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General Anesthesia in Schwartz-Jampel Syndrome: A Case Report

INTRODUCTION: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disease characterized by muscle weakness and stiffness, abnormal bone development, short stature, joint contractures, and facial dysmorphisms. Myopathy, anatomical deformities, and malignant hyperthermia are challenging for a...

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Detalles Bibliográficos
Autores principales: Derakhshan, Pooya, Habibi, Azadeh, Amniati, Saied
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Brieflands 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10664156/
https://www.ncbi.nlm.nih.gov/pubmed/38021333
http://dx.doi.org/10.5812/aapm-129305
Descripción
Sumario:INTRODUCTION: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disease characterized by muscle weakness and stiffness, abnormal bone development, short stature, joint contractures, and facial dysmorphisms. Myopathy, anatomical deformities, and malignant hyperthermia are challenging for anesthesiologists. CASE PRESENTATION: This case report describes one case of SJS. The female patient was scheduled for Blepharoplasty in Rasuol Akram General Hospital. CONCLUSIONS: These patients may have difficult intubation and be prone to malignant hyperthermia. We managed this patient by applying Rocuronium, propofol, and C-MAC video laryngoscopy.