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Obstetric and Anesthetic Management of Severe Congenital Myasthenia Syndrome
journal contribution
posted on 2023-05-17, 07:21 authored by Nicolaas TerblancheNicolaas Terblanche, Maxwell, C, Keunen, J, Carvalho, JCAThe 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. © 2008 International Anesthesia Research Society.
History
Publication title
Anesthesia and AnalgesiaVolume
107Issue
4Pagination
1313-1315ISSN
0003-2999Department/School
Menzies Institute for Medical ResearchPublisher
Lippincott Williams & WilkinsPlace of publication
530 Walnut St, Philadelphia, USA, Pa, 19106-3621Rights statement
Copyright 2008 International Anesthesia Research SocietyRepository Status
- Restricted