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

Cláudia Regina Fernandes; Washington Aspiculeta Pinto Filho; Lia Cavalcante Cezar; Josenília Maria Alves Gomes; Glauco Kleming Florencio da Cunha

doi:10.1590/S0034-70942013000300012

Clinical Information

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

Fatal recrudescence of malignant hyperthermia in an infant with Moebius syndrome

Cláudia Regina Fernandes; Washington Aspiculeta Pinto Filho; Lia Cavalcante Cezar; Josenília Maria Alves Gomes; Glauco Kleming Florencio da Cunha

http://dx.doi.org/10.1590/S0034-70942013000300012 Braz J Anesthesiol, vol.63, n3, p.296-300, 2013

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Resumo

JUSTIFICATIVA E OBJETIVOS: A hipertermia maligna (HM) é uma desordem farmacogenética da musculatura esquelética, caracterizada por estado hipermetabólico após anestesia com succinilcolina e/ou agentes anestésicos voláteis. Várias síndromes neuromusculares estão associadas com susceptibilidade, no entanto a síndrome de Moebius não é descrita. O dantrolene é o fármaco de escolha para o tratamento. Recrudescência pode ocorrer em até 20% dos casos após o tratamento do evento inicial. RELATO DO CASO: Lactente, masculino, primeiro gemelar, sete meses, 6,5 kg. Portador da síndrome de Moebius. Internado para correção de pé torto congênito. Apresentou HM após exposição à sevoflurano e succinilcolina, prontamente revertida com dantrolene, sendo o fármaco mantido por 24 horas. Dez horas após a interrupção do dantrolene, houve recrudescência da HM, a qual não respondeu satisfatoriamente ao tratamento, evoluindo para óbito. DISCUSSÃO: Doenças musculoesqueléticas em crianças estão associadas a aumento de risco para desenvolvimento de MH, embora a síndrome de Moebius ainda não tenha sido descrita. O dantrolene é fármaco de eleição para o tratamento da síndrome, está indicada profilaxia durante as primeiras 24-48 horas do episódio inicial. Os principais fatores associados à recrudescência são: tipo muscular, longa latência após exposição anestésica e aumento da temperatura. A criança tinha apenas um fator de risco. Este caso nos remete à reflexão de que devemos estar atentos a crianças com doença musculoesquelético e que devemos manter o tratamento durante 48 horas.

Palavras-chave

Hipertermia Maligna, ANESTÉSICOS, Volátil, sevoflurano, BOQUEADOR MUSCULAR, Succinilcolina, Síndrome de Möbius

Abstract

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, first 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 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. 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 first 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.

Keywords

Malignant Hyperthermia, Anesthesia, Inhalation, Succinylcholine, Mobius Syndrome

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

Fatal recrudescence of malignant hyperthermia in an infant with Moebius syndrome

Cláudia Regina Fernandes; Washington Aspiculeta Pinto Filho; Lia Cavalcante Cezar; Josenília Maria Alves Gomes; Glauco Kleming Florencio da Cunha

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