Parkes Weber syndrome (PWS) is a rare congenital condition characterized by capillary cutaneous malformation, limb hypertrophy and multiple arteriovenous fistulas of the affected extremity. Another feature is a port-wine stain on the affected area. PWS is caused by genetic variations in the RAS p21 protein activator (RASA1) gene which affects the development of the vascular system. We report a case of a female neonate presenting with dyspnoea and cardiovascular insufficiency at the time of birth. The left upper extremity (LUE) and shoulder were enlarged (circumference at the midpoint was 17cm compared to 11cm on the right arm), edematous, hyperemic with a port-wine stain. Structural changes of the bones of LUE were discovered on X-ray. Echocardiography revealed right-sided volume overload, a large ductus arteriosus, a possible pathology of the aortic arch and branch arteries. Chest X-rays showed cardiomegaly. Therapy with milrinone and diuretics was started. A multislice CT angiography scan revealed arteriovenous fistula (AVF) between a. subclavia sin. and v. brachiocephalica sin, arteriovenous malformations (AVM) and a dilated a.subclavia sin. of 11 mm, as well as dilatation of other arteries of the LUE. Next generation sequencing revealed a pathogenic variation (c.2245C>T, p.Arg749*) in the RASA1 gene in the heterozygous state. Four consecutive embolizations of the AVM and AVF were performed in the first 16 months.
Introduction: First-pass effect (FPE) is associated with improved clinical outcomes and reduced mortality in mechanical thrombectomy (MT) for large vessel occlusion strokes (LVOS). However, FPE is achieved in only 30-40% of patients with the current devices. The RapidPulse TM Cyclic Aspiration System (RP) is a novel technology consisting of a valve box that precisely cycles pressure from full to no vacuum multiple times per second adding kinetic energy to the suction forces. Initial clinical evaluation suggests that the system can achieve FPE rates in the 70% range. We aim to evaluate the RP as a frontline approach in LVOS. Methods: Prospective, multicenter, open-label, core lab adjudicated, two-arm study comparing the safety and efficacy of the RP System with non-randomized retrospective controls who were consecutively treated at the study sites based on similar eligibility criteria. Patients with LVOS involving the anterior or posterior circulations in whom the target lesion could be treated with the Medtronic React 71 aspiration catheter up to 24 hours from stroke onset were included in the RP arm. Controls consisted of comparable patients treated with 070-072 ID catheters. The primary outcome was the rate of FPE (complete/near reperfusion [mTICI ≥2c] after a single pass). Secondary outcomes include frontline technical success (defined as mTICI ≥2b after final device pass with no rescue therapy), final mTICI after all passes, symptomatic ICH, device-related complications, the proportion of patients achieving a modified Rankin Scale score of 0-2 at 90 days, and all-cause 90-day mortality. The study will enroll a maximum of 100 participants in the RP and 200 in the control arm at 5 centers in Spain, Turkey, Denmark, Latvia, and Brazil. Results: Final results will be presented at the conference. Conclusion: RapidPulseFS is the first prospective clinical trial aiming to compare cyclic versus standard aspiration technologies. This novel device may allow clinicians to achieve faster and better reperfusion while significantly reducing the disposable device costs associated with treating LVOS (ClinicalTrials.gov Identifier: NCT05122637).
Intracranial aneurysms (IAs) are most commonly found at the branch points of large arteries that form the circle of Willis. The prevalence of IAs in the adult population is 1–5%. IAs rupture is associated with subarachnoid haemorrhage (SAH) in 6–8 cases per 100 000 population, causing mortality in 40–50%. Aneurysm treatment is used to prevent rupture or rebleeding (for ruptured IAs). Randomised trials demonstrated the superiority of endovascular treatment (EVT) of ruptured aneurysms with coil systems over surgery. The objective of the study was to evaluate the effectiveness of the Barricade coil system in the treatment of intracranial aneurysms. Detachable platinum coils, since their introduction 25 years ago, have become the first choice EVT method for ruptured and unruptured IAs and have shown acceptable mortality (~2%). The retrospective study of intracranial aneurysms treated with the Barricade coil system at Pauls Stradiņš Clinical University Hospital (Rīga, Latvia) conducted in a 20-month period included 95 patients and 97 IAs. Thirty-one (32.6%) males and 64 (67.4%) females with median age 56 ± 15 years underwent endovascular treatment. The minority, 22 (23.16%) patients, were asymptomatic, while 73 (76.84%) patients had neurological symptoms directly associated with aneurysm progression and SAH development. Preoperatively, 52 (53.6%) aneurysms were ruptured, causing SAH, and 24 (25.26%) patients with unruptured IAs had neurological symptoms. Sixty-four (66.0%) IAs were treated using coils without neurovascular stent implantation or balloon assistance, 22 (22.7%) — with coils and stent implantation, and 11(11.3%) aneurysms were embolised with balloon-assisted coiling. The immediate anatomical result of endovascular treatment of IAs and technical success of aneurysm coiling was evaluated using the simplified Raymond scale. In the majority of cases, complete occlusion of the aneurysm was achieved while residual neck of the aneurysm or aneurysm remnant was uncommon. Immediate clinical results were evaluated using the modified Rankin scale (mRs). The majority of patients had favourable immediate clinical outcome (mRs 0–2), but four (4.21%) patients died in 1–6 days after the procedure as a consequence of SAH. In 72 (75.79%), patients no new neurological pathological symptoms developed 2–3 days after endovascular procedure and they were discharged from the hospital. Intraprocedural complications occurred in 4 (4.21%) cases. Technical issues occurred in two (2.1%) patients. In 19 (20%) patients, neurological symptoms remained even after the procedure, six (6.32%) patients had clinical worsening, and we had one case of procedural related mortality. Six-month follow-up evaluation was performed for 58 (61.0%) patients (59 IAs). In the majority of cases, complete occlusion of the aneurysm and favourable clinical outcome (mRs 0–2) was observed. Our experience showed that the treatment of ruptured and unruptured intracranial aneurysms with the Barricade coil system is feasible, effective, clinically safe and has a low risk of intraprocedural complications.
Background: Uterine artery embolisation (UAE) is an accepted treatment option for the majority of uterine fibroids. UAE in the case of submucosal fibroids is more contentious given the potential risk of expulsion of necrotic material. The aim of this study was to determine the suitability of UAE in cases where submucosal fibroids exist and to assess the procedural outcomes and complications. Method(s): Retrospective data was collected for UAEs conducted over a five-year period at a single tertiary centre. Patients with pre-and post-procedure MRI studies as well as submucosal fibroids were included. Alterations in the volume of the uterus and size of the dominant submucosal fibroid were calculated pre-and post-UAE. Post-UAE complications and surgical interventions were also documented. Patient satisfaction was assessed using the 40-point uterine fibroid symptom quality of life (UFS-QoL) questionnaire. Result(s): Between 2013-2018, 281 female patients underwent UAE. Of these, 26 (9.3%) patients were found to have submucosal fibroids (mean age 47.5±5.0 years; range: 35-56). The mean pre-UAE uterine volume was 986.5±565.1 CC, while mean post-UAE uterine volume was 666.9±542.0 CC (p<0.05). The mean dominant submucosal fibroid size pre-UAE was 5.3±2.5 cm, and post-UAE was 3.25±2.74 cm (p<0.05). 100% of fibroids were effectively devascularised. 7.7% of patients experienced post-UAE pelvic infection. 41.2% patients underwent further surgical intervention, while 58.8% were discharged from clinic. Conclusion(s): UAE is a safe and efficacious treatment option for submucosal fibroids, however a high percentage of patients may require adjunctive surgical intervention to augment therapeutic results.
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