Obstetric and anesthetic management of severe congenital myasthenia syndrome.

Abstract

The congenital myasthenia syndromes form a heterogeneous group of genetic diseases characterized by defective neuromuscular transmission. Although they have muscle fatigability in common with the acquired immune myasthenia syndrome, there are important pathophysiological, diagnostic, management and progression pattern differences between them. We report the management of a 28-yr-old patient with longstanding congenital myasthenia syndromes, who underwent an elective cesarean delivery under spinal anesthesia. Muscle imbalance plus weakness-related scoliosis and chronic respiratory failure complicated her management. Ultrasonography was used to facilitate the spinal anesthetic. Intraoperative noninvasive positive pressure ventilation maintained lung volumes effectively and prevented deterioration in respiratory function.