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CASE REPORTS • J. Vasc. Bras. 15 (3) • Jul-Sep 2016 • https://doi.org/10.1590/1677-5449.001016 copy

Endovascular treatment of an aneurysm of the brachiocephalic trunk in a patient with Ehlers-Danlos syndrome type IV

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Ehlers-Danlos syndrome is a genetic disease that results in abnormalities of collagen synthesis, causing extremely fragile connective tissue. This fragility predisposes patients to a series of vascular disorders, such as dissections, aneurysms and pseudoaneurysms. The authors describe the case of a 19-year-old patient with an aneurysm of the brachiocephalic trunk who was treated endovascularly by placement of covered stents. The case progressed with complications at the puncture site, which was also treated on an emergency basis, using endovascular techniques with a covered stent.

Keywords:
aneurysm; brachiocephalic trunk; Ehlers-Danlos syndrome

Ehlers-Danlos syndrome type IV
Defect	Of type III collagen production
Major criteria	Thin and translucent skin^* * Criteria observed in the case reported here
	Arterial/intestinal/uterine fragility or rupture^* * Criteria observed in the case reported here Characteristic facial appearance^* * Criteria observed in the case reported here Frequent and extensive hematomas^* * Criteria observed in the case reported here
Minor criteria	Acrogeria
	Hypermobility of small joints^* * Criteria observed in the case reported here
	Rupture of muscles and/or tendons
	Twisted feet^* * Criteria observed in the case reported here
	Premature development of varicose veins
	Arteriovenous fistula (carotid cavernous)
	Pneumothorax^* * Criteria observed in the case reported here Positive family history^* * Criteria observed in the case reported here
	Sudden death

Vascular complications	Number of patients	Percentage of patients
Arterial aneurysm^* * Complications of the disease observed in the case reported here.	93	40
Arterial rupture (non-aneurysmal)	75	33
Carotid cavernous fistula	39	18
Major hematoma (retroperitoneal/intestinal)	33	15
Operative hemorrhage^* * Complications of the disease observed in the case reported here.	30	13
Arterial dissection	28	12
Pseudoaneurysm	21	9
Aneurysmal rupture	23	10
Arteriovenous fistula	5	2
Coronary aneurysm	2	1

	Open surgery	Endovascular surgery ^* * Surgical procedure performed in the case reported here.	Miscellaneous	Total
Treatment (n)	44	33	42	119
Mortality (n/%)	13/30	8/24	25/60	46/39

Sociedade Brasileira de Angiologia e de Cirurgia Vascular (SBACV) Rua Estela, 515, bloco E, conj. 21, Vila Mariana, CEP04011-002 - São Paulo, SP, Tel.: (11) 5084.3482 / 5084.2853 - Porto Alegre - RS - Brazil
E-mail: secretaria@sbacv.org.br

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