Management of anesthesia in a pediatric patient with myotonic dystrophy type 1. Case report

  • Lleimi Alarcón-Pérez Department of Anesthesiology and Resuscitation, Hospital General Universitario Gregorio Marañón, Madrid, Spain
  • Cristina Pastor-Laín Department of Anesthesiology and Resuscitation, Hospital General Universitario Gregorio Marañón, Madrid, Spain
  • Lourdes Barragán-González Department of Anesthesiology and Resuscitation, Hospital General Universitario Gregorio Marañón, Madrid, Spain
  • Claudia Sarrais-Polo Department of Anesthesiology and Resuscitation, Hospital Clínico San Carlos, Madrid, Spain
  • María T. López-Gil Department of Anesthesiology and Resuscitation, Hospital General Universitario Gregorio Marañón, Madrid, Spain
  • Ignacio Mantilla-Martínez Department of Anesthesiology and Resuscitation, Hospital General Universitario Gregorio Marañón, Madrid, Spain
Keywords: Myotonic Dystrophy; Muscle; Skeletal; Child; Anesthesia; Muscle Hypotonia

Abstract

Myotonic dystrophy is a disease affecting the muscle fibers with loss of muscle mass. The principal characteristic of the disease is myotony or slow muscle relaxation following muscle contraction that is further aggravated as a result of stress, pain, cold, or by the administration of succinylcholine. Similar to other muscle pathologies, myotonic dystrophy is considered a multisystem disorder, usually with cardiac and respiratory involvement, a fact to be kept in mind when planning anesthesia. Moreover, there is a potential association with malignant hyperthermia or rhabdomyolysis associated with some muscle diseases. The case herein discussed is an example of the management of anesthesia in this group of patients to avoid the potential triggers of a myotonic crisis.

References

1. Ho G, Cardamone M, Farrar M. Congenital and childhood myotonic dystrophy: current aspects of disease and future directions. World J Clin Pediatr 2015;4:66-80.

2. Veyckemans F, Scholtes JL. Myotonic dystrophies type 1 and 2: anesthetic care. Pediatr Anesth 2013;23:794-803.

3. Pérez Ferrer A, Calvo Vecino JM. El niño con patologías muscu-loesqueléticas. En: Manual de Anestesiología pediátrica. Bogotá: Editorial Médica Panamericana; 2016.
How to Cite
1.
Alarcón-Pérez L, Pastor-Laín C, Barragán-González L, Sarrais-Polo C, López-Gil MT, Mantilla-Martínez I. Management of anesthesia in a pediatric patient with myotonic dystrophy type 1. Case report. Colomb. J. Anesthesiol. [Internet]. 2018 Jan. 1 [cited 2024 Jun. 15];46(1):72-4. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/338

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Published
2018-01-01
How to Cite
1.
Alarcón-Pérez L, Pastor-Laín C, Barragán-González L, Sarrais-Polo C, López-Gil MT, Mantilla-Martínez I. Management of anesthesia in a pediatric patient with myotonic dystrophy type 1. Case report. Colomb. J. Anesthesiol. [Internet]. 2018 Jan. 1 [cited 2024 Jun. 15];46(1):72-4. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/338
Section
Case Report / Case Series

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