Several challenges associated with the anesthetic management of Emery-Dreifuss muscular dystrophy patients: case report

Abstract Emery-Dreifuss Muscular Dystrophy is a very rare type of muscular dystrophy, associated with contractures, atrophy, and muscle weakness, besides cardiomyopathy with severe arrhythmias. Published studies focusing on this disorder are scarce. We describe the anesthetic management of a male patient with Emery-Dreifuss Muscular Dystrophy, to be submitted to umbilical and inguinal hernioplasty and hydrocele repair under epidural anesthesia. The anesthesia approach enabled us to circumvent the patient's susceptibility to malignant hyperthermia and his potentially difficult airway, in addition to maintaining hemodynamic stability. The day after surgery the patient resumed walking, and two days later he was discharged from the hospital.

Several challenges associated with the anesthetic management of Emery-Dreifuss muscular dystrophy patients: case report.

Emery-Dreifuss Muscular Dystrophy is a very rare type of muscular dystrophy, associated with contractures, atrophy, and muscle weakness, besides cardiomyopathy with severe arrhythmias. Published studies focusing on this disorder are scarce. We describe the anesthetic management of a male patient with Emery-Dreifuss Muscular Dystrophy, to be submitted to umbilical and inguinal hernioplasty and hydrocele repair under epidural anesthesia. The anesthesia approach enabled us to circumvent the patient...s susceptibility to malignant hyperthermia and his potentially difficult airway, in addition to maintaining hemodynamic stability. The day after surgery the patient resumed walking, and two days later he was discharged from the hospital.

General anesthesia for emergency cesarean delivery: simulation-based evaluation of residents.

INTRODUCTION: Decreased rates of General Anesthesia (GA) for Cesarean Section (C-section) create a learning problem for anesthesia trainees. In this context, training the management of GA for C-section using simulation techniques allows a safe environment for exposure, learning, performance improvement, and capability retention. OBJECTIVE: Analyze anesthesia residents' performance regarding a simulated clinical case of GA for emergency C-section and identify specific deficits in skill acquisition. METHODS: Between 2015 and 2018, we evaluated the performance of 25 anesthesiology residents challenged by a simulated clinical case of GA for emergency C-section after the conclusion of the obstetric anesthesia rotation. Each resident performed the clinical case once followed by the assessment of their performance. Final scores were given according to the completion rate of 14-tasks, going from 0% to 100%. Two study groups were considered according to residency year for subsequent comparison of results (Group 1, second and third residency years and Group 2, fourth and fifth residency years). RESULTS AND DISCUSSION: Mean score was 64.29% ± 13.62. Comparatively, Group 1 obtained a higher score than Group 2 (70.63% ± 14.02 vs. 60.27% ± 11.94), although with no statistically significant difference (p = 0.063). The tasks most frequently accomplished were opioid administration (100%), rapid sequence technique (100%), pre-oxygenation (92%), gastric content aspiration prophylaxis (84%), and previous clinical history (84%). Conversely, the tasks less frequently accomplished were confirming presence of pediatrician (64%), oxytocin administration (56%), PONV prophylaxis (56%), and preoperative airway assessment (48%). CONCLUSION: The performance of the residents observed in this study was comparable to results previously published. The final score did not depend on the residency year.

[The importance of simulation in team training on obstetric emergencies: results of the first phase of the national plan for continuous medical training]. / Importância da Simulação no Treino de Equipa para Eventos Críticos em Obstetrícia: Resultados da Primeira Fase do Plano Nacional de Educação Médica Contínua.

Obstetric emergencies are unexpected and random. The traditional model for medical training of these acute events has included lectures combined with sporadic clinical experiences, but this educational method has inherent limitations. Given the variety of manual skills that must be learned and high-risk environment, Obstetrics is uniquely suited for simulation. New technological educational tools provide an opportunity to learn and master technical skills needed in emergent situations as well as the opportunity to rehearse and learn from mistakes without risks to patients. The goals of this study are to assess which are the factors that trainees associate to human fallibility before and after clinical simulation based training; to compare the confidence level to solve emergent obstetric situations between interns and experts with up to 5 years of experience before and after training, and to determine the value that trainees give to simulation as a teaching tool on emergent events. 31 physicians participated at this course sessions. After the course, we verified changes in the factores that trainees associate to human fallibility, an increase in confidence level to solve emergent obstetric and an increase in the value that trainees give to simulation as a teaching tool.