Long-term results after open repair of inflammatory infrarenal aortic aneurysms.

OBJECTIVE: The objective of this study was to investigate the long-term outcome after open repair of inflammatory infrarenal aortic aneurysms. METHODS: A total of 62 patients (mean age, 68.9 ± 8.8 years; 91.9% male) undergoing open surgery for inflammatory aortic aneurysm from 1995 until 2014 in a high-volume vascular center were retrospectively evaluated. The patients' demographics, preoperative and postoperative clinical characteristics, imaging measurements, and procedural data were collected. Study end points were preoperative and postoperative sac diameter, evolution of periaortic fibrosis and development of hydroureteronephrosis detected by computed tomography (CT) scan, and mortality and morbidity after 30 days and at the time of maximum follow-up. RESULTS: The mean abdominal aortic aneurysm diameter was 67.3 ± 16.7 mm. A total of 30 patients (48.4%) were asymptomatic, 27 patients (43.5%) were symptomatic, and 5 patients (8.1%) were treated for ruptured aneurysm. In 25 patients (40.3%), an aorta-aortic tube graft was implanted; in 37 patients (59.7%), an aortic bifurcation graft was used. Median operating time was 208 minutes (range, 83-519 minutes). Median aortic clamping time was 31 minutes (range, 14-90 minutes); in 25 patients (40.3%), suprarenal aortic cross-clamping was necessary. Hydroureteronephrosis was preoperatively diagnosed by CT scan in 16 patients (25.8%), with the need for a ureteral stent in 11 patients (17.7%). Aneurysm- and procedure-associated 30-day mortality was 11.3% (n = 7), with septic multiple organ failure in four patients and cardiac arrest in three patients. The overall perioperative complication rate was 33.9% (n = 21 patients). Median follow-up was 71.0 months (range, 0.2-231.6 months). At 1 year, 2 years, 4 years, and 6 years, overall survival was 83.4%, 79.6%, 79.6%, and 72.6%, respectively. Six patients (9.7%) required a reintervention during follow-up, predominantly aneurysm related and caused by aortoenteric fistula and graft infection (three of five patients). Median maximum thickness of preoperative perianeurysmal inflammation on CT was 10 mm (range, 2-22 mm), which decreased in 15 of 16 (94%) patients with available postoperative CT scans. Postoperative median thickness of perianeurysmal inflammation on CT was 6 mm (range, 0-13 mm). Hydroureteronephrosis persisted in two of nine (22.2%) patients at the end of follow-up. CONCLUSIONS: Surgery in patients with inflammatory abdominal aortic aneurysms is associated with a substantial amount of perioperative complications. After surgery, the perianeurysmal inflammation decreases in most patients on follow-up CT. However, because the inflammatory process does not totally resolve, patients require lifelong surveillance for hydroureteronephrosis and development of aortoenteric fistulas.

Retroperitoneal fibrosis: a clinical and outcome analysis of 58 cases and review of literature.

The purpose of the study was to investigate the clinical features and outcomes of retroperitoneal fibrosis (RPF). Fifty-eight RPF treatment cases in the First Affiliated Hospital of China Medical University were retrospectively analyzed, including clinical characteristics and laboratory data. RPF was found predominantly in elderly men with atypical clinical manifestations of back pain, abdominal pain, and lower limb edemas. In laboratory examinations, the acute-phase reactants such as erythrocyte sedimentation rate and C-reactive protein levels increased significantly. Renal function failure was frequently found in patients with urethral obstruction. All patients had retroperitoneal soft tissue shadows or urethral obstructions on computed tomography (CT) or magnetic resonance imaging (MRI), seven of which had histological diagnosis of idiopathic RPF. Forty-two patients received surgical interventions; 29 patients received medication treatment alone including corticosteroids, immunosuppressants, and tamoxifen; 17 patients received corticosteroids after surgical intervention. Surgery followed by medication was most effective for RPF. CT and MRI help to exclude secondary causes, but biopsy remains the gold standard for diagnosis. Long-term low-dose corticosteroids and immunosuppressants may prevent relapse of RPF.

Inflammatory aneurysms treated with EVAR.

Inflammatory abdominal aortic aneurysms (IAAA) are being treated more frequently by endovascular aneurysm repair (EVAR). Some authors caution against treating IAAA by EVAR because retroperitoneal inflammation may not subside post-operatively. A recent experience of 69 IAAA treated by open and endovascular methods is presented with results supporting the use of EVAR for IAAA. Several other studies evaluating EVAR in the treatment of IAAA are discussed.

Value of acute-phase reactants in monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis.

BACKGROUND: Prospective evaluation of the value of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels in monitoring disease activity and treatment response in patients with idiopathic retroperitoneal fibrosis (RPF). METHODS: This study included 57 patients with idiopathic RPF receiving tamoxifen monotherapy with at least 8 months follow-up. Clinical, laboratory and radiological investigation was performed at presentation and at repeated follow-up. Remission was defined as significant clinical improvement within 6 weeks of treatment together with stable or decreasing mass size on follow-up computed tomography (CT) scanning at 4 months and definitive decrease in mass size on follow-up CT scanning at 8 months. RESULTS: ESR and CRP levels at presentation and their respective decreases over time correlated strongly with each other (P<0.001). Baseline ESR and CRP levels correlated with visual analogue scale (VAS) score for pain (ESR, P<0.01; CRP, P<0.001); baseline ESR levels also correlated with VAS score for discomfort (P<0.001). Short-term decreases in ESR or CRP levels at 6 weeks follow-up did not correlate with subsequent mass regression but decrease in ESR at 4 months and decrease in CRP at 4 and 8 months follow-up correlated with mass regression. Kaplan-Meier analysis showed no difference in remission rate between patients with normal or elevated baseline ESR or CRP (log-rank P=0.22/P=0.88) or between patients with or without (near-)normalization of ESR or CRP in first 6 weeks of treatment (log-rank P=0.12/P=0.32). CONCLUSIONS: Patients with idiopathic RPF who have elevated acute-phase reactant levels are more symptomatic. Neither acute-phase reactant levels or their initial changes can be taken as a major predictor for treatment success.

Inflammatory abdominal aortic aneurysm endovascular repair into the long-term follow-up.

BACKGROUND: To investigate long-term outcomes for inflammatory abdominal aortic aneurysms (IAAA) after endovascular treatment (EVAR). METHODS: Between May 1997 and January 2009, 9 male patients (Mean 67 years, range 54-75 years) with IAAA were treated with EVAR using commercially available endografts. Results were assessed using contrast-enhanced computed tomography (CT) at 3 months, 12 months, and biannually thereafter. Echo-color duplex scanning was also recommended at 3, 6, and 12 months after discharge, and annually thereafter. Primary endpoints were aneurysm-related mortality, aneurysm sac evolution, perianeurysmal fibrosis (PAF) thickness, and hydronephrosis progression. RESULTS: No aneurysm-related deaths were observed during the long-term follow-up of eight patients (one patient death unknown). Maximum aneurysm sac diameter progressively reduced in eight patients (89%) and remained unchanged in one (11%). The absolute mean reduction of the aneurysm size was 26.2%. PAF regressed in two patients (22%), reduced in five (56%), and remained unchanged in two (22%). The absolute median reduction of the PAF thickness was 55.1%. No endoleak was observed during the follow-up period. Hydronephrosis persisted in all three patients who were preoperatively diagnosed with this pathology. Survival rates were 89%, 66.7%, and 66.7% at 12, 36, and 60 months, respectively. CONCLUSIONS: This series suggests that EVAR for IAAA into the long-term is associated with a positive trend for both PAF and aneurysm diameter reduction. EVAR does not seem to offer any benefits for hydronephrosis, but seems to effectively exclude the aneurysm sac in anatomically suitable patients.

A systematic review of open versus endovascular repair of inflammatory abdominal aortic aneurysms.

INTRODUCTION: Inflammatory abdominal aortic aneurysms (IAAAs) have traditionally been treated by open surgical repair (OSR). Over the last decade, endovascular aneurysm repair (EVAR) has been increasingly employed. The optimal treatment option for IAAA remains unclear. This article aims to evaluate and compare outcomes of OSR and EVAR in IAAA repair. METHODS: All publications in the English language relating to IAAA were sought electronically using OVID and MEDLINE (1972-2008). Studies identifying 30-day mortality were considered. Periaortic inflammation (PAI), hydronephrosis and 1-year mortality were obtained from studies with at least 1-year computed tomography (CT) follow-up. Outcomes of OSR and EVAR were compared and analysed for statistical significance using Fisher's exact test. RESULTS: The results were obtained from 35 studies comprising 999 patients and 21 studies with 121 patients who underwent OSR and EVAR, respectively. One-year CT follow-up was available for 124 and 52 patients from the two groups, respectively. Thirty-day mortality after OSR was 6% (95% confidence interval (CI); 6-13) and 2% (95% CI; 0-7) after EVAR (p=0.1). At 1 year, PAI regressed in 73% (95% CI; 64-80) in the OSR group compared to 65% (95% CI; 49-77) of the EVAR group (p=0.7). Conversely, inflammation progressed in 1% and 4%, respectively (p=0.1). Forty-five patients undergoing OSR and 29 EVAR were found to have preoperative hydronephrosis. This regressed postoperatively in 69% (95% CI; 53.3-81.8) and 38% (95% CI; 20.6-57.7), respectively (p=0.01). Hydronephrosis progressed in 9% of patients after OSR and in 21% after EVAR (p=0.1). New-onset hydronephrosis developed in 6% undergoing OSR compared to 2% with EVAR (p=0.2). One-year all-cause mortality after OSR was 14% (95% CI; 6-18) compared to 2% (95% CI; 0-13) after EVAR (p=0.02). CONCLUSION: Either OSR or EVAR may be considered based on patient suitability. EVAR is associated with lower 1-year mortality compared to OSR. However, OSR may be preferred in those patients who have hydronephrosis and are deemed low risk.

Inflammatory abdominal aortic aneurysms. A 20-year experience.

AIM: The aim of the study was to report a 20-year single Institution experience, with the early and late outcomes of surgical treatment of inflammatory abdominal aortic aneurysms. METHODS: In a 20-year period, 2 275 consecutive patients underwent elective surgical repair for non-rupture abdominal aortic aneurysm. Fifty-two patients (2.3%) were classified as inflammatory abdominal aortic aneurysms. Early and late outcomes were analyzed. RESULTS: One patient died in the perioperative period, giving a mortality rate of 1.92%. One patient died from a pseudoaneurysm rupture 7 months after operation. Three patients developed an aortic pseudoaneurysm in the follow-up period (mean 12.1 years, range 1-20 years) and underwent a redo operation. CONCLUSION: Overall surgical outcome of these patients, in terms of short-term and long-term is good. A high rate of pseudoaneurysm formation was observed.

Inflammatory aneurysm in the infrarenal portion of thoracoabdominal aneurysms: an uncommon variant.

INTRODUCTION: We present an unusual variant of type IV thoracoabdominal aneurysm with inflammatory changes in part of the aortic wall. MATERIAL AND METHODS: Between January 1990 and December 2000, 5 male patients (mean age, 68 years) with inflammatory aneurysms of 29 with type IV thoracoabdominal aneurysms (17.2%) underwent surgery. All five had arterial hypertension. The diagnosis of inflammatory aneurysm was made on the basis of clinical suspicion supported by characteristic features on CT scans. Endoaneurysmorraphy was performed in all cases; a tube graft was inserted in three cases, and bifurcation was performed in 2. The macroscopic appearance of periaortic fibrosis was detected at the infrarrenal portion of the aneurysm in the 5 patients, but no retroperitoneal fibrosis was found at the level of the distal thoracic aorta in any case. RESULTS: There was no operative mortality. Paraparesis was not detected in any patient. One patient had acute kidney failure, and another required an extended stay in the intensive care unit because of respiratory insufficiency. Pathologic examination confirmed the diagnosis of inflammatory aneurysm in all 5 patients, with characteristic adventitial thickening caused by lymphoplasmacytic infiltrate and fibrosis around the ganglionic and nerve structures. CONCLUSIONS: Type IV thoracoabdominal aneurysm with inflammatory changes can be treated at surgery with a retroperitoneal approach. Usually the fibrotic response is confined to the infrarenal portion of the aneurysm.

Asbestos exposure: a potential cause of retroperitoneal fibrosis.

The etiology of retroperitoneal fibrosis is unknown in 70% of the cases. The aim of our study was to examine the possible association between occupational asbestos exposure and retroperitoneal fibrosis; only two cases have been reported in the literature. We gathered all the cases of retroperitoneal fibrosis diagnosed in the Tampere University Hospital between 1987 and 1995. We examined their hospital records to evaluate the possible etiology of the disease. We also sent a structured questionnaire to all living patients (10/13) to obtain information on their asbestos exposure. The chest radiographs of the patients were re-read to evaluate possible changes resulting from asbestos exposure. We found 13 cases of idiopathic retroperitoneal fibrosis. Seven patients (all male) had been exposed to asbestos in the past. The chest radiographs of the four most-exposed patients showed characteristic asbestos-related abnormalities, including bilateral pleural plaques, round atelectasis and small irregular lung opacities. In our study, we found that asbestos exposure and asbestos-induced changes in the lung and pleura were common among male retroperitoneal fibrosis patients. We suggest that occupational exposure to asbestos may be an important etiological factor for retroperitoneal fibrosis.

Diagnosis and management of 17 consecutive patients with inflammatory abdominal aortic aneurysm.

This report reviews the authors' experience in diagnosing and managing 17 consecutive patients with inflammatory abdominal aortic aneurysm (AAA). Among 491 patients undergoing repair for AAA during a 10-year period, 17 (3%) had evidence of associated periaortic fibrosis, which was confirmed histologically. No patient had acute rupture, and two patients (12%) had chronic contained rupture. Ureteral obstruction was evident in seven patients. In 41% of the patients, available surgical correlation demonstrated that computed tomographic (CT) scan accurately delineated the extent of the disease. Sixteen patients underwent aneurysm resection. Ureteral obstruction was relieved by ureterolysis in three patients treated early in this series. In the last period of the study, well-documented hydronephrosis spontaneously subsided in two patients without special treatment. Of these 17 patients, 15 (88%) were early (30-day) survivors. There were two late deaths at 2 months and 5 years; 12 (71%) patients are still alive and free of symptoms up to 10 years after operation. On the basis of our study, we conclude the following: (1) precise preoperative diagnosis and detailed anatomic information are widely available with CT; (2) aneurysm resection is the treatment of choice because the risk of rupture still exists, and this procedure seems to reverse the inflammatory process; (3) good early and late results can be expected with proper surgical technique; and (4) routine follow-up with CT is recommended to document resolution or progression of the fibrotic process after aneurysm resection.

Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases.

Sixty patients with idiopathic retroperitoneal fibrosis presenting between 1965 and 1984 are reviewed. Their mean age at presentation was 56 years and the male:female ratio was 3:1. The commonest presenting symptoms were flank and abdominal pain, weight loss, nausea and polyuria. Physical examination was usually normal, expect for the presence of hypertension. Anaemia and elevation of erythrocyte sedimentation rate were usually present. Proteinuria was found in less than a third of patients at presentation and significant bacteriuria was uncommon. The correct diagnosis was made or suspected in very few patients before referral. The cumulative actuarial survival rate was 86% at 1 year and 78% at 2 years. Seventeen patients died; they were significantly older and more uraemic at the time of referral than those who survived. A few patients did well with either corticosteroid therapy or ureterolysis alone. In the majority, both operation and steroid treatment were necessary. In bilateral obstruction with residual function in both kidneys, bilateral ureterolysis proved superior to unilateral operation (each followed by steroid therapy) in conserving renal function. Operation alone or steroid therapy alone should be considered in cases where steroids or surgery respectively present particular hazards. The less traumatic unilateral operation should be considered in poor risk patients and in those whose renal function is absent on one side. In many survivors, disease activity has persisted for many years. Life-long follow-up is recommended.

[Xanthofibrogranulomatosis with meningocortical involvement]. / Xanthofibrogranulomatose mit meningealer Beteiligung.

The history of a 52-year old female with xanthofibrogranulomatosis (X.) is presented. Clinical and pathologic-anatomical findings of this rare disease are described. We report the case, because of the unusual intracranial spread of the X.