Citations

AbstractWhile pediatric epilepsy shares some features with adult epilepsy, the growing and developing brain during childhood is associated with differences in epilepsy etiologies, syndromes, comorbidities, management, and outcomes. We must also consider the long-term effects of epilepsy and treatments. This section highlights unique considerations for pediatric epilepsy. These include pediatric onset electroclinical syndromes that commonly resolve prior to adulthood, treatment duration of self-limited pediatric epilepsy syndromes, pediatric-onset epilepsy with etiology-specific treatment, and therapies used more commonly in the pediatric population. In addition, pediatric-onset epilepsy is associated with a higher risk for developmental, psychiatric, and behavioral comorbidities in a bidirectional fashion. Family dynamics are affected by a child with epilepsy, and transitioning to the adult clinic is a particular challenge for this population. Finally, there are special issues that occur in pediatric epilepsy, including the lack of pediatric FDA approvals for newly available therapies and the need for sedation for investigations.

AbstractLiver transplantation is becoming increasingly common in the United States, with very favorable 1- and 5-year survival rates. With increasing long-term survival rates, postprocedural vascular complications can occur with increased frequency. Familiarity with posttransplant vascular anatomy is essential for interventional radiologists dealing with this patient population. This article discusses the posttransplant venous, arterial, and biliary anatomy and the implications for minimally invasive interventions.

Abstract Prolactinomas comprise half of all pituitary adenomas. This study explores the significance of measuring intraoperative (IO) prolactin (PRL) levels in guiding surgical decision-making and predicting operative success. Retrospective cohort study design of consecutive patients with prolactinomas who underwent transsphenoidal resection from June 2021 to May 2023. IO PRL was measured after tumor resection was completed. Remission was defined as normalization of PRL levels during follow-up without medication. Outcomes included factors associated with remission, utilizing significance thresholds at p < 0.05. Thirteen patients were included with a 5.3-month median follow-up (range, 3–15 months). Most of the tumors were microadenomas (61%), with a median preoperative PRL level of 116 ng/mL (range, 25–471). Gross total resection was achieved in 69% of patients, all of whom attained remission. The median IO PRL was 19.8 ng/mL (range, 1–329), the postoperative day 1 PRL of 3.6 ng/mL (range, 1–203), and the latest PRL at 9.3 ng/mL (range, 1–137). A ≥40% decline in IO PRL from baseline was identified as the optimal cutoff for predicting biochemical remission, with 89% sensitivity, 75% specificity, and 85% accuracy. Monitoring PRL levels intraoperatively may be a useful biomarker aiding surgeon's assessment of the extent of tumor resection and guide surgical decision-making. Further refinement of the immunoassay assay and clinical testing with more extensive prospective studies are needed.

AbstractAn in-depth understanding of portal venous anatomy, particularly its variants, is essential for interventional radiologists performing complex hepatobiliary procedures such as transjugular intrahepatic portosystemic shunt (TIPS) creation. Anatomical variations in the portal venous system occur in approximately 35% of the population, and failure to identify these variants can result in significant procedural complications including vascular injury and postprocedural liver failure. This article discusses the most common (types I–V) portal vein variants and the importance of their preprocedural identification, their potential impact on procedural outcomes, and the role of advanced imaging techniques in mitigating risks associated with these anatomical variations.

AbstractNew understanding of the lymphatic system as well as increasing recognition of its importance in various pathologies have increased the opportunity to perform lymphatic interventions. Advancement in our understanding of the lymphatic system anatomy, including variations, combined with improved imaging has renewed interest in both surgical and interventional management of a wide range of complex lymphatic pathologies. It is important for the proceduralist to recognize the anatomy, variability, and physiology of the lymphatic system, including lower extremity lymphatic drainage, cisterna chyli location, and thoracic duct drainage. The purpose of this study is to convey the practical aspects of performing lymphatic interventions in an effort to improve the speed and reliability of these procedures.

AbstractCholecystectomy is the standard treatment for acute cholecystitis, but it may not be suitable for all patients. For those who cannot undergo surgery, a percutaneous cholecystostomy tube (PCT) and ERCP-guided transpapillary gallbladder drainage are viable options. We aimed to perform a nationwide study to assess 30-day readmission rates, adverse events (AEs), and mortality rates in these two cohorts.We conducted a nationwide cohort study using data from the Nationwide Readmissions Database (NRD) from 2016 to 2019. We identified patients with acute cholecystitis during the index admission who underwent either PCT or ERCP-guided gallbladder drainage. Propensity score matching along with multivariable regression was used to compare cohorts.During the study period, 3,592 patients (average age 63.0 years) underwent endoscopic drainage, whereas 80,372 patients (average 70.8 years) underwent Interventional Radiology drainage. Utilizing multivariate Cox regression analysis, compared with ERCP, PCT had a higher risk for 30-day readmission (adjusted hazard ratio [aHR] 1.47; 95% confidence interval [CI] 1.27 to 1.71; P < 0.001). The PCT group had a significantly higher rate of readmission for acute cholecystitis compared with the ERCP group (2.72% vs 0.86%; P < 0.005). Cox proportional hazard ratio showed a 3.41-fold increased risk (95% CI 1.99 to 5.84) for readmission in the PCT group. ERCP was consistently associated with lower rates of post-procedural AEs compared with PCT including acute hypoxemic respiratory failure (P < 0.001), acute renal failure (P < 0.001), shock (P < 0.001), and need for blood transfusions (P < 0.001).Our nationwide analysis revealed that ERCP-guided gallbladder drainage should be the preferred approach for managing acute cholecystitis when unfit for surgery.

AbstractEndoscopic ultrasound-guided gallbladder drainage (EUS-GBD) for symptomatic gallbladder disease has been shown to be safe and effective in patients with high surgical risk, but data are lacking for patients with cirrhosis. We investigated the safety and effectiveness of EUS-GBD in patients with and without cirrhosis.This retrospective review included patients who underwent EUS-GBD at four (three US and one Spanish) international tertiary care centers. Outcomes, including technical success, clinical success, and procedure-related adverse events, were compared between patients with and without cirrhosis.170 patients (47 with cirrhosis, 123 without cirrhosis) were included. There was no difference in age, sex, race, comorbidities, antiplatelet use, hemoglobin, or international normalized ratio between the two groups (P > 0.05 for all). The most common etiology of cirrhosis was alcohol (42.6%) with mean Model of End-stage Liver Disease-Sodium (MELD-Na) score of 16.2 (SD 8.8). Acute cholecystitis was more common in patients with cirrhosis (74.5% vs. 56.9%; P = 0.02). Technical (cirrhosis 97.9% vs. no cirrhosis 95.1%; P = 0.67) and clinical (93.6% vs. 94.9%; P = 0.71) success rates were similar in the two groups. Adverse events were infrequent and similar between groups, the most common being stent maldeployment (cirrhosis 4.3% vs. no cirrhosis 5.7%; P = 0.99). Survival rates were similar at the end of follow-up.EUS-GBD was safe and effective in patients with cirrhosis, with outcomes similar to patients without cirrhosis when performed by experienced echoendoscopists. Patients with cirrhosis and symptomatic gallbladder disease can be considered for EUS-GBD.

Abstract Background Tafamidis is a kinetic stabilizer that binds to the transthyretin (TTR) gene, inhibiting its dissociation. It is the only disease-modifying treatment for hereditary TTR amyloidosis with peripheral neuropathy (ATTRv-PN) available in the National Therapeutic Form (Formulário Terapêutico Nacional, FTN, in Portuguese) of the Brazilian Unified Health System (Sistema Único de Saúde, SUS, in Portuguese). Objective To assess if the efficacy and safety of tafamidis in the Brazilian real-world experience are comparable to the results of clinical trials. Methods We retrospectively studied all patients with ATTRv-PN evaluated at our center from September 2011 to March 2022 (data cut-off) who were initiated on tafamidis and had at least 1 follow up visit 6 months after the initiation of the drug treatment. Neurologic and functional outcomes were compared from day 1 (D1) of the tafamidis treatment (baseline) to the last follow-up. Results In total, 33 patients were included, 18 (55%) of whom were female. All patients were carriers of the V30M mutation of ATTRv-PN, and 20 (61%) presented early onset (EO) ATTRv-PN. At baseline, the median age of the sample was of 40 (interquartile range [IQR]: 36–68) years, the median Neuropathy Impairment Score (NIS) was of 10 (6–24) points, and the median body mass index (BMI) was of 26 (23–28) kg/m2. The mean follow-up time was of 3.4 years. At the last follow-up, the BMI, the neurological impairment, and the level of disability slightly worsened compared with baseline, while the findings of the nerve conduction studies remained stable. These same results were observed across EO and late-onset (LO) ATTRv-PN patients. A total of 25 (75.8%) patients were considered responders, and 8 (24.2%), non-responders. Conclusion The efficacy and safety of tafamidis reported in clinical trials is expandable to the Brazilian real-world scenario in EO and late-onset (LO) ATTRv-PN.

AbstractEUS-guided gallbladder drainage (EUS-GBD) using lumen apposing metal stents (LAMS) has excellent technical and short-term clinical success for acute cholecystitis (AC). The goals of this study were to determine the long-term clinical outcomes and adverse events (AEs) of EUS-GBD with LAMS.A multicenter, retrospective study was conducted at 18 US tertiary care institutions. Inclusion criteria: any AC patient with attempted EUS-GBD with LAMS and minimum 30-day post-procedure follow-up. Long-term clinical success was defined as absence of recurrent acute cholecystitis (RAC) > 30 days and long-term AE was defined as occurring > 30 days from the index procedure.A total of 109 patients were included. Technical success was achieved in 108 of 109 (99.1%) and initial clinical success in 106 of 109 (97.2%). Long-term clinical success was achieved in 98 of 109 (89.9%) over a median follow-up of 140 days (range 30–1188). On multivariable analysis (MVA), acalculous cholecystitis (odds ratio [OR] 15.93, 95% confidence interval [CI] 1.22–208.52, P = 0.04) and the occurrence of a LAMS-specific AE (OR 63.60, 95% CI 5.08–799.29, P <0.01) were associated with RAC. AEs occurred in 38 of 109 patients (34.9%) at any time, and in 10 of 109 (9.17%) > 30 days from the index procedure. Most long-term AEs (7 of 109; 6.42%) were LAMS-specific. No technical or clinical factors were associated with occurrence of AEs. LAMS were removed in 24 of 109 patients (22%). There was no difference in RAC or AEs whether LAMS was removed or not.EUS-GBD with LAMS has a high rate of long-term clinical success and modest AE rates in patients with AC and is a reasonable destination therapy for high-risk surgical candidates.