Varying presentations in patients with symptomatic type IV vascular Ehlers-Danlos syndrome.

Ehlers-Danlos syndrome (EDS) represents a group of inheritable connective tissue disorders. Patients with type IV or vascular EDS, autosomal dominant pattern of inheritance, may present with aneurysm formation or arterial dissection. Due to vessel fragility, operative therapy for such disorders has been reserved for compelling indications in which benefit clearly warrants risk, yet assessment of risk is largely clinical with operative decisions guided by factors such as response to previous operations and age at onset of index vascular complications. We present 2 patients with differences in their clinical presentations and outcomes and review the literature with emphasis on operative decision making.

Endovascular versus open repair for descending thoracic aortic rupture: institutional experience and meta-analysis.

Rupture of thoracic aneurysm, acute type B dissection, blunt thoracic trauma, and penetrating aortic ulcer can present with a similar clinical profile of thoracic aortic rupture. We report a meta-analysis of comparative studies evaluating endoluminal graft versus open repair of these lesions as well as the early experience from our institution. We searched the following databases for reports of endovascular versus open repair of acute descending thoracic aortic rupture: Medline/PubMed, OVID, EMBASE, CINAHL, ClinicalTrials.gov, the Cochrane central register of controlled trials and the Cochrane database of systematic reviews. We used the random-effects model to calculate the odds ratio (OR) and 95% confidence intervals (CI) for mortality, paraplegia/paraparesis and stroke rates. Also, the medical records of the patients treated in our institution with this technique from 2000 to 2008 were reviewed. Demographics, comorbidities and operative procedure information were retrieved. Outcomes examined were mortality, paraplegia and stroke. Meta-analysis indicates that endoluminal graft repair is accompanied by lower procedure related mortality (OR 0.46, 95% CI 0.26-0.78, p=0.005) and paraplegia rates (OR 0.23, 95% CI 0.08-0.65, p=0.005), as compared to open repair. There was no difference in stroke rate between the two methods (OR 0.86, 95% CI 0.26-2.8, p=0.8). We have treated 13 patients with endoluminal stent-grafts. No conversion to open repair was necessary. Stroke rate was 15%, no patient died as a result of the stent-graft placement, one patient died as a result of massive head injury (overall 30-day mortality: 7.5%). There were no spinal cord ischemic complications. Our experience and meta-analysis indicate that thoracic endograft repair has low mortality and spinal cord complication rates for treatment of acute thoracic aortic rupture. If this method proves to be durable, it could replace open repair as the treatment of choice for these critically ill patients.

Meta-analysis of endovascular vs open repair for traumatic descending thoracic aortic rupture.

OBJECTIVES: Traumatic thoracic aortic injuries are associated with high mortality and morbidity. These patients often have multiple injuries, and delayed aortic repair is frequently used. Endoluminal grafts offer an alternative to open surgical repair. We performed a meta-analysis of comparative studies evaluating endovascular vs open repair of these injuries. METHODS: A systematic search of studies reporting treatment of traumatic aortic injury was performed using the following databases: Medline/PubMed, CINAHL, Proquest, Up to Date, Database of Abstracts of Reviews of Effects (DARE), ClinicalTrials.gov, the Cochrane Central Register of Controlled Trials and the Cochrane Database of Systematic Reviews. Search terms were thoracic aortic trauma, traumatic thoracic aortic injury, traumatic aortic rupture, stent graft repair, and endovascular repair. Outcomes analyzed were procedure-related mortality, overall 30-day mortality, and paraplegia/paraparesis rate using odds ratios (OR) and 95% confidence intervals (CI). Publication bias was investigated using funnel plots. Assessment of homogeneity was performed using the Q test; statistical heterogeneity was considered present at P < .05. Weighted averages of age, interval to repair, and injury severity score were compared with the Welch t test; P < .05 was considered statistically significant. RESULTS: Seventeen retrospective cohort studies from 2003 to 2007 were included. All were nonrandomized; no prospective randomized trials were found. These studies reported on 589 patients; 369 were treated with open repair, and 220 underwent thoracic stent graft placement. There was no significant difference in age (mean 38.8 years for both) or interval to repair (mean 1.5 days for endoluminal repair; 1 day for open repair). Injury severity score was higher for patients undergoing endoluminal repair (mean, 42.4 vs 37.4 for open repair, P < .001). Procedure-related mortality was significantly lower with endoluminal repair (OR, 0.31; 95% CI, 0.15-0.66; P = .002). Overall 30-day mortality was also lower after endoluminal repair (OR, 0.44; 95% CI, 0.25-0.78; P = .005). Sixteen studies reported data for postoperative paraplegia; 215 patients were treated with endograft placement and 333 with open repair. The risk of postoperative paraplegia was significantly less with endoluminal repair (OR, 0.32; 95% CI, 0.1-0.93; P = .037). The Q test did not indicate significant heterogeneity for the outcomes of interest; publication bias was limited. CONCLUSIONS: Meta-analysis of retrospective cohort studies indicates that endovascular treatment of descending thoracic aortic trauma is an alternative to open repair and is associated with lower postoperative mortality and ischemic spinal cord complication rates.