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A 21-month-old boy with CHARGE syndrome (Figure 50.1) was brought to the operating room for a gastroscopy, echocardiogram, auditory brainstem response (ABR) test, grommets, and examination of his ears and airway under general anesthesia. His medical and surgical history included tracheobronchomalacia, left choanal atresia, a tracheoesophageal fistula (TOF) repair at age 2 days and insertion of a percutaneous endoscopic gastrostomy (PEG). From his past anesthetic history, it was noted that he had difficulty to face-mask ventilation (FMV) and that the use of an extraglottic airway (EGD) did not improve his ventilation. It was also found that direct laryngoscopy and tracheal intubation were becoming increasingly difficult with successive procedures. The child was assessed preoperatively and it was reported that he remained clinically unchanged since the previous anesthesia one year ago.


A patient with CHARGE syndrome.

After an inhalation induction with Sevoflurane and oxygen, an intravenous cannula was placed, aided by ultrasound, and a satisfactory airway was achieved with FMV and 20 cm H2O continuous positive airway pressure (CPAP). An initial attempt at tracheal intubation via direct laryngoscopy revealed a Cormack-Lehane (CL) Grade 3 view of the epiglottis only. Tracheal intubation was unsuccessful. FMV then became impossible. A further attempt at tracheal intubation by direct laryngoscopy was also unsuccessful. In hindsight, a second attempt at tracheal intubation should have been optimized with patient positioning, apneic oxygenation, and included either video laryngoscopy (VL) or flexible bronchoscopy.1 The choice of VL blade for a child less than 5 kg for optimum efficacy would be a standard Macintosh curved blade. For larger children, it makes no difference whether the blade is hyperangulated or curved Macintosh style.2

Rigid bronchoscopy was rapidly performed by an ENT surgeon in an attempt to establish an airway, but this did not provide adequate oxygenation to the patient. In this case, the resulting hypoxemia caused severe bradycardia leading to cardiac arrest which required 15 minutes of cardiopulmonary resuscitation. A needle tracheotomy was attempted and failed. This was followed by an urgent surgical tracheotomy by a surgeon, which was successful in establishing an airway and adequate oxygenation. The tracheotomy consisted of a longitudinal midline scalpel incision through the skin and subcutaneous tissue. Very little blood loss occurred and this was attributed to the cardiac arrest. The midline dissection continued down to the trachea and a midline cut was performed through approximately three tracheal rings providing sufficient space to advance an endotracheal tube (ETT) under direct vision.

All planned procedures were postponed and the child was transferred to the pediatric intensive care unit. He was cooled and remained sedated for 48 hours, after which he was allowed to wake up. Despite the prolonged resuscitation, his neurological function was equivalent to his preoperative state.


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