An Illustration of a Challenging Airway Where Expertise Prevails Over Technology – Goldenhar Syndrome

To the Editor: According to the American Society of Anesthesiologists (ASA) Task Force on Management of the Difficult Airway, a difficult airway is “the clinical condition in which an anesthesiologist with conventional training encounters difficulty with facemask ventilation of the upper airway, trouble with tracheal intubation, or both.”[1] The anesthesiologist’s toolkit is stocked full of methods and equipment that can be used to successfully treat a challenging airway.[2] Experience and familiarity with the tools at hand, however, will dictate a person’s strategy and are unquestionably more significant than the gadget itself.[3] Goldenhar syndrome, also known as oculo-auriculo-vertebral dysplasia, is characterized by a wide range of congenital anomalies, such as micrognathia, oral cavity malformations, and vertebral anomalies, and always causes difficult airways.[4] Our patient, a female toddler aged 3 years, was scheduled for intracardiac repair of a ventricular septal defect. The patient’s birth history was uncomplicated, and cyanosis or syncopal attacks were not previously reported. With the aid of two-dimensional (2D) echocardiography and auscultation, ventricular septal defect (VSD) was recently identified in a patient with a history of recurrent upper respiratory infections. The patient’s mouth opening was deemed sufficient during the pre-anesthesia evaluation, and the mallampati grade was determined to be class II. On the neck and bottom edge of both cheeks, there were skin tags on each side, and there was some facial asymmetry. Mandibular hypoplasia was not apparent upon inspecting the profile of the patient [Figure 1].Figure 1: The patient with suspected Goldenhar syndrome showing facial asymmetry and bilateral auricular appendagesInj glycopyrrolate (5 mcg/kg), midazolam (0.1 mg/kg), ketamine (2 mg/kg), and fentanyl (2 mcg/kg) were administered during induction in accordance with body weight, followed by the administration of a muscle relaxant (Inj vecuronium 0.1 mg/kg) once ventilation was verified. A skilled anesthesiologist performed a laryngoscopy, however, only the epiglottis could be seen after proper alignment, external manipulation, and laryngoscopy. The next attempt involved the use of a bougie to assist in intubation, but this failed since it was challenging to get the bougie past the epiglottis. This kind of situation with unanticipated difficult intubation can be tackled with the help of a laryngeal mask airway, fiber-optic bronchoscope, video laryngoscopy or backward upward rightward pressure on larynx externally (BURP) maneuver. However, the availability of all these is always an issue, especially in adequate sizes for a pediatric patient. In this case, a second anesthesiologist with experience joined the team to offer assistance, and the intubation was attempted by the two experts. To enable the best possible view of the vocal chords, one anesthesiologist performed laryngoscopy with one hand and self-applied external laryngeal pressure with another hand. Then another anesthesiologist passed the uncuffed endotracheal tube sized 5.5 over the shoulder. The first anesthesiologist and intubation were done, but there was a leak noted. A five-sized cuffed endotracheal tube was then used for the last attempt at intubation and was successful, which was confirmed with auscultation and end-tidal carbon dioxide [Figure 2].Figure 2: Post intubation showing a receding mandible and skin tagThere was never an episode of desaturation, bradycardia, or tachycardia during all this time. The fact that the patient stayed stable throughout the intubation of a pediatric child for cardiac surgery is due to the experience and collaborative efforts of the two anesthesiologists. After that, the patient was maintained under anesthesia using oxygen + air at 50-50% with the inhalation of sevoflurane. Intravenous infusion of continued at 0.5 mcg/kg/hr. Fentanyl was used intermittently for adequate analgesia, with a total dose under 20 mcg/kg. All the standard ASA parameters were monitored, along with intra-arterial blood pressure and central venous pressure. After this patient was taken over for cardio-pulmonary bypass, VSD was repaired and then the patient was gradually weaned off the cardiopulmonary bypass. Post procedure, no residual VSD was noted on transesophageal echocardiography. The patient was hemodynamically stable, requiring minimal support. The patient was shifted intubated to the cardiothoracic surgery recovery intensive care unit (ICU), where mechanical ventilation was continued throughout the night electively. The patient was extubated the following morning after the sedation had been stopped, and high-flow nasal oxygen was continued for some time. The remainder of the postoperative period went without any incident, and the patient was discharged on day five. Retrospectively, with all the findings, this patient was found to be a suspected case of Goldenhar syndrome. There have been numerous attempts to forecast challenging laryngoscopy in pediatric patients.[5] The Schwartz hyoid maneuver estimates the possible laryngoscopy space by measuring the anteroposterior distance between the hyoid bone and the center of the inside of the mandible. The anesthesiologist should be ready for challenging intubation if there is a decrease in this area, which should be roughly 3 cm in adults and 1.4–1.5 cm in neonates. Unfortunately, even a thorough examination cannot identify every incidence of challenging intubation, which leaves room for unforeseen issues.[6] Our experience with this disease highlights the value of two anesthesiologists combining knowledge and teamwork in overcoming challenges to enable patients to attain a secured airway while maintaining stable vital signs and preventing desaturation. The most crucial factor is the confidence one develops through time and with a strong team. Data availability statement The data used in support of this article has been added in the references. Author contribution Dr VKS and Dr SH did clinical management of patient and preparation of initial draft of manuscript. Dr PR have written the case report and did required modifications and revisions. All the authors provided critical inputs into this case report writing, and approved its final version. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.