Safety and Feasibility of Gun-Sight Technique for Transjugular Intra-hepatic Portosystemic Shunt (TIPS) Creation.

PURPOSE: To review technical details, indications for use, success rates and complications of gun-sight technique for transjugular intra-hepatic portosystemic shunt (TIPS) creation. MATERIALS AND METHODS: A multicenter retrospective review was performed. Forty-two TIPS procedures with gun-sight technique were identified between 2016 and 2021. Eighty-six percent of patients had portal vein thrombosis (PVT), and 21% had undergone prior failed TIPS creation. Demographics, procedure details and outcomes were reviewed. Differences between the groups, event rates and patency rates were evaluated using nonparametric two-sample Wilcoxon rank-sum (Mann-Whitney) test, Fisher's exact test, Kaplan-Meier curves, and log-rank test. RESULTS: Technical success was 98%. Sixty-seven percent of subjects had transsplenic and 26% had transhepatic access for TIPS creation. Twenty-one adverse events were noted (48%), four of which were definitely related and four were probably related to the use of gun-sight technique. Early (within 90 days) thrombosis occurred in 7/41 patients (17%), all of whom had existing PVT. CONCLUSION: Gun-sight technique for TIPS creation has a high success rate in this challenging cohort of patients. While complications can occur, most of the adverse events noted were likely associated with TIPS creation itself rather than gun-sight. Early thrombosis only occurred in patients with PVT. Level of Evidence Level 4, Case Series.

CT Texture Analysis and Machine Learning Improve Post-ablation Prognostication in Patients with Adrenal Metastases: A Proof of Concept.

INTRODUCTION: To assess the performance of pre-ablation computed tomography texture features of adrenal metastases to predict post-treatment local progression and survival in patients who underwent ablation using machine learning as a prediction tool. MATERIALS AND METHODS: This is a pilot retrospective study of patients with adrenal metastases undergoing ablation. Clinical variables were collected. Thirty-two texture features were extracted from manually segmented adrenal tumors. A univariate cox proportional hazard model was used for prediction of local progression and survival. A linear support vector machine (SVM) learning technique was applied to the texture features and clinical variables, with leave-one-out cross-validation. Receiver operating characteristic analysis and the area under the curve (AUC) were used to assess performance between using clinical variables only versus clinical variables and texture features. RESULTS: Twenty-one patients (61% male, age 64.1 ± 10.3 years) were included. Mean time to local progression was 29.8 months. Five texture features exhibited association with progression (p < 0.05). The SVM model based on clinical variables alone resulted in an AUC of 0.52, whereas the SVM model that included texture features resulted in an AUC 0.93 (p = 0.01). Mean overall survival was 35 months. Fourteen texture features were associated with survival in the univariate model (p < 0.05). While the trained SVM model based on clinical variables resulted in an AUC of 0.68, the SVM model that included texture features resulted in an AUC of 0.93 (p = 0.024). DISCUSSION: Pre-ablation texture analysis and machine learning improve local tumor progression and survival prediction in patients with adrenal metastases who undergo ablation.

Systemic Air Embolism after Percutaneous Lung Biopsy: A Manageable Complication.

CT-guided percutaneous biopsy is a resourceful and widely used tool to evaluate pulmonary nodules that frequently avoids costly and unnecessary surgeries. Severe complications occur in less than 1% of cases and include gas embolism, which is rarely documented. We report a case of gas embolism after transthoracic biopsies and discuss the pathophysiology and the benefits of early diagnosis and proper management.

Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders.

OBJECTIVES:: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD:: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS:: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION:: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders

OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

Basic evaluation of the immunocompetence of Brazilian patients with Kabuki syndrome

Kabuki syndrome is a genetic disorder of unknown etiology associated with characteristic features (long palpebral fissures, everted lower lids, and arched eyebrows), mental retardation, congenital malformations and increased susceptibility to infections, cancer and autoimmune diseases. However, few studies concerning the immunological aspects of the disease have been performed, and no clear cause of immunodeficiency has been established. Methods: Basic immunological evaluation of nine Brazilian children and adolescents with Kabuki syndrome was performed. Results: Clinical findings include recurrent respiratory infections (mainly otitis media and pneumonia) in 8 patients and allergic respiratory and/ or cutaneous diseases in 5 children. Laboratory findings include selective IgA deficiency, mildly decreased total IgG levels, decreased IgG2 and lack of an adequate response to delayed skin hypersensitivity testing. All of the patients seroconverted after vaccination with heptavalent conjugate pneumococcal vaccine. In two children, clinical and cytogenetic diagnosis of Turner syndrome was also established, while one of them also presented selective IgA deficiency. Conclusion: Due to these findings, we believe that further research is needed for a better understanding of the underlying causes of immunodeficiency in Kabuki syndrome...

A síndrome de Kabuki é uma condição genética de etiologia desconhecida caracterizada por retardo mental, baixa estatura, malformações congênitas, fáscies característico (fissuras palpebrais longas, eversão das pálpebras inferiores e supercílios arqueados) e uma susceptibilidade aumentada a infecções, doenças autoimunes e neoplasias. No entanto, há poucos estudos disponíveis sobre os aspectos imunológicos desta síndrome e até o momento não se estabeleceu causa para essa imunodeficiência. Método: Foi realizada uma avaliação imunológica básica de nove crianças e adolescentes brasileiros com síndrome de Kabuki. Resultados: Os achados clínicos incluíram infecções respiratórias recorrentes (principalmente otite média e pneumonia) em 8 dos pacientes e atopia (dermatite, rinite ou asma) em 5 crianças. Dentre os achados laboratoriais, pode-se citar deficiência seletiva de IgA, níveis reduzidos de IgG, redução de IgG2 e ausência de resposta aos testes cutâneos de hipersensibilidade tardia. Todos os pacientes soroconverteram após imunização com vacina pneumocócica conjugada heptavalente. Dois dos pacientes tiveram ainda o diagnóstico clínico e citogenético de síndrome de Turner, sendo que um deles também apresentava deficiência seletiva de IgA. Conclusão: Devido a estes achados, acreditamos que mais estudos devem ser realizados para que se tenha uma melhor compreensão das causas de imunodeficiência na síndrome de Kabuki...