Abstract
Among Ehlers-Danlos syndromes, the vascular type is the most severe because of its vascular complications. Transcatheter embolization of medium-sized arteries has become the first-line therapy for life-threatening hemorrhage. Ongoing multiple lesions causing hemorrhagic or ischemic complications in the acute phase can challenge patient management. Multivessel endovascular treatment has never been reported. In this study, we report successful single-session treatment by coiling of a ruptured pseudoaneurysm of the hepatic artery with stenting of dissected right renal and iliac arteries in a 46-year-old female. Percutaneous transfemoral approach was gained and sealed with a plug-based closure device. Genetic disease was subsequently confirmed by molecular analysis.
Highlights
The Ehlers-Danlos syndromes (EDS) are genetically heterogeneous diseases involving the connective tissue, characterized by joint hypermobility, skin hyperextensibility, and tissue fragility
In the present case, following unilateral renal artery dissection, a known clinical onset of vEDS, a ruptured hepatic pseudoaneurysm was clearly depicted by MDCT.[13,14]
We reported successful transcatheter embolization with coiling of a ruptured hepatic artery pseudoaneurysm followed in the same session by stenting for dissection of the right renal and iliac arteries in a patient subsequently diagnosed as vEDS
Summary
The Ehlers-Danlos syndromes (EDS) are genetically heterogeneous diseases involving the connective tissue, characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. EDS (vEDS), previously known as Type IV, is the most feared and lethal form It is caused by mutations in the COL3A1 gene encoding in the pro-alpha 1 chain of Type III procollagen, widely present in vascular system.[1]. Dissections of the celiac trunk, proper hepatic, superior mesenteric, and common iliac arteries were present. As right renal stenting was deemed sufficient to preserve global kidney function, the dissection of the contralateral artery was left untreated because of the technical challenges and unfavorable risk/benefit ratio. CT scan, performed 5-months later for relapsing pneumothorax, conservatively managed, showed consistency of the hepatic coiling without signs of reperfusion, regular patency of the stented arteries, right kidney hypertrophy, and inferior cortical scars on the left kidney (Figure 2c and d). At 13 months follow-u p, the patient remained asymptomatic
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