Airway management in Escobar syndrome: A formidable challenge
An 1-year-old female child diagnosed with Escobar syndrome since birth, weighing 10 kg was posted for bilateral hamstring to quadriceps transfer. Pre-operative examination revealed multiple joint flexion contractures. Airway examination showed adequate mouth opening, whereas neck extension was limited due to flexion contracture over neck. Systemic and haematological examinations were unremarkable. The lateral X-ray of the neck showed that there was no cervical spine fusion. The child also had atrial septal defect at birth, which was now closed, with normal electrocardiography (ECG) and echocardiography findings. After adequate nil per oral, patient was shifted to the operation theatre (OT) without premedication. Difficult airway cart was already kept ready in the OT. Preinduction monitors were five lead ECG, pulse oximetry and non-invasive blood pressure. Child was induced by inhalation induction with 6-8% sevoflurane and 100% oxygen. After adequate depth of anaesthesia, intravenous (IV) cannulation was carried out in the right upper limb. After checking ability to mask ventilate, 0.1 mg IV glycopyrrolate, 10 μg IV fentanyl, 5 mg IV propofol were given to increase depth of anaesthesia. Child was paralyzed with 5 mg IV atracurium, and mask ventilation was carried out with 2-3% sevoflurane and oxygen. After 3 min, intubation was attempted with conventional laryngoscopy. Intubation was unsuccessful twice wherein only the tip of epiglottis could be visualized even after optimal external laryngeal manipulation. Further attempts at conventional laryngoscopy were abandoned considering possibility of airway trauma and edema. Patient's saturation was maintained around 96-98% during this period. A size 1.5 proseal laryngeal mask airway (PLMA) was secured in place and capnogram was obtained. Ryles tube was inserted through the gastric channel to deflate the stomach. Even though there was optimal chest expansion, it was accompanied by an increase in end tidal CO2 values to about 55-60 mmHg after 5 min of controlled ventilation. Furthermore, as the surgery was expected to last about 3 h, we decided to proceed with PLMA-assisted fibreoptic guided endotracheal intubation. A 4.0 mm internal diameter uncuffed portex endotracheal tube (ETT) was railroaded over the bronchoscope of 2.4 mm outer diameter and then the bronchoscope was introduced through the lumen of PLMA into the trachea. The ETT was then advanced through the lumen of the PLMA into the trachea and tip position was confirmed above the carina by visualization through bronchoscope. Equal bilateral air entry was confirmed by auscultation. The PLMA with ETT in situ was fixed as a single unit []. Anaesthesia was maintained with 66% nitrous oxide, 33% oxygen and sevoflurane 1.5-2%. With adequate ventilation, the ETCO2 of 35-37 mmHg was maintained throughout surgery. Caudal analgesia was given after careful positioning with 10 mL of 0.2% ropivacaine and 10 μg clonidine. Surgery was carried out in supine position with application of tourniquet. Rest of the intraoperative period was uneventful. At the end of the procedure, child was reversed, and when she was fully awake and breathing adequately, the PLMA and ETT were removed as a single unit after oral and gastric suctioning. Child was shifted to a high dependency unit for monitoring.