Korean J Anesthesiol.  2016 Oct;69(5):523-526. 10.4097/kjae.2016.69.5.523.

Bronchoscopic concerns in Proteus syndrome: a case report

Affiliations
  • 1Department of Anesthesia and Pain Medicine, Pusan National University Hospital, Busan, Korea. lhjksk@pusan.ac.kr
  • 2Medical Research Institute, Pusan National University School of Medicine, Busan, Korea.

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.

Keyword

Difficult intubation; Hamartomatous disorder; Proteus syndrome

MeSH Terms

Anesthesia
Anesthesia, General
Bronchoscopy
Child
Ear
Facial Asymmetry
Female
Humans
Intubation
Neck
Proteus Syndrome*
Proteus*
Scoliosis
Tooth
Torticollis
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