Sedation with dexmedetomidine on Klippel Feil Syndrome infant patient

Dear Editor,

Klippel Feil Syndrome (KFS) is a congenital malformation which has a failure of segmantation of cervical somites in the 8th weeks of gestation and fusion at least two cervical segments.¹ It is described as the triad of short neck, low posterior hairline and decreased range of neck motions.² Especially decreased range of neck motions lead many anesthesiologists to difficult airway management. Dexmedetomidine is used for sedation especially without respiratory depression and for use in very early age group pediatric patients was not observed in reported cases.³ In this case; we aimed to present successful sedation by dexmedetomidine to infant patient who is 65 days aged, diagnosed KFS, limited neck motions and known difficult airway.

Infant patient, who is 65 days aged, 4 kg weight, 81 cm height and diagnosed KFS, was scheduled for a neck MRI. On evaluation of patient; short neck, low posterior hairline, thorax deformity and floating finger was found. The mouth opening of patient was normal but the extension of neck was particularly restricted. Systemic examination revealed no other abnormalities. The chest X-ray showed hypoplastic ribs. In medical history of patient; there was intermittent respiratory distress. The patient was taken to the anesthetic preparation room. Standard monitorization (electrocardiography, heart rate, oxygen saturation) were performed and intravenous (IV) line was obtained. After preoxygenation for 5 min by facemask, dexmedetomidine infusion was administered by IV route as bolus of 0.5 mcg.kg^-1 dose for 10 min. Monitorization data and Ramsey Sedation Score of the patient were recorded at intervals of 5 min. Ear plugs was inserted into patient for protection of loud sounds in MRI room. After bolus administration, dexmedetomidine infusion was continued for maintenance dose as 0.6 mg.kg^-1.h^-1. Dexmedetomidine infusion was continued for 20 min throughout the procedure. Mean values of monitoring data of the patient were recorded as peripheral oxygen saturation: 98% (range 96-99), heart rate: 138 beats/min (range 130-171), respiratory rate: 26/min (range 23-30) (Fig. 1). During the process, Ramsey Sedation Score was found 4. Dexmedetomidine infusion was terminated at the end of operation and patient follow-up was continued in the postanesthesia care unit. Patient was sent to service after the full awakeness and the patient was discharged on the same day.

Figure 1
Monitorization data.

There was no need an application such as mask ventilation, laryngoscopy, intubation and laryngeal mask in this case with difficult airway. It has been reported the use of dexmedetomidine has provide adequate sedation without respiratory depression.⁴

Because of these beneficial effects, we approved the administration of dexmedetomidine to 65 days aged infant and diagnosed KFS patient. Sedation is important in such cases which is considered limited neck motions and difficult airway. We believe that dexmedetomidine is an agent may be preferred an alternative agent ensuring respiratory control in anesthesia procedures for sedation.

References

• ¹
  Manivel S, Prasad R, Jacob R. Anesthetic management of a child with Klippel-Feil syndrome in the radiology suite. Paediatr Anaesth. 2005;15:171-2.
• ²
  Cakmakkaya OS, Kaya G, Altintas F, et al. Anesthetic management of a child with Arnold-Chiari malformation and Klippel-Feil syndrome. Paediatr Anaesth. 2006;16:355-6.
• ³
  Subramanyam R, Cudilo EM, Hossain MM, et al. To pretreat or not to pretreat: prophylactic anticholinergic administration before dexmedetomidine in pediatric imaging. Anesth Analg. 2015;121:479-85.
• ⁴
  Shah TH, Badve MS, Olajide KO, et al. Dexmedetomidine for an awake fiber-optic intubation of a parturient with Klippel-Feil syndrome. Type I Arnold Chiari malformation and status post released tethered spinal cord presenting for repeat cesarean section. Clin Pract. 2011;1:57.

Publication Dates

• Publication in this collection
  Jul-Aug 2017