Airway management in newborn with Klippel-Feil syndrome / Manejo de vias aéreas em recém-nascido com síndrome de Klippel-Feil

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, 3300 g 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 2 min. Her Cornmack Lehane score was 2 and oral intubation was performed with 3.5 mm ID non-cuffed endotracheal tube in first attempt. Operation lasted for 45 min. 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.

Resumo A síndrome de Klippel-Feil (SKF) envolve uma tríade clássica que inclui pescoço curto, linha de implantação dos cabelos baixa e restrição do movimento do pescoço e é uma das causas congênitas de via aérea difícil. Apresentamos o caso de uma recém-nascida, de 26 dias, 3.300 g de peso, com SKF, que foi agendada para cirurgia de correção de uma obstrução intestinal. A paciente apresentava características acentuadas da SKF. A anestesia foi induzida com inalação de sevoflurano a 2-3% em 100% de oxigênio. A inalação de sevoflurano foi interrompida após dois minutos. O escore de Cormack-Lehane da paciente era 2 e a intubação orotraqueal foi feita na primeira tentativa, com tubo endotraqueal de 3,5 mm ID sem balão. O tempo de cirurgia foi de 45 minutos. Após a cirurgia sem intercorrências, a paciente foi transferida para a unidade de reanimação neonatal ainda intubada. No terceiro dia de pós-operatório, foi a óbito por causa de uma insuficiência cardíaca hiperdinâmica. Esse caso apresenta a criança mais jovem com síndrome de Klippel-Feil na literatura e na qual a intubação orotraqueal foi feita. Acreditamos também que o posicionamento de pacientes dessa faixa etária mais jovem pode ser mais fácil do que o de pacientes de faixas etárias mais altas, por causa do processo de ossificação incompleto.

Airway management in newborn with Klippel-Feil syndrome.

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.