Anestesia em paciente portadora de síndrome de Mckusick-Kaufman: relato de caso

Hobaika, Adriano Bechara de Souza; Borges, Ziltomar Donizetti de Oliveira; Teixeira, Vera Coelho

doi:10.1590/s0034-70942004000600008

Cited by 1 publication

(1 citation statement)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Currently, dantrolene remains the primary basis for the successful management of MH 16,17. The syndrome recrudescence may occur in up to 20% of cases, mostly within 16 hours of the clinical condition onset 18 . Moebius syndrome is a rare disorder, characterized by unilateral or bilateral facial paralysis and extraocular movements defects, secondary to congenital paralysis of the facial (VII) and abducens (VI) nerves 19,20 . It is followed by other cranial nerve dysfunctions, orofacial abnormalities, musculoskeletal hypotonia, and orthopedic anomalies, including clubfoot 21 .…”

Section: Introductionmentioning

confidence: 99%

Recrudescência fatal de hipertermia maligna em lactente com síndrome de Moebius. Relato de caso

Fernandes¹,

Filho²,

Cezar³

et al. 2013

Rev. Bras. Anestesiol.

View full text Add to dashboard Cite

Background and objectives: 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. Case Report: Male infant, fi rst twin, 7 months old, weighing 6.5 kg and presenting with Moebius syndrome was admitted for clubfoot repair. The patient had MH after exposure to sevofl urane 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. Discussion: 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 refl ect on how we must be attentive to children with musculoskeletal disease and maintain treatment for 48 hours.

show abstract

Section: Introductionmentioning

confidence: 99%