Anesthesia in a newborn with Klippel-Feil syndrome

I read the case report of Altay et al.¹ about anesthesia management of a newborn with Klippel-Feil syndrome (KFS) with interest. The authors presented their case as “the youngest child with KFS on whom oral intubation was performed”. I appreciate the colleagues for their management of this challenging case, but there are some points that have to be discussed.

Altay et al. performed a successful intubation at first attempt with Direct Laryngoscopy (DL), which was consistent with the literature. According to the literature, KFS alone may not be a predictor of difficult airway management in infants. Naguib et al.² had reported a three-week-old boy diagnosed with KFS successfully intubated with DL. Creighton et al.³ had reported 8 infants with KFS (6 of them had also cleft palate, most probably some of them were newborns) on whom oral or nasal intubation was performed with DL using regular laryngoscope. They performed awake DL successfully, despite the other present conditions that complicate intubation like cleft palate and lateral position in addition to KFS.

Recently we have reviewed the airway management and the success of DL in children with KFS⁴ and found that there is no report describing difficult mask ventilation or unsuccessful Laryngeal Mask Airway (LMA) insertion in the literature. Also, there is no report of an unsuccessful DL in infants with KFS. We think that the success rate of tracheal intubation with DL in early ages (probably before adolescence) seems to be increased when other predictors of difficult intubation does not accompany. These findings may encourage us for attempting DL in children with KFS alone, but accompanying airway anomalies are not rare in KFS and have to be investigated before anesthesia induction. Also, a previous successful DL does not ensure successful intubation because cervical fusion may become progressively worsen over time and DL may be challenging in older ages.

Another point: the authors mostly dwell on the airway management of the patient, but the anesthesia technique might be questionable. As, providing an adequate depth of anesthesia to ablate the rise in pulmonary vascular resistance associated with surgical stimuli is one of the primary goals in anesthesia management of these patients, what was the reason to use sevoflurane as a sole anesthetic agent in a cardiac patient with persistent pulmonary hypertension, if early postoperative extubation was not planned?

References

• ¹
  Altay N, Yuce HH, Aydogan H, et al. Airway management in newborn with Klippel-Feil syndrome. Braz J Anesthesiol. 2016;66:551-3.
• ²
  Naguib M, Farag H, Ibrahim Ael-W. Anaesthetic considerations in Klippel-Feil syndrome. Can Anaesth Soc J. 1986;33:66-70.
• ³
  Creighton RE, Relton JE, Meridy HW. Anesthesia for occipital encephalocoele. Can Anaesth Soc J. 1974;21:403-6.
• ⁴
  Bakan M, Umutoglu T, Zengin SU, et al. The success of direct laryngoscopy in children with Klippel-Feil syndrome. Minerva Anestesiol. 2015;81:1384-6.

Publication Dates

• Publication in this collection
  Nov-Dec 2017