Anesthetic implications of Möbius syndrome

Gondipalli, Prathima; Tobias, Joseph D.

doi:10.1016/j.jclinane.2005.05.005

Cited by 22 publications

(17 citation statements)

References 36 publications

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“…La mayoría de los pacientes no presentan relación genética, pero se ha documentado casos con transmisión autosómica. Los dos locus implicados se localizan en los cromosomas 3 y 13 (3q21-q22 y 13q12.1-q13, respectivamente) (5,6) .…”

Section: Resultsunclassified

Consideraciones anestésicas en el síndrome de Moebius: a propósito de un caso

Rodríguez-Campoó¹,

Troncoso²,

Catalán³

2020

Revista Mexicana De Anestesiología

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RESUMEN. El síndrome de Moebius es una entidad rara que puede acompañarse de alteraciones que dificulten la anestesia, tales como dificultad de venoclisis, mayor incidencia de depresión respiratoria o de vía aérea difícil. En ocasiones se presenta de forma atípica y puede tener alteraciones del gen RyR con susceptibilidad a hipertermia maligna. Nuestro objetivo en este caso clínico es revisar la preparación necesaria para estos pacientes previa al acto anestésico y quirúrgico, centrándonos en la hipertermia maligna. ABSTRACT. Moebius syndrome is an uncommon illness that can be associated with different alterations related toanesthesia such as difficult peripheral venous catheterization, more respiratory failure incidence or difficult airway. When this syndrome is accompanied by atypical presentation it can be associated with RyR gen alterations and malignant hyperthermia susceptibility. Our objective with this case is to review which is the preparation required in order to a general anesthesia, focusing on malignant hyperthermia. Palabras clave:Síndrome de Moebius, anestesia general, hipertermia maligna.

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Section: Resultsunclassified

Consideraciones anestésicas en el síndrome de Moebius: a propósito de un caso

Rodríguez-Campoó¹,

Troncoso²,

Catalán³

2020

Revista Mexicana De Anestesiología

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“…Moebius syndrome is characterized by congenital palsy of the 6th and 7th cranial nerves, but may also be associated with palsies of other cranial nerves, including the 9th and 10th, creating dysfunction of the pharynx, dysphagia, feeding difficulties, retention of oral secretions and recurrent bouts of aspiration pneumonia [8][9][10][11]25], and can also be associated with central alveolar hypoventilation. Postoperative use of opioid, sedative and anesthetic agents in th presence of central hypoventilation can predispose to grave respiratory consequences.…”

Section: Discussionmentioning

confidence: 99%

“…Postoperative use of opioid, sedative and anesthetic agents in th presence of central hypoventilation can predispose to grave respiratory consequences. In addition, postoperative pain assessment is difficult due to absence of facial expression because of the facial palsy [9,10,25,26].…”

Section: Discussionmentioning

confidence: 99%

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Airway Management in Neonates with Pierre Robin Syndrome along with Mobius Syndrome and Platoglossal Fusion

Elmatite¹,

Haykal²,

Upadhyay³

et al. 2017

J Anesth Clin Res

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Pierre Robin Syndrome is a rare congenital syndrome presenting challenges of airway management not only during anesthesia, but also in the Neonatal Intensive Care Unit (NICU). We present a case of Pierre Robin Syndrome which was unusual because it had Mobius Syndrome and palatoglossal fusion as comorbidies, further complicating airway management. We successfully intubated the patient using a fiberoptic laryngoscope via the nasal route.

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“…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.

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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.

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Anesthetic implications of Möbius syndrome

Cited by 22 publications

References 36 publications

Consideraciones anestésicas en el síndrome de Moebius: a propósito de un caso

Consideraciones anestésicas en el síndrome de Moebius: a propósito de un caso

Airway Management in Neonates with Pierre Robin Syndrome along with Mobius Syndrome and Platoglossal Fusion

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

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