A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta-a clinical and diagnostic challenge.

Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycophenolate mofetil. The prevalence of the disease remains unknown because it is often misdiagnosed and can mimic many malignant, infectious, and inflammatory conditions. Autoimmune pancreatitis is a common presenting condition of IgG4-RD. Because As IgG4-RD is responsive to steroids, diagnosing IgG4-related RPF early can prevent the exposure of patients with RPF to unnecessary diagnostic and therapeutic interventions.

Midterm outcomes of thoracic aortic stent-grafts: complications and imaging techniques.

PURPOSE: To evaluate the midterm outcomes of thoracic aortic stent-grafting and the performance of computed tomographic angiography (CTA), radiography, and magnetic resonance angiography (MRA) in endograft surveillance. METHODS: Forty-seven patients with traumatic thoracic aortic ruptures (n=16), aneurysms (n=14), false aneurysms (n=3), penetrating ulcers (n=3), and dissections (n=11) treated with stent-grafts were monitored in follow-up using chest radiography and CTA in all patients and MRA in 23 patients. Two perpendicular maximal aortic diameters, the sum of these diameters, and the elliptical cross-sectional area were determined and compared to baseline for the entire group and in subgroup analyses according to lesion type. CTA, MRA, and radiography were compared for their ability to detect endoleak, monitor stent-graft configuration, and measure aortic diameters. RESULTS: The mortality rate was 8.5%. Severe complications were observed in 14.8% (6% neurological complications); 12 (25.5%) patients had primary endoleaks. Over a mean 11-month follow-up (range 0.25-46 months), the aortic diameters decreased for all patients without endoleak (p<0.001). In the diameter/area subgroup analyses, only the traumatic rupture cohort demonstrated significant decreases in all 4 measurements. CTA and MRA measurements correlated well, but chest radiography was superior to both for visualizing stent-graft shape. In terms of endoleak detection, MRA missed only 1 (12.5%) endoleak (type II) seen on CTA; there were no false positive results with MRA. CONCLUSIONS: Morbidity and mortality observed after thoracic stent-grafting are acceptable. Radiography is better for monitoring stent-graft conformation, while CTA provides the best overall morphological information. The performance of MRA in endoleak detection is encouraging.

Stent-graft treatment of penetrating thoracic aortic ulcers.

PURPOSE: To evaluate the efficacy of stent-graft placement for the treatment of penetrating thoracic aortic ulcers. METHODS: Ten patients (7 men; mean age 73.8 years, range 69-79) were treated for penetrating thoracic aortic ulcers using Talent or Excluder stent-grafts. Preoperative examinations included computed tomographic angiography (CTA), transesophageal echography, and digital subtraction angiography (DSA). Follow-up included predischarge multimodal imaging and periodic CTA scans after discharge. Endoleaks, aortic diameter changes, and clinical complications were tracked. RESULTS: Technical success was achieved in 100%, but 1 major neurological complication led to death 3 months after the procedure. Radiological follow-up detected 4 early, endoleaks (3 type I and a type II), all of which spontaneously regressed, and 1 secondary type II endoleak. The mean aortic diameter decreased by 22% over a mean 9-month follow-up. CONCLUSIONS: Aortic ulcers are potentially lethal lesions. Considering its low morbidity and mortality, endovascular repair could widen the treatment options for these lesions.