A 17-year-old male, ASA 3, with a BMI of 16.3 kg/m2 (height:1.6 m, weight: 43 kg) and a past medical history of CLS, was scheduled for a resection of a right-sided neck mass diagnosed as a low-grade pleomorphic adenoma through fine needle aspiration cytology. Preoperative examination revealed classical features of CLS, including facial dysmorphisms: hypertelorism, thick nasal septum, prominent low-set ears, retrognathia, and macroglossia - these facial abnormalities complicated airway management due to the risk of difficult ventilation, intubation, and aspiration. The patient’s basic metabolic panel was unremarkable. Neck examination identified a 2.5 centimeter mass underneath the tail of the parotid and a smaller mass immediately below it. Subsequently, imaging findings revealed a 1.3 × 2.1 × 2.2 cm hypoechoic lesion in the subcutaneous tissues of the right neck and right jugulodigastric lymphadenopathy with the largest discrete nodes in the level IB and IIA stations, measuring up to 1.3 cm in the short axis and 2.6 cm in the greatest dimension.

Although CLS typically involves cardiovascular, skeletal, and pulmonary abnormalities, our patient had unremarkable findings in these areas. The patient did not undergo preoperative work-up such as a transthoracic echocardiogram, pulmonary function tests, or bone imaging. However, the decision was made to proceed with surgery as clinically, the patient’s cardiopulmonary status was greater than 10 Metabolic Equivalents of Task (METS). His neurological exam was significant for moderate intellectual disability, developmental delay, and autism, which made cooperation and a thorough examination - like assessment of Mallampati and mouth opening challenging. The estimated thyromental distance was less than two fingerbreadths. Given the risk of a difficult airway, we discussed our tailored anesthesia approach with the patient’s guardian, which included a high-risk, well-informed consent regarding the potential of tracheostomy as a backup plan. The patient’s neurological exam rendered the standard awake intubation approach impractical. Instead, an asleep nasal fiberoptic intubation with the primary objective of maintaining spontaneous ventilation was planned. The surgical intervention included right neck mass excision with facial nerve monitoring. Figure 1 demonstrates the various facial anomalies, notably retrognathia and low-set prominent ears.

Demonstrates the patient’s facial anomalies, notably retrognathia and low-set prominent ears

Our preparation encompassed several vital strategies: Firstly, we maximized patient cooperation through distraction techniques. For airway management, we prepared a range of adjuncts, including a bougie, airway exchange catheter, oral and nasopharyngeal airways, supraglottic devices, bronchoscopes, and direct and indirect laryngoscopes. Additionally, an interdisciplinary discussion with the otolaryngology team ensured preparation for a surgical airway for a failed intubation. We planned to avoid long-acting agents and discussed protocols for waking the patient if initial intubation attempts were unsuccessful. To begin the procedure, a pre-operative intravenous (IV) line was established with distraction technique, and the patient received pre-medication with IV midazolam and glycopyrrolate. The patient was then transferred to the operating room, where a combined approach of mask induction with nitrous oxide and air, along with continuous IV sedation using dexmedetomidine, was performed. Following this, sevoflurane induction was initiated with slow titration to ensure the patient maintained spontaneous ventilation. Once an adequate depth of anesthesia was achieved, nasal fiberoptic intubation with a 6.5 cuffed nasal RAE ETT was performed through the left nares as it appeared more patent and provided better access during surgery. Propofol was administered at a concentration of 10 mg/mL in divided doses, with a total dose of 50 mg given. Successful intubation was confirmed through capnometry, assessment of bilateral breath sounds, and observation of chest rise. The duration of surgery was about 2 h and 45 min. The surgical intervention proceeded without complications, and the patient tolerated the procedure well. After completion of the surgery, the patient was extubated awake and transferred to the post-anesthesia care unit (PACU) for close observation. The patient had adequate pain management and maintained oxygen saturation on room air during recovery in the PACU. Figure 2 demonstrates the successful nasal intubation with features of hypertelorism, small mouth opening, and macroglossia.

Demonstrates successful nasal intubation and facial anomalies including hypertelorism, small mouth opening, macroglossia