet J Rare Dis

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“…1 Recently, Khan et al reported successful embolization of a CCF in a patient with EDS IV by direct puncture of the highest cervical segment of the ICA after failure of a transvenous approach. 12 In addition, Hollands et al reported a case of initial surgical exposure of the ipsilateral carotid artery and internal jugular vein with placement of endovascular sheaths in lieu of a transfemoral access. 13 Our patient had a direct CCF which, in addition to the typical symptoms, was causing significant cerebral venous hypertension.…”

Section: Discussionmentioning

confidence: 99%

Endovascular repair of direct carotid–cavernous fistula in Ehlers–Danlos type IV

Linfante¹,

Lin²,

Knott

et al. 2014

BMJ Case Reports

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Ehlers-Danlos syndrome (EDS) type IV is a collagen vascular disease with an autosomal dominant inheritance caused by COL3A1 mutation. Patients with EDS type IV can present with organ rupture, spontaneous arterial dissections and ruptured aneurysms. Because of their propensity to form arterial dissections, aneurysms and rupture, they can develop carotid-cavernous fistula (CCF) after minor trauma or spontaneously. In EDS, it has been reported that even conventional catheter diagnostic angiography may result in large artery dissections and vessel rupture. In addition, the treatment of CCF in EDS type IV can result in up to 59% mortality after initial treatment, of which 23% is attributed to direct complications of treatment. We present the case of a patient with EDS type IV who previously had spontaneous dissection and multiple pseudoaneurysms of both the iliac and femoral arteries and the distal abdominal aorta. Several years later the patient developed a direct type A CCF which was successfully treated with endovascular embolization using a transvenous approach with detachable coils. The literature pertaining to CCF in EDS type IV and its treatment is reviewed.

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“…The complications are severe and include retroperitoneal hematoma, colonic rupture and iliac artery perforations 1. Recently, Khan et al reported successful embolization of a CCF in a patient with EDS IV by direct puncture of the highest cervical segment of the ICA after failure of a transvenous approach 12. In addition, Hollands et al reported a case of initial surgical exposure of the ipsilateral carotid artery and internal jugular vein with placement of endovascular sheaths in lieu of a transfemoral access 13…”

Section: Discussionmentioning

confidence: 99%

Endovascular repair of direct carotid–cavernous fistula in Ehlers–Danlos type IV

Linfante¹,

Lin²,

Knott

et al. 2014

J NeuroIntervent Surg

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Ehlers-Danlos syndrome (EDS) type IV is a collagen vascular disease with an autosomal dominant inheritance caused by COL3A1 mutation. Patients with EDS type IV can present with organ rupture, spontaneous arterial dissections and ruptured aneurysms. Because of their propensity to form arterial dissections, aneurysms and rupture, they can develop carotidcavernous fistula (CCF) after minor trauma or spontaneously. In EDS, it has been reported that even conventional catheter diagnostic angiography may result in large artery dissections and vessel rupture. In addition, the treatment of CCF in EDS type IV can result in up to 59% mortality after initial treatment, of which 23% is attributed to direct complications of treatment. We present the case of a patient with EDS type IV who previously had spontaneous dissection and multiple pseudoaneurysms of both the iliac and femoral arteries and the distal abdominal aorta. Several years later the patient developed a direct type A CCF which was successfully treated with endovascular embolization using a transvenous approach with detachable coils. The literature pertaining to CCF in EDS type IV and its treatment is reviewed. BACKGROUND

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Direct puncture of the highest cervical segment of the internal carotid artery for treatment of an iatrogenic carotid cavernous fistula in a patient with Ehlers-Danlos syndrome: Figure 1

Cited by 11 publications

References 10 publications

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review

Endovascular repair of direct carotid–cavernous fistula in Ehlers–Danlos type IV

Endovascular repair of direct carotid–cavernous fistula in Ehlers–Danlos type IV

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