Airway management in newborn with Klippel-Feil syndrome

Braz J Anesthesiol. Sep-Oct 2016;66(5):551-3. doi: 10.1016/j.bjane.2014.03.006. Epub 2014 Apr 29.


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.

Keywords: Anormalidadeas congênitas; Cirurgia geral; Congenital anomalies; Congenital heart disease; Critical care; Cuidados intensivos; Difficult airway; Doença cardíaca congênita; Neonatal; Surgery; Vias aéreas difícil.

Publication types

  • Case Reports

MeSH terms

  • Airway Management / methods*
  • Fatal Outcome
  • Female
  • Humans
  • Infant, Newborn
  • Intestinal Obstruction / etiology
  • Intestinal Obstruction / surgery
  • Klippel-Feil Syndrome / surgery
  • Klippel-Feil Syndrome / therapy*