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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Published inKorean journal of anesthesiology Vol. 69; no. 5; pp. 523 - 526
Main Authors Hong, Jung-Min, Lee, Hyeon Jeong, Kim, Eun Soo, Kim, Hae-Kyu, Jeon, Soeun, Kim, Hyae-Jin
Format Journal Article
LanguageEnglish
Published Korea (South) The Korean Society of Anesthesiologists 01.10.2016
Korean Society of Anesthesiologists
대한마취통증의학회
Subjects
Case Report
difficult intubation
hamartomatous disorder
proteus syndrome
마취과학
Hamartomatous disorder
Proteus syndrome
Difficult intubation
Online AccessGet full text
ISSN2005-6419
2005-7563
DOI10.4097/kjae.2016.69.5.523

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Abstract 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.
AbstractList 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.
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.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.
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. KCI Citation Count: 0
Author Hong, Jung-Min
Kim, Hae-Kyu
Jeon, Soeun
Kim, Hyae-Jin
Kim, Eun Soo
Lee, Hyeon Jeong
AuthorAffiliation 2 Medical Research Institute, Pusan National University School of Medicine, Busan, Korea
1 Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea
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Issue 5
Keywords Hamartomatous disorder
Proteus syndrome
Difficult intubation
Language English
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SubjectTerms Case Report
difficult intubation
hamartomatous disorder
proteus syndrome
마취과학
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Title Bronchoscopic concerns in Proteus syndrome: a case report
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