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CASE PRESENTATION

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A 21-month-old boy with CHARGE syndrome was brought to the operating room for a gastroscopy, echocardiogram, auditory brainstem response (ABR) test, ear tubes and examination of his airway under general anesthesia. His past 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 record, it was noted that he was difficult to perform bag-mask-ventilation (BMV) and that the use of an extraglottic airway device (EGD) did not improve ventilation of the lungs. It was also found that direct laryngoscopy (DL) 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 anesthetic 1 year ago.

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After induction with sevoflurane and oxygen, an intravenous cannula was placed, and satisfactory ventilation and oxygenation were maintained with BMV and 20 cm H2O continuous positive airway pressure (CPAP). An initial attempt at tracheal intubation under DL with a Miller laryngoscope revealed a Cormack–Lehane Grade 3 view. Tracheal intubation was unsuccessful. BMV then became impossible. A further attempt at tracheal intubation was also unsuccessful.

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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. The resulting hypoxemia caused severe bradycardia leading to cardiac arrest which required 15 minutes of cardiopulmonary resuscitation (CPR). A needle tracheotomy was also attempted and failed. This was followed by an urgent surgical tracheotomy by a surgeon, which was successful at establishing an airway and adequate oxygenation.

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The tracheotomy consisted of a longitudinal midline scalpel incision through 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 a tracheal tube under direct vision.

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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. His neurological function was returned to his baseline preoperative state.

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INTRODUCTION

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What Is the CHARGE Syndrome and What Are the Pathological Features?

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CHARGE syndrome involves multiple congenital anomalies that can be life threatening. The acronym CHARGE stands for Colobomas of the eye, Heart disease, Atresia of the choanae, Retarded growth or central nervous system anomalies, Genital anomalies or hypogonadism, and Ear anomalies or deafness.

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Features have been further divided into major and minor criteria of the CHARGE syndrome. Major include the classic 4Cs (Choanal atresia, Coloboma, Characteristic ear, and Cranial nerve anomalies). Minor criteria include cardiovascular malformations, genital hypoplasia, ...

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