Résumé
Juvenile myasthenia gravis [JMG] is the rare form of myasthenia gravis presenting in childhood and adolescence. When medical management fails, thymectomy is offered for these patients. Complete resection of the thymus is best achieved through transsternal thymectomy. Anesthetic management of patients with JMG is challenging, particularly in regards to the goals of postoperative pain control, respiratory function, and extubation. We retrospectively reviewed the medical records of 13 patients, ranging in age from 6 to 22 years, who underwent transsternal thymectomy for JMG. Information on patient demographics, characteristics of their disease and treatment, anesthetic management, and postoperative course were collected. All patients had undergone multiple treatment modalities and presented for surgery because of inadequate symptom control with medical management. As expected for a pediatric population, anesthesia induction was age dependent. 40% of the patients underwent an inhalation induction and 60% underwent an intravenous induction. Anesthesia was maintained with a low-dose inhalation agent in all patients, supplemented in 84% of patients with a remifentanil infusion, and in 69% of patients with an epidural infusion. Muscle relaxants were avoided in all patients. With this regimen, 92% of patients could be extubated successfully in the operating room. We found that avoidance of muscle relaxants and use of remifentanil with a low-dose hypnotic agent provided a stable intraoperative course, facilitated rapid emergence, and allowed early extubation in patients with JMG undergoing transsternal thymectomy. Epidural analgesia reduced the need for intra- and postoperative intravenous opioids and did not have an adverse effect on respiratory strength