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.
Subject(s)
Child , Female , Humans , Anesthesia , Anesthesia, General , Bronchoscopy , Ear , Facial Asymmetry , Intubation , Neck , Proteus Syndrome , Proteus , Scoliosis , Tooth , TorticollisABSTRACT
Proteus syndrome is a rare condition that can be loosely categorized as a hamartomatous disorder. It is a complex disorder with multisystem involvement and great clinical variability. At this time, it is unknown how many individuals have Proteus syndrome, since not all cases of Proteus syndrome are documented. We do not know whether anesthetic problems in Proteus syndrome are common or not, but as there is a high incidence of cervical spine involvement, we anticipate significant anesthetic problems. We experienced such a case under general anesthesia for liposuction to lipomatosis in the posterior neck and right upper back, in a 18-year-old boy with Proteus syndrome. We report this case of Proteus syndrome and review the literature on this rare disease.