Klippel-Feil syndrome (KFS) has an incidence of 1:42,000 births and is among one of the congenital causes of difficult airway. Short neck with limited range of movement and cervical instability can lead to neurological damage during laryngoscopy, intubation and positioning for surgery. Associated bone abnormalities such as shoulder changes, congenital elevation and shortening of the scapula can cause ventilatory difficulties due to changes in lung development. We describe airway management of a patient of KFS with Glidescope GVL® (Verathon Inc., Bothell, WA) scheduled for corrective surgery for Sprengel's shoulder, under general anesthesia.
A 29-year-old patient, American Society of Anesthesiologist grade 1, known case of KFS type II was scheduled for corrective surgery for Sprengel's shoulder under general anesthesia. Physical and systemic examinations were normal. Airway was assessed as Malampatti grade I. Patient had a short webbed neck, with limited neck extension (<20°), low posterior hairline, mild cervico thoracic scoliosis and a rotated upturned left scapula. Blood investigations, arterial blood gas analysis, basal spirometry, electrokardiogram, echokardiography and ultrasound abdomen revealed no abnormality. X-ray of cervical spine revealed fusion of C4-7 vertebrae and deviation of the trachea to the right. A three dimensional computerized tomography scan of cervical spine and thorax confirmed fusion of C4-7 vertebrae, crowding of ribs on left side with raised left scapula [Figure 1a] and an omovertebral bar connecting left scapula to vertebral lamina [Figure 1b].
In the operating room, intravenous (IV) access was secured and continuous monitoring of electrokardiogram, non-invasive and invasive blood pressure, arterial oxygen saturation and end tidal carbon dioxide were done (AS5, Datex Ohmeda, Finland). We planned to use Glidescope GVL® (Verathon Inc., Bothell, WA) for elective check laryngoscopy. Difficult airway cart including flexible fibreoptic laryngoscope was also kept available. After preoxygenation, fentanyl 80 mcg and propofol 80 mg was given IV. A check laryngoscopy with Glidescope® revealed Cormack Lehane (CL) grade II view. We decided to proceed with general anesthetic technique, and patient was paralyzed with vecuronium bromide 6.0 mg. Airway was secured with 7.0 mm ID cuffed endotracheal tube using Glidescope® and the position was confirmed. The lungs were mechanically ventilated. Anesthesia was maintained with 60% nitrous oxide in oxygen with 0.4-0.6% isoflurane. Patient had an uneventful intraoperative period. At the end of surgery, residual neuromuscular blockade was reversed. Awake extubation was done after ensuring regular spontaneous breathing. Post-operatively patient was shifted to high dependency unit for observation and discharged from hospital after 10 days.
Patients with KFS type II have fusion of one or two cervical interspaces with hemivertebrae and atlanto-occipital fusion. The lungs of these patients are often difficult to ventilate due to changes in lung development associated with anomalies of cardiac, genitourinary, skeletal and neurologic system. Association of KFS with other syndromes like Goldenhar syndrome, Down's syndrome and fetal alcohol syndrome may complicate anesthetic management of these patients.
A retrospective analysis of 21 patients with KFS showed that 43% of patients developed neurologic deficits with spontaneous, or with minimal trauma and thus forceful neck movements should be avoided. Blind naso-tracheal intubation and laryngeal mask airway have been used in some cases. Awake fibreoptic intubation is considered the safest method for the management of airway.
The role of video laryngoscope in difficult airway has not been defined. The Italian Society of Anesthesia, Resuscitation and Intensive Therapy (SIAARTI) algorithm has suggested the use of video laryngoscope as an alternative to flexible fibreoptic in predicted difficult airway with adequate mouth opening. We decided to perform check laryngoscopy with Glidescope®, asit has an inherent advantage of providing a view of the glottis without need of alignment of the oral, pharyngeal and tracheal axis. Its unique curve of 60° at the midline, ‘look round the corner’, real time visualization, improves CL view grading and the stylet provided appropriately helps in intubation with a rapid learning curve.
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