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Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms.

ABSTRACT: Aneurysms-osteoarthritis syndrome (AOS), caused by SMAD3 mutations, is a recently described autosomal-dominant syndrome characterized by arterial aneurysms, tortuosity, and aortic dissections in combination with osteoarthritis. Our objective was to evaluate the AOS-related vascular consequences in the visceral and iliac arteries and raise awareness for this aggressive syndrome among vascular specialists.All AOS patients were monitored regularly according to our clinical AOS protocol. The study included those with one or more visceral aneurysms or tortuosity, or both. Clinical and surgical data were obtained from record abstraction.The study included 17 AOS patients (47% men) aged 47±13 years. A total of 73 aneurysms were encountered, of which 46 were located in the abdomen. The common iliac artery was most commonly affected (37%), followed by the superior mesenteric artery (15%), celiac trunk (11%), and splenic artery (9%). Rapid aneurysm growth?1 year was found in three arteries (gastric, hepatic, and vertebral artery). Furthermore, arterial tortuosity was noted in 94% of patients. Four patients underwent six elective (endo) vascular interventions for aneurysms in the iliac, hepatic, gastric, or splenic artery, without major perioperative or postoperative complications.AOS predisposes patients to widespread visceral and iliac artery aneurysms and extreme arterial tortuosity. Early elective aneurysm repair should be considered because the risk of aneurysm rupture is estimated to be very high and elective (endo) vascular interventions were not complicated by fragility of arterial tissue. Given the aggressive behavior of AOS, it is of utmost importance that vascular specialists are aware of this new syndrome.

SUBMITTER: van der Linde D

PROVIDER: S-EPMC4044827 | biostudies-literature | 2013 Jan

REPOSITORIES: biostudies-literature

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Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms.

van der Linde Denise D Verhagen Hence J M HJ Moelker Adriaan A van de Laar Ingrid M B H IM Van Herzeele Isabelle I De Backer Julie J Dietz Harry C HC Roos-Hesselink Jolien W JW

Journal of vascular surgery 20120911 1

<h4>Objective</h4>Aneurysms-osteoarthritis syndrome (AOS), caused by SMAD3 mutations, is a recently described autosomal-dominant syndrome characterized by arterial aneurysms, tortuosity, and aortic dissections in combination with osteoarthritis. Our objective was to evaluate the AOS-related vascular consequences in the visceral and iliac arteries and raise awareness for this aggressive syndrome among vascular specialists.<h4>Methods</h4>All AOS patients were monitored regularly according to our ...[more]

PMID: 22975338

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Project description:ObjectiveInternal iliac artery (IIA) preservation is associated with improved outcomes after both open and endovascular aortoiliac aneurysm repair. Total robotic laparoscopic repair of aortoiliac aneurysms has been reported in the past, but not in combination with sutureless anastomosis applied to the IIAs. The objective of this study was to demonstrate the feasibility of the total robotic laparoscopic technique including a method of deploying the Gore Hybrid Vascular Graft (GHVG; W. L. Gore & Associates, Flagstaff, Ariz) using robotic instruments.MethodsBetween June 2015 and December 2016, four patients underwent total robotic laparoscopic repair of isolated common iliac artery (CIA) aneurysms. Two patients had unilateral aneurysms and two had bilateral aneurysms. Unilateral CIA aneurysms were treated with a graft from the proximal CIA to the proximal external iliac artery, and bilateral CIA aneurysms were treated with a bifurcated graft between the distal aorta and both proximal external iliac arteries. The nitinol reinforced section of the GHVG was then inserted and deployed into the corresponding IIA, and the nonreinforced segment was sewn in an end-to-side fashion to the iliac graft.ResultsThe median age of patients was 55.5 years (range, 48-64 years); median body mass index was 24.9 kg/m2 (range, 23-26.4 kg/m2). All four cases were technically successful. Operative times were 325 and 332 minutes for unilateral cases and 491 and 385 minutes for bilateral cases. For the entire series, median proximal clamping time was 143 minutes (range, 110-163 minutes), and the median time to deploy the GHVG was 15 minutes (range, 8-27 minutes). The median estimated blood loss was 1800 mL (range, 800-2100 mL). Intraoperative cell salvage was used in all cases. No intraoperative or postoperative complications occurred. No patient required blood transfusion. All patients tolerated a regular diet on postoperative day 2 and were discharged on postoperative day 4. Patients returned to work and full physical activity within 6 weeks (range, 2-6 weeks). At 6-month follow-up, computed tomography angiography demonstrated 100% patency of iliac artery grafts as well as of the GHVGs.ConclusionsTotal robotic laparoscopic CIA aneurysm repair is feasible in both unilateral and bilateral cases in carefully selected patients. The GHVG can be successfully deployed using robotic technique for IIA preservation during total robotic CIA aneurysm repair.

Project description:PurposeTo determine outcomes of aneurysmal common iliac arteries (aCIA) used for landing zones (LZs) during endovascular aneurysm repair (EVAR).MethodsThis single-center study retrospectively compared 57 EVAR patients (mean age 72±8 years; 56 men) with 70 aCIAs (diameter ≥20 mm) to 25 control EVAR subjects (mean age 73±7 years; 20 men) with 50 normal (≤15-mm) CIA LZs treated consecutively during the same time interval. The CIA LZ measurements were analyzed using random effects linear mixed models to determine diameter change over time. Life tables were used to estimate freedom from endoleak, reintervention, and all-cause mortality.ResultsThe mean maximum preoperative CIA diameter in the aCIA LZ group was 24.8±4.5 mm (range 20.0-47.3, median 23.9) vs 13.6±1.5 mm (range 9.2-15.0, median 13.9; p<0.001) in the controls. Nineteen aCIA LZs were treated outside the instructions for use of the device. Median follow-up in the aCIAs LZ cohort was 39.2 months [interquartile range (IQR) 15, 61] vs 49.3 months (IQR 36, 61) in the controls (p=0.06). The rate of aCIA LZ change (0.09 mm/mo, 95% CI 0.07 to 0.1) was significantly greater than controls (0.03 mm/mo, 95% CI -0.009 to 0.07; p<0.0001). No type Ib endoleaks developed in either group; however, aCIA LZ patients had 6 (11%) iliac limb-related reinterventions. There were significantly more endograft-related reinterventions in the aCIA LZ patients (n=10, 14%) compared with controls (n=2, 4%; p=0.06). There was no difference in mortality or freedom from any post-hospital discharge endoleak.ConclusionAneurysmal CIA LZs used during EVAR experience greater dilatation compared with normal LZs, but no significant difference in outcome was noted in midterm follow-up. However, an increased incidence of graft limb complications or endograft-related reintervention may be encountered. Use of aCIA LZs appears to be safe; however, greater patient numbers and longer follow-up are needed to understand the clinical implications of morphologic changes in these vessels when used during EVAR.

Dataset Information

Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms.

Publications

Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms.

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