Risks and benefits of renal artery stenting in fibromuscular dysplasia: Lessons from the ARCADIA-POL study.

INTRODUCTION: Although renal stenting is the standard revascularization method for atherosclerotic renal artery stenosis (RAS) (FMD-RAS), stenting in fibromuscular dysplasia (FMD) RAS is usually limited to periprocedural complications of angioplasty and primary arterial dissection. The main aim of the study was to retrospectively analyze the immediate and long-term results of renal stenting versus angioplasty in patients with FMD. METHODS: Of 343 patients in the ARCADIA-POL registry, 58 patients underwent percutaneous treatment due to FMD-RAS (in 70 arteries). Percutaneous transluminal renal angioplasty (PTRA) was performed as an initial treatment in 61 arteries (PTRA-group), whereas primary stenting was undertaken in nine arteries (stent-group). Stent-related complications were defined as: in-stent restenosis > 50% (ISR); stent fracture; under-expansion; or migration. RESULTS: In the PTRA-group, the initial restenosis rate was 50.8%. A second procedure was then performed in 22 arteries: re-PTRA (12 arteries) or stenting (10 arteries). The incidence of recurrent restenosis after re-PTRA was 41.7%. Complications occurred in seven of 10 (70%) arteries secondarily treated by stenting: two with under-expansion and five with ISR. In the stent-group, stent under-expansion occurred in one case (11.1%) and ISR in three of nine stents (33.3%). In combined analysis of stented arteries, either primarily or secondarily, stent-related complications occurred in 11/19 stenting procedures (57.9%): three due to under-expansion and eight due to ISRs. Finally, despite several revascularization attempts, four of 19 (21%) stented arteries were totally occluded and one was significantly stenosed at follow-up imaging. CONCLUSION: Our study indicates that renal stenting in FMD-RAS may carry a high risk of late complications, including stent occlusion. Further observational data from large-scale registries are required.

Systematic and Multidisciplinary Evaluation of Fibromuscular Dysplasia Patients Reveals High Prevalence of Previously Undetected Fibromuscular Dysplasia Lesions and Affects Clinical Decisions: The ARCADIA-POL Study.

Fibromuscular dysplasia (FMD), regarded as a generalized vascular disease, may affect all vascular beds and may result in arterial stenosis, occlusion, aneurysm, or dissection. It has been proposed to systematically evaluate all vascular beds in patients with FMD, regardless of initial FMD involvement. However, the impact of this approach on clinical decisions and on management is unknown. Within the prospective ARCADIA-POL study (Assessment of Renal and Cervical Artery Dysplasia-Poland), we evaluated 232 patients with FMD lesions confirmed in at least one vascular bed, out of 343 patients included in the registry. All patients underwent a detailed clinical evaluation including computed tomography angiography of intracranial and cervical arteries, as well as computed tomography angiography of the abdominal aorta, its branches, and upper and lower extremity arteries. In the study group, FMD lesions were most frequently found in renal arteries (87.5%). FMD was also found in cerebrovascular (24.6%), mesenteric (13.8%), and upper (3.0%) and lower extremity (9.9 %) arteries. Newly diagnosed FMD lesions were found in 34.1% of the patients, and previously undetected vascular complications were found in 25% of the patients. Among all FMD patients included in the study, one out of every 4 evaluated patients qualified for interventional treatment due to newly diagnosed FMD lesions or vascular complications. The ARCADIA-POL study shows for the first time that the systematic and multidisciplinary evaluation of patients with FMD based on a whole-body computed tomography angiography scan has an impact on their clinical management. This proved the necessity of the systematic evaluation of all vascular beds in patients with FMD, regardless of initial FMD involvement.

Dissecting visceral fibromuscular dysplasia reveals a new vascular phenotype of the disease: a report from the ARCADIA-POL study.

OBJECTIVE: Visceral artery fibromuscular dysplasia (VA FMD) manifestations range from asymptomatic to life-threatening. The aim of the study is to evaluate the prevalence and clinical characteristics of VA FMD. METHODS: A total of 232 FMD patients enrolled into ongoing ARCADIA-POL study were included in this analysis. All patients underwent detailed clinical evaluation including ambulatory blood pressure monitoring, biobanking, duplex Doppler of carotid and abdominal arteries and whole body angio-computed tomography. Three control groups (patients with renal FMD without visceral involvement, healthy normotensive patients and resistant hypertensive patients) matched for age and sex were included. RESULTS: VA FMD was present in 32 patients (13.8%). Among these patients (women: 84.4%), FMD lesions were more frequent in celiac trunk (83.1%), 62.5% of patients showed at least one visceral aneurysm, and five patients presented with severe complications related to VA FMD. No demographic differences were found between patients with VA FMD and individuals from the three control groups, with the exception of lower weight (P < 0.001) and BMI (P < 0.001) in VA FMD patients. Patients with FMD (with or without visceral artery involvement) showed significantly smaller visceral arterial diameters compared with controls without FMD. CONCLUSION: Patients with FMD showed smaller visceral arterial diameters when compared with patients without FMD. This may reflect a new phenotype of FMD, as a generalized arteriopathy, what needs further investigation. Lower BMI in patients with VA FMD might be explained by chronic mesenteric ischemia resulting from FMD lesions. FMD visceral involvement and visceral arterial aneurysms in patients with renal FMD are far to be rare. This strengthens the need for a systematic evaluation of all vascular beds, including visceral arteries, regardless of initial FMD involvement.

Association of intrarenal blood flow with renal function and target organ damage in hypertensive patients with fibromuscular dysplasia: the ARCADIA-POL study.

INTRODUCTION Data on the assessment of intrarenal blood flow parameters in patients with renal fibromuscular dysplasia (FMD) are scarce. OBJECTIVES The aim of the study was to evaluate intrarenal blood flow parameters in patients with FMD and significant or nonsignificant renal artery stenosis (RAS). PATIENTS AND METHODS We evaluated intrarenal blood flow parameters by Doppler ultrasonography in 153 patients with renal FMD enrolled in the ARCADIA­POL study: 32 and 121 patients with and without significant RAS, respectively, compared with 60 matched patients with essential hypertension and 60 healthy controls. RESULTS Patients with FMD and significant RAS had a lower renal resistive index (RRI) compared with patients with FMD without significant RAS, patients with essential hypertension, and normotensive controls (mean [SD], 0.51 [0.08] vs 0.60 [0.07], 0.62 [0.06], and 0.61 [0.06], respectively; P <0.001). In patients with nonsignificant RAS, RRI correlated significantly with carotid intima-media thickness, 24­hour diastolic blood pressure, 24­hour pulse pressure, left ventricular diastolic function, known duration time of hypertension, and age. In patients with significant RAS, there was a significant correlation between RRI and known duration time of hypertension, left ventricular diastolic function, and age. In a separate, "per­kidney" analysis, renal arteries with FMD and significant RAS were characterized by lower RRI values, higher maximal blood flow velocity, higher renal aortic ratio, and longer acceleration time compared with renal arteries with FMD and nonsignificant RAS as well as renal arteries without FMD. CONCLUSIONS In contrast to atherosclerotic RAS, intrarenal blood flow in patients with FMD and RAS is preserved, confirming that renal vasculature is relatively intact in these patients.

High incidence and clinical characteristics of fibromuscular dysplasia in patients with spontaneous cervical artery dissection: The ARCADIA-POL study.

The association between fibromuscular dysplasia (FMD) and spontaneous cervical artery dissection (SCeAD) has been recognized, but the available evidence on this relationship is scant. Therefore, the main goal of our study was to systematically evaluate FMD frequency, clinical characteristics and vascular bed involvement in patients with SCeAD. Among 230 patients referred to the ARCADIA-POL study, 43 patients (mean age 44.1 ± 8.9 years; 15 men and 28 women) with SCeAD were referred. Also, 135 patients with FMD were compared to patients with and without SCeAD. Patients underwent: ambulatory blood pressure measurements, biochemical evaluation, echocardiographic examination, and whole body computed tomographic angiography. FMD changes were found in 39.5% of patients with SCeAD. There were no differences in clinical characteristics between patients with SCeAD and FMD and those without FMD, except for a tendency towards a higher female ratio in SCeAD patients with FMD. There were no differences in other parameters describing target organ and SCeAD characteristics. Patients with SCeAD and FMD compared to those without SCeAD were characterized by a lower frequency of hypertension and a higher frequency of hyperlipidemia and history of contraceptive hormone use. Our study indicates a high incidence (39.5%) of FMD in subjects with SCeAD. Since there are no distinctive discriminating factors between patients with SCeAD and FMD and those without FMD, FMD should be suspected in all patients with SCeAD.

Prevalence of smoking and clinical characteristics in fibromuscular dysplasia. The ARCADIA-POL study.

PURPOSE: Smoking was identified as a potential factor contributing to fibromuscular dysplasia (FMD). To evaluate the prevalence of smoking and clinical characteristics in FMD subjects. MATERIAL AND METHODS: We analysed 190 patients with confirmed FMD in at least one vascular bed. The rate of smokers in FMD patients was compared to that in two control groups selected from a nationwide survey. RESULTS: The rate of smokers in FMD patients was 42.6%. There were no differences in frequency of smokers between FMD patients and: a group of 994 matched control subjects from general population and a group of matched hypertensive subjects. There were no differences in the characteristics of FMD (including rates of multisite FMD and significant renal artery stenosis) and its complications (including rates of dissections and aneurysms) between smokers and non-smokers. Smokers as compared with non-smokers were characterized by higher left ventricle mass index. CONCLUSIONS: There is no difference in the rate of smokers between FMD patients and subjects from the general population. Moreover, we did not find any association between smoking and clinical characteristics of FMD patients nor its extent and vascular complications. Our results do not support the hypothesis that smoking is involved in the pathophysiology of FMD.

Embolization of iatrogenic renal arteriovenous fistula - a case report.

Renal artery pseudoaneurysms and arteriovenous fistulae most often occur as an iatrogenic complication. The article discusses a case of a patient diagnosed with an arteriovenous fistula and a pseudoaneurysm. A 64-year-old woman was admitted to the hospital due to nonspecific pain in the lumbar region. Imaging showed a typical picture of clear cell renal carcinoma. The patient was qualified for surgical treatment. After tumor resection, the patient developed microhematuria. Arteriovenous fistula and renal pseudoaneurysm were diagnosed using Doppler and computed tomography scans. The patient was qualified for arteriography with simultaneous embolization of the lesion. A follow-up evaluation confirmed the exclusion of aneurysm and fistula. Treatment outcomes were monitored using Doppler ultrasound. Doppler ultrasonography is the first method of choice in detecting and monitoring renal artery irregularities. Safety, non-invasiveness and easy access to this tool make it play a key role in the diagnosis of renal artery fistulas and pseudoaneurysms.

Echocardiographic assessment of left ventricular morphology and function in patients with fibromuscular dysplasia: the ARCADIA-POL study.

OBJECTIVE: To provide a comprehensive assessment of left ventricle (LV) structure, and function and to detect alterations in cardiac properties in relationship to presence, subtypes and extent of fibromuscular dysplasia (FMD). METHODS: We studied 144 patients with FMD. The control group consisted of 50 matched individuals. Office and ambulatory blood pressure levels were evaluated. Echocardiography was employed to assess: left ventricular mass index (LVMI), systolic function including speckle tracking echocardiography and diastolic function assessed by mitral flow and tissue Doppler imaging. RESULTS: There were no differences in LV morphology and function between patients with FMD and the control group. Among 128 patients with renal FMD, there were no differences in LVMI and LV systolic function between patients with unifocal and multifocal FMD. The patients with multifocal FMD were characterized by lower early diastolic velocity (e') as compared with unifocal FMD and control groups. However, in a multivariate regression model, e' was not independently correlated with FMD. There were no associations between echocardiographic indexes and vascular involvement of FMD. Also, there were no differences in LV morphology and function in patients with significant renal artery stenosis (RAS) compared with patients with history of significant RAS and patients with nonsignificant RAS. CONCLUSION: Our study in contrast to those with atherosclerotic RAS, did not show differences in LV morphology and function between FMD patients and matched controls. Although FMD can result in hypertension and serious vascular complications, there is no proof that it can alter LV regardless of FMD type and its extent.

Endovascular Embolization of Renal Cell Carcinoma in a Patient with Solitary Kidney.

BACKGROUND: Kidney tumors account for about 3% of tumors in adults. The primary therapy of renal cancer is the surgical removal. Traditionally, and also modern procedures are performed to remove the kidneys, especially when the tumor involves the entire kidney. In the cases of unresectable tumors embolization is used as a palliative procedure. CASE REPORT: The aim of this study is to present the case of endovascular treatment of renal cell carcinoma in patient with solitary kidney. 77-years old patient had an ultrasound examination because of the pain in left lumbar region. MRI confirmed the presence of tumor size 29×45 mm in the left kidney. The right kidney had been removed eight years earlier because of clear cell carcinoma. Histopathological diagnosis was renal clear cell carinoma. The patient did not consent to surgical treatment. Tumor embolization was proceeded as a minimally invasive procedure. Pathological tumor vessels were closed using particles filling the entire vascular tumor. Next, the blood vessels supplying the tumor were closed using a mixture of lipiodolu and glubranu. Control angiographiy of the left renal artery confirmed the effective closure of all vascular pathology. In a recent ultrasound examination which was done 15 months after surgery no evidence of vascular pathology was found. CONCLUSIONS: Embolization of kidney cancer in particular cases may be an alternative way of treatment and give a good result in the form of stopping the growth of the tumor with simultaneous retaining the remaining parenchyma and renal function.

Diagnosis and management of multiple paragangliomas of the head and neck.

Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients with multiple paragangliomas of the head and neck. A retrospective review of the records of 84 patients with head and neck PGs, diagnosed and treated in our institution was performed for the years 1983-2013 to identify patients with multiple tumors. Fourteen (16.6 %) patients developed multiple PGs, synchronous or metachronous, within 4-21 years of follow-up. Clinical data of these patients were reviewed to evaluate the diagnosis, location, stage and management strategy. There was a total number of 37 tumors in 14 patients. There were 20/37 (54.0 %) carotid PGs, 9/37 (24.3 %) jugular PGs and 8/37 (21.7 %) vagal PGs. Carotid PGs were observed in 12/14 (86 %) patients and in 8/14 (57 %) cases bilateral tumors occurred. Vagal PGs developed in 7/14 (50 %) patients and bilateral tumors were found in 1/14 (7 %) case. Jugular PGs occurred in 9/14 (64 %) patients. There were 30 synchronous tumors and seven metachronous PGs diagnosed 2-18 years after removal of the first tumor. Single metachronous mediastinal PG occurred. All patients had at least one tumor removed, with histopathological confirmation of the diagnosis. One patient had positive history of familial PGs. Carotid PGs are most common multiple paragangliomas. Radiological survey of the head and neck is required to detect multicentric tumors. Metachronous mediastinal and abdominal tumors may occur. Regular, prolonged follow-up is essential to identify metachronous PGs and possible postoperative gradual ICA occlusion.

Endovascular transcatheter embolization of recurrent postsurgical varicocele: anatomic reasons for surgical failure.

BACKGROUND: Formation or pre-existence of collateral gonadal veins in varicocele patients has been reported as the main cause of surgical treatment failure. PURPOSE: To describe venographic findings in patients with postsurgical recurrent varicoceles and to assess the efficacy of the following minimally invasive endovascular treatment. MATERIAL AND METHODS: Thirty-three men with failed surgical treatment of left-sided varicocele were examined between 2006 and 2013, using retrograde venography to assess the anatomy of varicocele draining veins before the attempted transcatheter embolization. Anatomic variants of gonadal veins were categorized according to the classification modified for the purpose of the present study. 3% polidocanol was used as an embolic agent together with pushable fibered coils. RESULTS: In 31 (93%) out of 33 patients venography demonstrated incompetence of the gonadal vein or veins draining varicoceles after failed surgical treatment. The most frequent venographic finding was gonadal vein duplication - 66% of cases (39% in its mid-portion). Technical success of embolization was achieved in all 31 patients. No major complications were observed. CONCLUSION: Retrograde varicocele embolization may be superior to surgery because of its ability to detect gonadal vein variants. In our study group, transcatheter embolization with 3% polidocanol and fibered coils allowed successful, minimally invasive treatment of postsurgical varicoceles.

Two types of lateral extension in juvenile nasopharyngeal angiofibroma: diagnostic and therapeutic management.

Juvenile nasopharyngeal angiofibroma is a benign, locally aggressive nasopharyngeal tumor. Apart from anterior lateral extension to the pterygopalatine fossa, it may spread laterally posterior to the pterygoid process, showing posterior lateral growth pattern, which is less common and more difficult to identify during surgery. We analyzed the routes of lateral spread, modalities useful in its diagnosis, the incidence of lateral extension and its influence on outcomes of surgical treatment. The records of 37 patients with laterally extending JNA treated at our institution between 1987 and 2011 were retrospectively evaluated. Computed tomography was performed in all patients and magnetic resonance imaging in 17 (46 %) patients. CT and MRI were evaluated to determine routes and extension of JNA lateral spread. Anterior lateral extension to the pterygopalatine fossa occurred in 36 (97 %) patients and further to the infratemporal fossa in 20 (54 %) patients. In 16 (43 %) cases posterior lateral spread was observed: posterior to the pterygoid process and/or between its plates. The recurrence rate was 29.7 % (11/37). The majority of residual lesions was located behind the pterygoid process (7/11). Recurrent disease occurred in 3/21 patients with anterior lateral extension, in 7/15 patients with both types of lateral extensions and in 1 patient with posterior lateral extension. JNA posterior lateral extension may spread behind the pterygoid process or between its plates. The recurrence rate in patients with anterior and/or posterior lateral extension is significantly higher than in patients with anterior lateral extension only. Both CT and MRI allow identification of the anterior and posterior lateral extensions.

Saccular aneurysm of superior vena cava treated with percutaneous, transcatheter thrombin injection.

We report the case of successful endovascular treatment of large saccular aneurysm of SVC in a patient with vascular malformation of right hand and chest. Considering the high risk of surgery, the patient was referred for percutaneous intervention. Venography showed communication between the aneurysm and SVC, just below brachiocephalic confluence. That is why the decision of balloon-protected transcatheter thrombin injection was made. Selective catheter was placed in the aneurysm and balloon occlusion catheter in SVC. Both catheters were withdrawn right after thrombin injection. During follow-up, aneurysm slightly enlarged in early observation and after a year shrinkage was observed.

Invasive growth patterns of juvenile nasopharyngeal angiofibroma: radiological imaging and clinical implications.

Juvenile nasopharyngeal angiofibroma is a benign lesion with locally aggressive nature. Knowledge of its typical growth patterns is crucial for precise preoperative staging and adequate preoperative patient counseling. This pictorial essay focuses on characteristic radiological features and paths of invasive growth of this rare tumor. Also, the impact of accurate preoperative evaluation of tumor extensions on surgical planning and results of treatment are discussed.

Onyx(®) in endovascular treatment of cerebral arteriovenous malformations - a review.

Arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. In most cases, the disorder may be asymptomatic. The objective of endovascular AVM treatment is set individually for each case upon consultations with a neurosurgeon and a neurologist. The endpoint of the treatment should consist in prevention of AVM bleeding in a management procedure characterized by a significantly lower risk of complications as compared to the natural history of AVM. Endovascular interventions within AVM may include curative exclusion of AVM from circulation, embolization adjuvant to resection or radiation therapy, targeted closure of a previously identified bleeding site as well as palliative embolization. Onyx was first described in the 1990s. It is a non-adhesive and radiolucent compound. Onyx-based closure of the lumen of the targeted vessel is obtained by means of precipitation. The process is enhanced peripherally to the main flux of the injected mixture. This facilitates angiographic monitoring of embolization at any stage. The degree of lumen closure is associated with the location of the vessel. Supratentorial and cortical locations are most advantageous. Dense and plexiform structure of AVM nidus as well as a low number of supplying vessels and a single superficial drainage vein are usually advantageous for Onyx administration. Unfavorable factors include nidus drainage into multiple compartments as well as multiarterial supply of the AVM, particularly from meningeal arteries, en-passant arteries or perforating feeders. Onyx appears to be a safe and efficient material for embolization of cerebral AVMs, also in cases of intracranial bleeding associated with AVM. Curative embolization of small cerebral AVMs is an efficient and safe alternative to neurosurgical and radiosurgical methods. Careful angiographic assessment of individual arteriovenous malformations should be performed before each Onyx administration.

Extranasopharyngeal angiofibroma: clinical and radiological presentation.

Nasopharyngeal angiofibroma (NA) is a rare, vascular tumor affecting adolescent males. Due to aggressive local growth, skull base location and risk of profound hemorrhage, NA is a challenge for surgeons. Angiofibromas have been sporadically described in extanasopharyngeal locations. We review ten cases of extranasopharyngeal angiofibroma (ENA) and discuss the incidence, clinical presentation and management of this pathology. The group consisted of 4 males and 5 females aged 8-49. There were 7 patients with nasal angiofibroma, 1 patient with laryngeal angiofibroma, 1 patient with oral angiofibroma and another patient with infratemporal fossa tumor. In patients with nasal angiofibroma most common presenting symptoms were nasal obstruction and epistaxis. Patients with laryngeal angiofibroma suffered from mild dysphagia and patients with the infratemporal fossa tumor had painless cheek swelling. In four patients with nasal tumor computed tomography (CT) demonstrated mass with strong to intermediate contrast enhancement. In one patient with nasal tumor carotid angiography demonstrated pathological vessels without intensive tumor blush. Infratemporal fossa tumor showed intensive contrast enhancement on CT and magnetic resonance imaging (MRI) scans, and abundant vascularity on angiography. Laryngeal and oral angiofibroma required no radiological imaging. Three nasal tumors were evaluated before introduction of CT to clinical practice. All patients underwent surgery. No recurrences developed. ENAs differ significantly from NAs regarding clinical and radiological presentations. They lack typical clinical and radiological features as they develop in all age groups and in females, may be less vascularised, arise from various sites and produce a variety of symptoms.

[Chemoembolization (DEM-TACE) in hepatocellularcarcinoma. Report of a case and review of treatment standards in advanced stage disease]. / Zastosowanie chemoembolizacji (TACE) w leczeniu raka watrobowokomórkowego. Opis przypadku oraz przeglqd standardów postepowania w zaawansowanej postaci choroby.

Despite, new diagnostic and treatment techniques, the wide majority of patients are still diagnosed too late and only from 5% to 10% of patients are qualified for tumour surgical resection. Up to date, treatment for advanced, inoperable liver tumors includes systemic chemotherapy, sorafenib therapy and selective, local arterial injection of chemotherapeutic drug into tumour vessels with simultaneous vessel closure - transarterial chemoembolisation (TACE). Beceause of recent development of palliative treatment techniques, the treatment guidelines are changed, especially in advanced stage disease with HCC. Unfortunately, too low percentage of patients is qualified for this type of treatment. We present a case of a patient suffering from advenced stage hepatocellular cancer treated with palliative chemoembolisation using microspheres. In whom, after treatment, regression of the tumor was observed and patient was referred to radical surgery.

[Management of ureteric sticture via a percutaneous double J stent implantation--description of 2 cases]. / Leczenie zwezenia moczowodu za pomoca przezskórnej implantacji cewnika Double J--opis 2 przypadków.

Ureteric stricture is the cause of urinary retention above the stricture level and obstructive nephropathy development with progressive renal parenchyma destruction leading to the renal failure. In the management of ureteric stricture, apart from surgery, less-invasive methods of recanalization are applied: transurethral (retrograde) double J stent implantation or, if the above method is unsuccessful, percutaneous (antegrade) double J stent implantation. In this paper we present 2 cases of percutaneous double J stent implantation: in patient after cystoprostatectomy with ureteric stricture at the level of uretero-ileostomy and in patient after renal transplantation with ureteric stricture at the level of ureterovesicostomy.