Brain iron accumulation in Wilson's disease: A longitudinal imaging case study during anticopper treatment using 7.0T MRI and transcranial sonography.

LEVEL OF EVIDENCE: 5 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:282-285.

Pseudoaneurysm of the sural artery: a rare complication of lower limb angioplasty.

INTRODUCTION: Percutaneous transluminal angioplasty (PTA) is a common therapeutic option for the treatment of peripheral vascular disease. Pseudoaneurysm at the puncture site is a well-documented complication in patients. CASE PRESENTATION: This case report describes a patient who presented to hospital several days following a left superficial femoral and popliteal artery PTA with lower limb pain and swelling. The working diagnosis included a deep vein thrombosis based on the Wells criteria. However, a combination of a duplex scan and computed tomography angiography confirmed a clinically rare occurrence of pseudoaneurysm of the sural artery, a branch of the popliteal artery. This was managed successfully with a thrombin injection, leading to complete resolution of the patient's symptoms. CONCLUSION: This case highlights the importance of the technical aspects of performing a PTA. We believe that the guide-wire position was not confirmed to be completely in the popliteal artery upon inflation of the balloon.

Hypoplastic internal carotid artery stenosis with a low-lying carotid bifurcation causing cerebral ischemia.

Congenital abnormalities of the internal carotid artery (ICA) are infrequent and can be associated with aberrations of the Circle of Willis. A 47-year-old gentleman presented with transient neurological symptoms and cerebral infarction and carotid Doppler showed a stenotic right ICA. Subsequent computed tomographic angiography showed a hypoplastic ICA with a low-lying bifurcation at the C6 level and aplasia of the anterior communicating artery. This patient was commenced on aggressive medical therapy and at 7-month follow-up was symptom-free. This case report highlights the need for a centralized registry with long-term follow-up data in order to identify optimal management.

Complication rates using balloon-expandable and self-expanding stents for the treatment of intracranial atherosclerotic stenoses : analysis of the INTRASTENT multicentric registry.

INTRODUCTION: Using balloon-expandable stents (BES) for treatment of intracranial stenoses, high inflation pressures and rigidity of the device are regarded as major drawbacks limiting feasibility and safety of the procedure. Self-expanding stents (SES) were developed to facilitate lesion access and to allow for less aggressive dilatation. We analyzed data of the INTRASTENT multicentric registry to assess whether self-expanding stents significantly reduced peri-interventional complication rates. METHODS: Records of intracranial stent procedures were entered consecutively into the registry. Datasets were divided into two groups according to the type of stent used. For outcome measurement, we chose three categories: TIA/minor stroke [modified Rankin score (mRS) <2], disabling stroke, and patient death. Clinical outcome was compared between BES and SES. We analyzed types of adverse events occurring in each group in addition. RESULTS: Of 409 atherosclerotic lesions, 254 were treated with BES and 155 with SES. Technical success rates were 97.6% and 98.7%, respectively. Adverse event rates were 4.9%, 3.7%, and 0.8% for TIA/nondisabling stroke, disabling stroke, and death in the BES group compared with 5.3%, 6.0%, and 4.0% in the SES group. The differences were not statistically significant. We observed more perforator strokes after use of BES, but thromboembolic events occurred more often in the SES treatment group. CONCLUSION: Data of the INTRASTENT registry do not support the hypothesis that introduction of SES lowered the overall complication rate of intracranial stent procedures. There might be an advantage using self-expanding stents in vessel segments with important perforating arteries.

In-hospital complication rates after stent treatment of 388 symptomatic intracranial stenoses: results from the INTRASTENT multicentric registry.

BACKGROUND AND PURPOSE: Stenting is increasingly used as an adjunct to medical therapy in symptomatic intracranial stenoses. High periprocedural adverse event rates are one of the limitations of endovascular treatment. Data from the INTRASTENT multicentric registry should demonstrate in-hospital complications at the current stage of clinical development of the stent procedure. METHODS: Participating centers entered the records of all their consecutive intracranial stent procedures into the database. To determine the clinical outcome in the acute phase, we distinguished transient ischemic attack/nondisabling stroke (modified Rankin Scale <2), disabling stroke, death, and intracranial hemorrhage as clinical complications and analyzed whether they were associated with patient- or stenosis-related risk factors. RESULTS: Data from 372 patients with 388 stenoses proved 4.8% disabling strokes and 2.2% deaths. Transient or minor events were detected in 5.4% of the cases. Hemorrhagic events (3.5%) occurred more frequently after treatment of middle cerebral artery stenoses (P=0.004) and were associated with significantly higher morbidity and mortality rates. Ischemic strokes by compromise of perforating branches were detected mainly in the posterior circulation. However, the overall rate of severe adverse events was not dependent from location, degree, and morphology of the stenosis or from patient's age, gender, vascular risk factors, or type of qualifying event. CONCLUSIONS: The complication rates within the registry are within the limits of previously published data. Severe adverse events were equally distributed between potential risk groups with similar rates but different types of main complications in the anterior and posterior circulation.

Real-World Treatment Patterns of Disease Modifying Therapy (DMT) for Patients with Relapse-Remitting Multiple Sclerosis and Patient Satisfaction with Therapy: Results of the Non-Interventional SKARLET Study in Slovakia.

BACKGROUND: During long-term multiple sclerosis therapy, patient satisfaction with received treatment has considerable impact on treatment outcomes. Here we report the results of a non-interventional real-world study that mapped the treatment patterns of disease-modifying therapy (DMT) and assessed treatment satisfaction with DMT. PATIENTS AND METHODS: The SKARLET study was a non-interventional, cross-sectional study in Slovakia running from May 2016 to March 2017. Patients with relapsing-remitting multiple sclerosis on DMT for ≥3 months and ≤2 years (per local labelling) from 10 multiple sclerosis centers across Slovakia were included. The primary objective was to collect the Treatment Satisfaction Questionnaire for Medication version 9 (TSQM 9) score regarding perceived effectiveness, convenience and overall satisfaction with DMT. RESULTS: The following TSQM 9 scores (mean; 95% confidence interval) were reported from 415 patients: convenience (75.05; 73.49-76.61), effectiveness (68.15; 66.56-69.75) and global satisfaction scale (66.94; 65.26-68.62). All three parameters of the TSQM 9 were analyzed by the route of DMT administration, with infusions best rated for effectiveness and global satisfaction in comparison to oral dosage and injections. For convenience, however, oral dosage forms were appraised highly (82.66; 80.59-84.73) followed by infusions (74.40; 70.12-78.69), while injections were rated as the worst (66.92; 64.81-69.04). The difference of TSQM 9 scores according to the route of DMT administration is statistically significant for convenience (p < 0.001) and global satisfaction (p = 0.004), but not for effectiveness (p = 0.185). CONCLUSION: In the present study, it was confirmed that patients find oral DMTs as most convenient; however, the infusion form of treatment outweighs oral DMTs in global satisfaction and effectiveness. The differences of TSQM 9 scores among DMT dosage forms were significant for convenience and global satisfaction. In conclusion, the results of this detailed survey increase our understanding of RRMS patient population characteristics and patient satisfaction with DMT treatment.

Digitized Image Analysis of Insula Echogenicity Detected by TCS-MR Fusion Imaging in Wilson's and Early-Onset Parkinson's Diseases.

Transcranial sonography (TCS) can reveal pathology in brain structures including insula. This study compared insula echogenicity among 22 patients with Wilson's disease (WD), 21 patients with early-onset Parkinson's disease (EO-PD) and 24 healthy patients. Echogenicity of predefined brain structures (insula, lentiform nucleus, caudate nucleus, substantia nigra and raphe nuclei) was evaluated using digitized analysis of TCS fusion imaging with magnetic resonance. Cortical, subcortical and cerebellar atrophy and ventricle diameters were determined from magnetic resonance images. The mean echogenicity index of insula did not differ between males and females (p = 0.92), but the echogenicity of insula was higher in patients with WD than in patients with EO-PD and healthy patients (p < 0.05). The substantia nigra echogenicity was higher in patients with EO-PD, and lentiform nucleus echogenicity was higher in patients with WD (p < 0.05). The echogenicity of insula correlated with lentiform nucleus echogenicity (r = 0.75) but not with age (r = -0.14), disease duration (r = -0.36), symptom severity (r = 0.28), cortical (r = 0.11) nor subcortical (r = 0.05) atrophy.

Real-life LDL-C treatment goals achievement in patients with heterozygous familial hypercholesterolemia in the Czech Republic and Slovakia: Results of the PLANET registry.

BACKGROUND AND AIMS: Despite the high prevalence of familial hypercholesterolemia (FH) and available effective lipid-lowering therapy, most of the individuals with this disorder remain undiagnosed and undertreated. The aim of the PLANET registry was to assess the real-life attainment of low-density lipoprotein cholesterol (LDL-C) therapeutic target level in patients with heterozygous FH, to characterize prescribed lipid-lowering therapy with assessment of its efficiency according to the attainment of the target LDL-C level, and to characterize cardiovascular events observed in this patient population again in relation to LDL-C target level attainment. METHODS: PLANET registry was designed as a non-interventional, retrospective, cross-sectional, multicentre disease registry for adult patients with heterozygous FH in the Czech Republic and Slovakia. RESULTS: Overall, 1755 patients were enrolled at 32 sites specialized in FH treatment. 15.4% of patients attained the target LDL-C value. The proportion of patients with LDL-C goal achievement increased to 17.3% in the subgroup of patients receiving high-intensity statin therapy (54.6% of study population). Out of 55 patients receiving inhibitors of proprotein convertase subtilisin/kexin type 9 (PCSK9), 61.8% reached the LDL-C treatment goal. Of all cardiovascular events reported, 14.0% occurred in patients attaining the LDL-C goal, while it was 86.0% in the not-at-target group. It was documented (p=0.004) that the longer is the patient in care at the specialized FH centre, the higher is the probability that he/she will attain the target LDL-C level. CONCLUSIONS: Although target LDL-C level attainment remains relatively low, the likelihood of LDL-C goal attainment increases with duration of specialized care.

Pathological findings of uterine leiomyomas and adenomyosis following uterine artery embolization.

Uterine artery embolization (UAE) is an effective and accepted treatment option for symptomatic uterine leiomyoma. Between 2000 and 2005, 91 women were treated using this method, and were prospectively followed at our institution. Twenty of them subsequently underwent surgery. One of these patients was subjected to four surgical procedures. We describe the pathological findings of 23 surgical specimens obtained from these 20 patients. The embolic material used consisted of tris-acryl gelatin microspheres (TGMS) in 15 patients (18 surgical specimens), polyvinyl alcohol particles (PVA) in three patients, and a combination of PVA and TGMS in two patients. Histologically, of the 23 specimens examined, 20 were diagnosed as leiomyomas, and three as adenomyosis. Particles used for embolization were found in all but three specimens. Necrosis was present in 15 of 20 leiomyoma specimens. Hyaline necrosis was found in 12 specimens, coagulative necrosis in one case, and a combination of hyaline and coagulative or suppurative necrosis in two cases. The foci of adenomyosis remained unaltered.

Uterine fibroid embolization versus myomectomy in women wishing to preserve fertility: preliminary results of a randomized controlled trial.

OBJECTIVE: To compare the clinical results of surgical and endovascular treatment of uterine myomas in young women. STUDY DESIGN: In a university-affiliated, tertiary care center a prospective trial was conducted. Sixty-three women, wishing to retain fertility, with intramural fibroid(s) larger than 4 cm, were randomly selected either for uterine artery embolization or myomectomy. Invasiveness, efficacy, and complications of both procedures were compared. RESULTS: Thirty embolizations and 33 myomectomies (15 laparoscopic, 18 open) were performed. The mean follow-up was 17 months. In embolized patients, there was a significantly shorter procedure length (p<0.0001), hospital stay (p<0.001) and disability period (p<0.0001), lower CRP (p<0.001) and higher hemoglobin (p<0.0001) concentrations the 2nd day after procedure. But there was a higher incidence of re-interventions (p<0.01) and a lower rate of total symptomatic relief (p<0.1). The groups did not significantly differ in: technical success rate, febrile morbidity, FSH levels 6 months after the procedure, and complication rates. CONCLUSIONS: Although the reproductive outcomes of uterine artery embolization and myomectomy cannot be evaluated at the moment, our first results indicate that both methods are clinically successful in the majority of cases and are not connected with significant number of serious complications.

Comparison of transcranial sonography-magnetic resonance fusion imaging in Wilson's and early-onset Parkinson's diseases.

INTRODUCTION: Wilson's disease (WD) is a hereditary disorder caused by ATP7B mutations resulting in systemic copper accumulation. WD may manifest as early-adulthood parkinsonism; and atypical cases may be difficult to distinguish from early-onset Parkinson's disease (EO-PD), a neurodegenerative disorder with onset ≤40 years of age. The aim of our study was to compare transcranial sonography (TCS)-magnetic resonance fusion imaging in WD and EO-PD and examine whether TCS can provide clinically useful information. METHODS: We examined 22 WD, 16 EO-PD, and 24 healthy control subjects. We measured echogenicity and determined presence of MRI signal changes in T2-weighted images in the substantia nigra (SN) and lentiform nucleus (NL). TCS with the capability of magnetic resonance fusion and Virtual Navigator was used. The echogenicity indices of SN and NL were processed using digital image analysis to eliminate subjective evaluation errors. RESULTS: Mean SN echogenicity index in EO-PD (39.8 ± 5.9 [SD]) was higher compared to WD (28.0 ± 4.6, p < 0.0001) and control subjects (28.8 ± 4.9, p < 0.0001). Mean NL echogenicity index was higher in WD (117.5 ± 37.0) compared to EO-PD (61.6 ± 5.4, p < 0.0001) and control subjects (54.9 ± 11.2, p < 0.0001). The SN hyperechogenicity had sensitivity 93.8%, and specificity 90.9%, while the NL hyperechogenicity had sensitivity 95.5% and specificity 93.8% for differentiation of WD and EO-PD. NL hyperechogenicity was more pronounced in WD subjects with putaminal MRI T2 hyperintensity (p < 0.05) but was also present in subjects without MRI abnormality. CONCLUSIONS: There are distinct TCS findings in WD and EO-PD complementary to MRI that can be utilized as highly sensitive and specific biomarkers of these disorders.

Hepatic vein stenosis and inferior vena cava stenosis in a patient with chronic obstructive pulmonary disease.

The case is presented of a patient with stenosis of the intrahepatic portion of the inferior vena cava and the terminal part of hepatic veins, a state manifesting itself in ascites resistant to treatment. The case was originally seen as decompensated cirrhosis of the liver. Once properly diagnosed, it was successfully treated with angioplasty and subsequent anticoagulation. After all the relevant hypercoagulation states potentially leading to IVC and hepatic vein stenosis were ruled out, the cause of the patient's stenosis could be traced to: 1. secondary polycythemia concomitant to severe pulmonary obstruction disease and 2. simultaneous corticosteroid therapy. This cause has not been described in literature before.

Dialysis access-associated steal syndrome: the role of ultrasonography.

BACKGROUND: Ischemic steal syndrome is an infrequent, but potentially disabling complication of hemodialysis (HD) access creation. We analyzed the ability of duplex Doppler ultrasonography to reveal potential causes of ischemic steal syndrome in antebrachial accesses. METHODS: We performed 212 examinations on 121 patients. Ten patients suffered from ischemic steal syndrome. Complete length access evaluation was performed by a linear array 7.5 MHz ultrasound probe. RESULTS: Hand ischemia was explained by inflow artery stenosis in five cases. Excessive fistula flow due to large arteriovenous anastomosis was the suspected cause in two cases. The remaining two cases were characterized by high-resistant minimal flow in the ulnar artery with bi-directional flow in the distal part of the radial artery, suggesting stenoses located in the arcus palmaris. Duplex Doppler ultrasound had not revealed the etiology of clinically apparent hand ischemia in one case. Isolated inflow artery stenoses were treated successfully by percutaneous transluminal angioplasty. Patients with ischemic steal syndrome and high fistula flow were treated successfully by outflow vein banding. CONCLUSIONS: Duplex Doppler ultrasonography is a valuable tool for diagnosing the cause of ischemic steal syndrome and can probably replace angiography in some cases.

Many clinically silent access stenoses can be identified by ultrasonography.

BACKGROUND: Stenoses of vascular accesses are leading factors limiting access survival. Besides physical examination, screening of access stenoses is based mainly on the "dysfunction hypothesis", which states that progressive stenosis causes graft dysfunction, such as decreased flow. We tested whether Doppler ultrasonography could detect a number of clinically hidden access stenoses in otherwise well-managed patients. Indications from clinical evaluation regarding the presence of stenosis were compared with ultrasound findings. METHODS: We made 258 examinations in 193 patients. Whole-length morphological ultrasound examinations of vascular access were done with a 7.5 MHz linear array transducer. The combination of > 50% stenosis in B-mode and at least doubling of peak systolic velocity was the criterion for significant stenosis. We compared the specificity and sensitivity of clinical diagnosis or ultrasound. RESULTS: The sensitivity and specificity of the clinical diagnosis of stenosis were 35.8% and 92.8%, respectively. CONCLUSIONS: A considerable number of otherwise appropriately managed hemodialysis patients suffer from significant access stenosis. Wider use of Doppler ultrasonography would increase the proportion of clinically hidden stenoses diagnosed in time and thus might prolong access patency. Clinical suspicion of access stenosis is highly specific and such patients should be examined directly by angiography.

Uterine artery embolization versus laparoscopic uterine artery occlusion: the outcomes of a prospective, nonrandomized clinical trial.

PURPOSE: To compare outcomes of two different types of occlusive therapy of uterine fibroids. METHODS: Women with fibroid(s) unsuitable for laparoscopic myomectomy (LM) were treated with uterine artery embolization (UAE) or laparoscopic uterine artery occlusion (LUAO). RESULTS: Before the procedure, patients treated with UAE (n = 100) had a dominant fibroid greater in size (68 vs. 48 mm) and a mean age lower (33.1 vs. 34.9 years) than surgically treated patients (n = 100). After 6 months, mean shrinkage of fibroid volume was 53 % after UAE and 39 % after LUAO (p = 0.063); 82 % of women after UAE, but only 23 % after LUAO, had complete myoma infarction (p = 0.001). Women treated with UAE had more complications (31 vs. 11 cases, p = 0.006) and greater incidence of hysteroscopically verified intrauterine necrosis (31 vs. 3 %, p = 0.001). Both groups were comparable in markers of ovarian functions and number of nonelective reinterventions. The groups did not differ in pregnancy (69 % after UAE vs. 67 % after LUAO), delivery (50 vs. 46 %), or abortion (34 vs. 33 %) rates. The mean birth weight of neonates was greater (3270 vs. 2768 g, p = 0.013) and the incidence of intrauterine growth restriction lower (13 vs. 38 %, p = 0.046) in post-UAE patients. CONCLUSION: Both methods are effective in the treatment of women with future reproductive plans and fibroids not suitable for LM. UAE is more effective in causing complete ischemia of fibroids, but it is associated with greater risk of intrauterine necrosis. Both methods have low rate of serious complications (except for a high abortion rate).

Comparative outcomes of hysteroscopic examinations performed after uterine artery embolization or laparoscopic uterine artery occlusion to treat leiomyomas.

Hysteroscopic examination of the uterine cavity revealed that patients previously treated for intramural myoma(s) by uterine artery embolization had a significantly higher incidence of abnormal findings compared with patients treated by laparoscopic occlusion of uterine arteries (59.5% vs. 2.7%). In particular, there was a higher incidence of necrosis in the uterine cavity of patients subjected to uterine artery embolization (43.2%) compared with patients after surgical uterine artery occlusion (2.7%).

Pregnancy outcomes after uterine artery occlusion: prospective multicentric study.

OBJECTIVE: To assess the reproductive outcomes after laparoscopic uterine artery occlusion (LUAO) and uterine artery embolization (UAE) in women with symptomatic fibroids. DESIGN: Prospective, clinical multicentric study. SETTING: Endoscopic center in the department of obstetrics and gynecology at a hospital in the Czech Republic. PATIENT(S): Thirty-eight pregnant women after LUAO and 20 pregnant women after UAE. INTERVENTION(S): Laparoscopic uterine artery occlusion and UAE. MAIN OUTCOME MEASURE(S): Pregnancy, abortion, preterm delivery, and live-birth rates. RESULT(S): Pregnancies after uterine embolization had a statistically significantly higher rate for spontaneous abortion (56%) than did pregnancies after surgical uterine artery occlusion (10.5%). The risk of malpresentation (20%) and the rate for cesarean section (80%) after UAE similarly were higher than was the risk after laparoscopic occlusion; however, these differences were not statistically significant. Also, there were no significant differences between the groups in preterm deliveries (15.3% in the LUAO group vs. 20% in the UAE group). CONCLUSION(S): Pregnancies of women who were treated with uterine embolization were at significantly increased risk for spontaneous abortion when compared with pregnancies of women treated with LUAO.

Midterm clinical and first reproductive results of a randomized controlled trial comparing uterine fibroid embolization and myomectomy.

The purpose of this study was to compare the midterm results of a radiological and surgical approach to uterine fibroids. One hundred twenty-one women with reproductive plans who presented with an intramural fibroid(s) larger than 4 cm were randomly selected for either uterine artery embolization (UAE) or myomectomy. We compared the efficacy and safety of the two procedures and their impact on patient fertility. Fifty-eight embolizations and 63 myomectomies (42 laparoscopic, 21 open) were performed. One hundred eighteen patients have finished at least a 12-month follow-up; the mean follow-up in the entire study population was 24.9 months. Embolized patients underwent a significantly shorter procedure and required a shorter hospital stay and recovery period. They also presented with a lower CRP concentration on the second day after the procedure (p < 0.0001 for all parameters). There were no significant differences between the two groups in the rate of technical success, symptomatic effectiveness, postprocedural follicle stimulating hormone levels, number of reinterventions for fibroid recurrence or regrowth, or complication rates. Forty women after myomectomy and 26 after UAE have tried to conceive, and of these we registered 50 gestations in 45 women. There were more pregnancies (33) and labors (19) and fewer abortions (6) after surgery than after embolization (17 pregnancies, 5 labors, 9 abortions) (p < 0.05). Obstetrical and perinatal results were similar in both groups, possibly due to the low number of labors after UAE to date. We conclude that UAE is less invasive and as symptomatically effective and safe as myomectomy, but myomectomy appears to have superior reproductive outcomes in the first 2 years after treatment.

Hysteroscopy after uterine fibroid embolization in women of fertile age.

AIM: Uterine artery embolization for fibroids is a controversial issue for women with incomplete reproductive plans. Ovarian failure and uterine infection are the most dreaded complications of this procedure. The purpose of the present study was to assess the types and the frequency of intrauterine abnormalities and the histological features of the endometrium after embolization. METHODS: Uterine artery embolization was performed on 51 women (average age 34.5 years) with intramural fibroid/s larger than 4 cm. Hysteroscopy and endometrial biopsy was performed from 3 to 9 months later in the luteal phase of the cycle. RESULTS: Despite all women having no major symptoms prior to hysteroscopy, only 19 (37%) had completely normal hysteroscopic findings. There was intrauterine protrusion of fibroid/s in 19 cases (37%), yellowish coloration of the endometrium in 14 (28%), intrauterine or cervical adhesions in seven (14%), and communication between the myoma and the uterine cavity in five cases (10%). A normal, functional endometrium was histologically verified in 44 women of 49 (90%) who could be evaluated. Regressive changes (necrosis or hyalinization) of leiomyoma or of indefinite origin were found in 17 patients and embolization particles in five, including one patient with microspheres inside the endometrial vessel. No case of Asherman syndrome or endometrial atrophy was observed. CONCLUSION: The frequency of abnormal hysteroscopic findings after embolization is surprisingly high. The clinical significance, reversibility, and impact on fertility of abnormal hysteroscopic findings after embolization remain unclear. Regardless, hysteroscopy should be strongly recommended to all patients after uterine fibroid embolization, prior to conception.