Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants.

Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. Results: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair). Conclusions: Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.

Intubación despierto con videolaringoscopio y traqueostomía percutánea guiada por ecografía en una paciente con cáncer anaplásico de tiroides y vía aérea difícil: reporte de caso / Awake intubation with videolaryngoscopy and ultrasound-guided percutaneous tracheostomy in a patient with anaplastic thyroid cancer and difficult airway: a case report

Abstract Introduction: Difficult airway management is an important cause of morbidity and mortality, and the implementation of new techniques and devices has contributed to the reduction of complication rates. Case presentation: A 62-year-old woman with a large neck mass scheduled for palliative percutaneous tracheostomy in which awake intubation with videolaryngoscopy was performed successfully in the first attempt; additionally, it was necessary to perform percutaneous tracheostomy under ultrasound guidance because of the significant degree of anatomical distortion of her neck. Conclusion: There is little evidence about awake intubation with videolaryngoscope in patients with neck masses, which makes our case interesting. Ultrasonography should be immediately available in difficult airway cases, because it facilitates the identification of structures and helps guide percutaneous access in real time.

Resumen Introducción: La dificultad en el manejo de la vía aérea es una importante causa de morbimortalidad, la implementación de nuevas técnicas y dispositivos han ayudado a reducir las tasas de complicaciones. Presentación del caso: Mujer de 62 años con una gran masa cervical programada para traqueostomía percutánea paliativa en la cual se realizó intubación despierta con videolaringoscopio exitosa en el primer intento, y en quien fue necesario realizar la traqueostomía percutánea con guía ecográfica dada la gran distorsión anatómica del cuello. Conclusión: La literatura es escasa con respecto a la intubación despierto con videolaringoscopio en pacientes con masa cervical, lo cual hace interesante nuestro caso. La ultrasonografía debe ser una herramienta de disponibilidad inmediata en el manejo de pacientes con vía aérea difícil, ya que facilita la identificación de las estructuras para guiar su acceso percutáneo en tiempo real.

Ergonomía en los bloqueos nerviosos guiados por ultrasonografía / Ergonomics in ultrasound-guided nerve blocks

Introduction: Performing ultrasound-guided nerve blocks has proven to be safe, but ergonomic considerations are essential. Methods: A non-systematic literature search was conducted in the Medline/Pubmed and Embase databases. Objective: To describe the influence of operator position, monitor location, and hand position in performing ultrasound-guided nerve blocks. Results: Poor ergonomics is a key factor of error when performing nerve blocks under ultrasound guidance. "In-plane" needle insertion increases success. An inappropriate position may be a cause of muscle-skeletal disorders. Based on these results, we propose specific element location and operator position for the performance of certain nerve blocks. Conclusion:Performing ultrasound-guided nerve blocks requires an ergonomic work area allowing for greater comfort and effectiveness, thus reducing the occurrence of muscle-skeletal disorders.

Introducción: Los bloqueos guiados por ultrasonografía han demostrado ser eficaces, pero requieren considerar aspectos como la ergonomía. Objetivo: Describir influencia de la postura del operador, ubicación del monitor y posición de las manos, al realizar bloqueos con ultrasonografía. Métodos: Se realizó una búsqueda no sistemática de literatura en Medline/Pubmed y Embase. Resultados: Una mala ergonomía es un factor de error al realizar bloqueos con ultrasonido. La inserción de la aguja "en plano" aumenta el éxito. Una posición inapropiada puede producir trastornosmusculo-esqueléticos en el operador. Basados en esto, se propone una ubicación de elementos y posición del operador para realizar algunos bloqueos. Conclusión: Realizar bloqueos con ultrasonido requiere un área de trabajo ergonómica para lograr mayor eficacia, y disminuir aparición de trastornos músculo-esqueléticos.

High doses of 4-aminopyridine improve functionality in chronic complete spinal cord injury patients with MRI evidence of cord continuity.

BACKGROUND AND AIMS: Many patients with complete spinal cord injury (SCI) exhibit demyelinated and poorly myelinated nerve fibers traversing the lesion site. Conventional doses of 4-aminopyridine (4-AP, 30 mg/day) have shown to provide no or minor functional improvement in these patients. We undertook this study to test the functional effect of high doses of 4-AP on patients with chronic complete SCI with cord continuity at the site of injury demonstrated by magnetic resonance imaging. METHODS: Fourteen patients were included in a double-blind, randomized, placebo-controlled trial followed by an open label long-term follow-up. Initially, patients received 4-AP or placebo orally, with 4-AP being increased gradually (5 mg/week) to reach 30 mg/day. For long-term treatment, 4-AP was increased 10 mg periodically according to negative electroencephalogram and blood test abnormalities and minor adverse reactions. Pre-treatment, 12 and 24 weeks of the controlled trial, and 6 and 12 months of open trial evaluations, or with the highest doses reached were obtained. RESULTS: Three of 12 patients were able to walk with the assistance of orthopedic devices, 1/12 became incomplete (AIS B), 7/12 improved their somatosensory evoked potentials, 5/12 had sensation and control of bladder and anal sphincters, and 4/9 male patients had psychogenic erection. CONCLUSIONS: Positive changes were seen mainly in patients with cyst (4/5) or atrophy (3/5) of the injury site. Two patients withdrew from the study: one had seizures and one had intolerant adverse reactions. We conclude that high doses of 4-AP in the studied population produced several functional benefits not observed using lower doses.

Efficacy and safety of 4-aminopyridine in patients with long-term spinal cord injury: a randomized, double-blind, placebo-controlled trial.

OBJECTIVES: To study the efficacy and safety of 4-aminopyridine (4-AP), and to document sensorimotor changes after discontinuation of the drug in patients with long-term spinal cord injury. DESIGN: Randomized, double-blind, placebo-controlled trial. SETTING: Clinical research unit. PATIENTS: Twenty-seven patients with long-term spinal cord injury. INTERVENTION: Patients were randomized to receive either oral 4-AP 5 mg/day, which was increased by 5 mg/week to a maximum dosage of 30 mg/day, or placebo for 12 weeks. They switched to the opposite treatment for the next 12 weeks. MEASUREMENTS AND MAIN RESULTS: Twenty-five patients finished the study. The results from the first 12 weeks were used to test efficacy. Positive gains in motor function, sensation, and independence occurred more frequently in patients receiving 4-AP (69%) than those receiving placebo (46%). Significant functional improvement was also noted in those treated with 4-AP (chi2, p=0.042). When each evaluation scale was considered separately, significant improvement was seen only in motor function (4-AP 92% vs placebo 46%, Fisher exact test, p=0.03). Persistent effects of the drug were assessed at week 24 in the group that initially received 4-AP. A persistent, significant 4-AP effect was observed in evaluations of sensation and independence (67% and 83% of patients, respectively; Wilcoxon signed rank test, p=0.032 and 0.042, respectively). Fourteen (56%) patients had 26 adverse reactions. One moderate adverse reaction--posterior tibial artery vasospasm--and 25 mild adverse reactions, such as dry mouth, dizziness, nausea, gastritis, oral and peripheral paresthesia, resolved adequately. Six (24%) patients experienced transitory alterations of enzyme levels (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, and creatine kinase) and thrombocytopenia. CONCLUSION: Patients who received 4-AP showed significant improvement in motor function, and a persistent effect on sensation and independent function occurred. The drug is safe; however, after starting 4-AP therapy, patients must be carefully monitored for the possible occurrence of peripheral vasospasm.