title: Fatal recrudescence of malignant hyperthermia in an infant with Moebius syndrome.
abstract: OBJECTIVE
  Malignant hyperthermia (MH) is a pharmacogenetic skeletal muscle disorder characterized by a hypermetabolic state after anesthesia with succinylcholine and/ or volatile anesthetics. Various neuromuscular syndromes are associated with susceptibility; however, Moebius syndrome has not been reported. Dantrolene is the drug of choice for treatment. Recurrence may occur in up to 20% of cases after the initial event treatment.
  
  
  METHODS
  Male infant, fi rst twin, 7 months old, weighing 6.5kg and presenting with Moebius syndrome was admitted for clubfoot repair. The patient had MH after exposure to sevoflurane and succinylcholine, which was readily reversed with dantrolene maintained for 24 hours. Ten hours after dantrolene discontinuation, there was recrudescence of MH that did not respond satisfactorily to treatment, and the patient died.
  
  
  CONCLUSIONS
  Musculoskeletal disorders in children are associated with increased risk of developing MH, although Moebius syndrome has not yet been reported. Dantrolene is the drug of choice for treating this syndrome; prophylaxis is indicated during the fi rst 24-48 hours of the episode onset. The main risk factors for recurrence are muscular type, long latency after anesthetic exposure, and increased temperature. The child had only one risk factor. This case leads us to reflect on how we must be attentive to children with musculoskeletal disease and maintain treatment for 48 hours.
authors.affiliation: Hospital Universitário Walter Cantídio, UFC, Fortaleza, Ceará, Brazil. clauregifer@gmail.com
authors.forename: Cláudia Regina
authors.initials: CR
authors.lastname: Fernandes
authors.forename: Washington Aspiculeta
authors.initials: WA
authors.lastname: Pinto Filho
authors.forename: Lia Cavalcante
authors.initials: LC
authors.lastname: Cezar
authors.forename: Josenília Maria
authors.initials: JM
authors.lastname: Alves Gomes
authors.forename: Glauco Kleming
authors.initials: GK
authors.lastname: Florencio da Cunha
mesh_terms: D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D008305:Malignant Hyperthermia / Q000209:etiology*; D020331:Mobius Syndrome / Q000150:complications*