A rare case of difficult airway management in a Klippel-Feil syndrome pediatric patient with osseous torticollis undergone orthopedic surgery

Background Orthopedic surgery for cervical torticollis poses potential threat to airway management both in tracheal intubation and extubation. Klippel-Feil syndrome (KFS) is a complex syndrome of osseous and visceral anomalies. The anatomical characteristics of KFS might have significant implications for airway management. Case presentation This is a rare case of an 8-year-old boy presenting with osseous torticollis, congenital occipito-atlantal deformity, congenital basilar invagination and KFS undergone elective torticollis correction surgery. Though with difficulty, tracheal intubation was successfully performed. Extubation failed twice on postoperative day 2 and 10, and required tracheostomy. Based on radiological findings, we speculated that prolonged airway edema accounted for the main reason of the failed extubation, the hypertrophic tonsil and occipito-cervical fusion resulted in reduced oropharyngeal space and limited cervical range of motion. Moreover, the Chiari malformation and KFS complicated the airway condition and lead to prolonged airway obstruction. The tracheostomy casing was removed 1 month later. Conclusions Cautions should be taken in extubation of pediatric patients undergone major osseous torticollis surgery. Reintubation should be prepared in case of failed extubation. Severe post-operative airway edema, complicated with hypertrophic tonsil, the structural abnormalities in the oropharyngeal cavity, and occipito-cervical deformities constituted the decreased oropharyngeal space and resulted in failed extubation. For severe airway compromise and prolonged intubation, tracheostomy should be considered. Keywords: Difficult extubation, Osseous torticollis, Airway edema, Tonsil hypertrophy, Occipito-cervical fixation, Pediatric, Syndrome