Airway concerns in a neonate with Treacher Collins syndrome posted for tracheoesophageal fistula repair

Abstract

Sir, A 3-day-old male, term neonate, weighing 2.9 kg was referred to our institute for tracheoesophageal fistula repair with provisional diagnosis of Treacher Collins syndrome (TCS). Examination revealed tachypnea, heart rate 160 bpm, blood pressure 68/48 mmHg, and SpO2 reading 98% with 2 L oxygen. Facial dysmorphic features included hypoplasia of facial bones (mandible and maxilla), down-sloping palpebral fissures, coloboma of the left upper eyelid, scanty lower eyelash, micrognathia, retrognathia, and malformation of auricle with atresia of the external auditory canals, diagnosed as TCS [Figure 1]. Thoracotomy for ligation of tracheoesophageal fistula with primary repair of esophagus was planned under general anesthesia. Blood investigations and arterial blood gas analysis were unremarkable. Echocardiography revealed atrial septal defect of 4 mm with left-to-right shunt. Inhalational anesthesia with spontaneous breathing was planned to administer anesthesia. Standard monitoring was done. Difficult airway cart was kept ready in view of difficult airway secondary to micrognathia and retrognathia, including face mask, oropharyngeal, nasopharyngeal airway, endotracheal tube, and laryngeal mask airway of assorted sizes, stylet, laryngoscope with straight and curved blades, and tracheostomy tray. Intravenous glycopyrrolate 30 mcg and dexamethasone 0.3 mg was given as premedication. After preoxygenation, anesthetic plane was deepened with gradually increasing concentration of sevoflurane up to eight volume percent with 100% oxygen via Jackson Rees circuit. Effective mask ventilation was achieved with two-hand technique and jaw thrust. Gentle laryngoscopy was performed by experienced anaesthetist to avoid airway trauma. Laryngoscopy was performed with Miller size 0 blade, which showed Cormack Lehane Grade III view and without any improvement with external laryngeal maneuver. Further attempt of laryngoscopy was performed with Miller size 1 blade which improved laryngoscopic view to Cormack Lehane Grade II with external laryngeal maneuver followed by orotracheal intubation with 2.5-sized endotracheal tube with stylet. Anesthesia was maintained with 100% oxygen (as air not available with institute), sevoflurane, and intermittent doses of fentanyl and atracurium. Surgery was uneventful with minimal intraoperative blood loss. Neonate was shifted to intensive care unit with ventilatory support for postoperative care. He succumbed on the 5th postoperative day due to septicemia.