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Bronchoscopic concerns in Proteus syndrome: a case report

Proteus syndrome (PS) is a rare congenital hamartomatous disorder with multisystem involvement. PS shows highly clinical variability due to overgrowth of the affected areas, and several features can make anesthetic management challenging. Little is known about the airway problem associated with anes...

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Detalles Bibliográficos
Autores principales: Hong, Jung-Min, Lee, Hyeon Jeong, Kim, Eun Soo, Kim, Hae-Kyu, Jeon, Soeun, Kim, Hyae-Jin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Society of Anesthesiologists 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5047991/
https://www.ncbi.nlm.nih.gov/pubmed/27703636
http://dx.doi.org/10.4097/kjae.2016.69.5.523
Descripción
Sumario:Proteus syndrome (PS) is a rare congenital hamartomatous disorder with multisystem involvement. PS shows highly clinical variability due to overgrowth of the affected areas, and several features can make anesthetic management challenging. Little is known about the airway problem associated with anesthesia in PS patients. An 11-year-old girl with PS was scheduled for ear surgery under general anesthesia. She had features complicating intubation including facial asymmetry and disproportion, abnormal teeth, limitation of neck movement due to torticollis, and thoracolumbar scoliosis. This study reports on a case of deformed airway of a PS patient under fiberoptic bronchoscopy.