Spontaneous Ruptured Dissection of the Right Common Iliac Artery in a Patient with Classic Ehlers–Danlos Syndrome Phenotype

2015 ◽  
Vol 29 (3) ◽  
pp. 595.e11-595.e14 ◽  
Author(s):  
Rick Gaines ◽  
Brad T. Tinkle ◽  
Pegge M. Halandras ◽  
Omar Al-Nouri ◽  
Paul Crisostomo ◽  
...  
2019 ◽  
Vol 12 (11) ◽  
pp. e231537
Author(s):  
Matthew Lavoie ◽  
Jason Reese

We report a case of a 35-year-old woman found to have vascular Ehlers-Danlos syndrome (vEDS) after family history of sudden death due to aortic dissection in her otherwise healthy brother prompted further imaging workup and consideration of an underlying heritable genetic condition. CT angiogram of the aorta with intravenous contrast revealed an abdominal aortic artery dissection below the level of the renal arteries extending from the bifurcation into the left common iliac artery with an additional focal dissection of the right common iliac artery. To the author’s knowledge, this is the first report of asymptomatic bilateral common iliac artery dissections as a part of the initial presentation of a patient with underlying vEDS. Additionally, this case highlights the importance of familial diagnostic screening in inherited vasculopathies. Clinical history, genetic testing and management are discussed.


2020 ◽  
Vol 8 (C) ◽  
pp. 156-160
Author(s):  
Aleksandar Gjoreski ◽  
Ivona Jovanoska ◽  
Gjorgi Dungevski ◽  
Nikola Lazovski ◽  
Menka Lazareska

BACKGROUND: Ehlers-Danlos syndrome (EDS) type IV is a heritable disorder of connective tissue that is mainly associated with vascular maladies such as aneurysms, pseudoaneurysms, and dissections with or without spontaneous rupture. Historically, vascular complications in EDS IV have been treated conservatively whenever possible, due to the high morbidity and mortality after vascular interventions, whether open or endovascular. We present a case of a ruptured pseudoaneurysm of the right common iliac artery in a 18-year-old male, who was successfully treated by endovascular approach and later diagnosed with EDS type IV. CASE PRESENTATION: A 18-year-old male patient was admitted in ER with sharp pain in the right hypogastrium, hypotensive and with reduced blood parameters. Multiphasic modern computed tomography (MDCT) scan of abdomen and pelvis revealed massive ride sided pelvic and retroperitoneal hematoma. The presence of pseudoaneurysms on both common iliac arteries (CIA) was detected, with small ulcer on the right side and a focal dissection on the left side. An urgent endovascular repair of the ruptured pseudoaneurysm on the right CIA with covered stent was performed. Patient’s laboratory parameters and clinical status improved significantly within the next few days. CONCLUSIONS: Vascular repair in EDS-IV patients carries significant risk and should be indicated very carefully. Endovascular treatment for these patients is feasible and should be considered as an alternative to open surgery in some challenging cases as this one.


Hernia ◽  
2013 ◽  
Vol 18 (6) ◽  
pp. 907-909 ◽  
Author(s):  
M. Ardelt ◽  
Y. Dittmar ◽  
H. Scheuerlein ◽  
E. Bärthel ◽  
U. Settmacher

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Filipa Pereira ◽  
Teresa Cardoso ◽  
Paula Sá

Ehlers-Danlos syndrome(EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in theCOL3A1gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome.


1987 ◽  
Vol 2 (3) ◽  
pp. 173-179 ◽  
Author(s):  
Syde A. Taheri ◽  
Paul Nowakowski ◽  
David Pendergast ◽  
Julie Cullen ◽  
Steve Pisano ◽  
...  

The iliocaval compression syndrome is a disorder, frequently found in young women, in which extrinsic compression of the left iliocaval junction produces signs and symptoms of lower extremity venous insufficiency. The anatomic variant which gives rise to this syndrome consists of compression of the left common iliac vein by the overlying right common iliac artery, near its junction with the vena cava. Additional reduction of outflow results from intraluminal venous webs and tight adhesions between the iliac artery and vein. Pain, swelling, pigmentation, and venous claudication characterize this syndrome, which affects predominantly the left leg. The syndrome may progress to iliofemoral thrombosis, phlegmasia cerulea dolens, and venous gangrene. Longstanding iliocaval stenosis may produce valvular incompetence. Exercise plethysmography is a non-invasive test useful in screening patients for iliocaval compression. The definitive diagnosis is made by venography, both ascending and descending, to determine the degree of outflow stenosis. Iliocaval patch angioplasty with retrocaval positioning of the right iliac artery, decreases venous hypertension and leads to improvement in the clinical condition. To date, we have performed iliocaval angioplasty, with retrocaval repositioning of the right common iliac artery, on 18 patients. Of these, 83% have had good results as determined by hemodynamic and clinical assessment.


2020 ◽  
Vol 92 (2) ◽  
Author(s):  
Eugenio Di Grazia ◽  
Tiziana La Malfa ◽  
Gherardo Gasso

Ureter-arterial fistula (UAF) is an uncommon condition. The presentation is usually a life-threatening intermittent massive gross hematuria and the diagnosis is still a challenge for urologist. Idiopathic Retroperitoneal fibrosis (IRF) is a condition of unknown etiology characterized by a highly fibrotic retroperitoneal mass that frequently causes ureteral obstruction. To our knowledge we report the first case describing the UAF in a patient suffering from IRF. We hypothesize that inflammation and fibrosis resulted in fixation of the ureter to the adjacent artery causing a fistulous path. UAF was managed by deploying a 10 x 59 mm endo-graft at the intersection of common iliac artery bifurcation with the right ureter. Post treatment course was uneventful.


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