Airway management in newborn with Klippel–Feil syndrome

Abstract

Klippel-Feil syndrome (KFS) has a classical triad that includes short neck, low hair line and restriction in neck motion and is among one of the congenital causes of difficult airway. Herein, we present a 26-day, 3300g newborn with KFS who was planned to be operated for correction of an intestinal obstruction. She had features of severe KFS. Anesthesia was induced by inhalation of sevoflurane 2-3% in percentage 100 oxygen. Sevoflurane inhalation was stopped after 2min. Her Cornmack Lehane score was 2 and oral intubation was performed with 3.5mm ID non-cuffed endotracheal tube in first attempt. Operation lasted for 45min. Following uneventful surgery, she was not extubated and was transferred to the newborn reanimation unit. On the postoperative third day, the patient died due to hyperdynamic heart failure. This case is the youngest child with Klippel-Feil syndrome in literature and on whom oral intubation was performed. We also think that positioning of this younger age group might be easier than older age groups due to incomplete ossification process.

Otolaryngologic Manifestations of Klippel-Feil Syndrome in Children

“…Several case reports with respect to difficult intubations in patients with KFS exist in the otolaryngology literature . A useful strategy, as used in one of our patients with a grade 4 view on direct laryngoscopy, is to perform intubation with an endotracheal tube loaded on a flexible bronchoscope advanced through an laryngeal mask airway .…”

“…Several case reports with respect to difficult intubations in patients with KFS exist in the otolaryngology literature. [13][14][15][38][39][40][41][42][43] A useful strategy, as used in one of our patients with a grade 4 view on direct laryngoscopy, is to perform intubation with an endotracheal tube loaded on a flexible bronchoscope advanced through an laryngeal mask airway. 14,39,40,42 Under such circumstances, it is imperative to maintain spontaneous ventilation, as even this intubation technique may not prove successful.…”

Klippel–Feil syndrome: Interchange of Plan A and B for airway management in the same patient under different circumstances

“…Klippel–Feil syndrome, characterised by the triad of short neck, decreased cervical spine mobility and low posterior hairline, poses a challenge to the anaesthetist during airway management. [ 1 2 ] We describe the airway management in a patient with Klippel–Feil syndrome which required the interchange of airway management plan in two different surgical contexts.…”