2025 FSA Podium and Poster Abstracts

Introduction/Background: Goldenhar Syndrome is a congenital syndrome caused by malformation of the first and second pharyngeal arches. Its characteristic feature is hemifacial microsomia and TMJ abnormalities, affecting skeletal and soft tissue. However, it also often includes vertebral, ocular, auricular, and cardiac abnormalities. This population often undergoes multiple ENT and maxillofacial surgeries throughout childhood and adolescence, but difficulty securing an airway should be expected. No standardized protocol for airway management exists for management of these patients especially in imaging suites and non-OR settings. 

Case Description: This challenging case details a 12 year old 32 kg boy with Goldenhar syndrome and associated facial manifestations including a cleft lip and palate repaired, as well as cardiac abnormalities including dextrocardia and a repaired vascular ring. He also had extensive ocular abnormalities, including left eye blindness with a prosthesis, and auricular manifestations including left sided hearing loss and developmental delay. His extensive surgical history includes left sided orbital reconstruction and an intraoral palate reconstruction with radial forearm free flap, which had resulted in an intraoral scar band from the maxillary tuberosity towards the left oral commissure causing pain and limited oral mobility. He presented as an outpatient for a scar band release with plastic surgery. He was flagged as a difficult airway, with grade IIb views on video laryngoscope, with multiple intubation attempts in past surgeries. In assessing the patient’s airway, he was Mallampati IV with a small mouth opening and 2 fingerbreadth hyomental distance with the inability to prognath. Therefore, an anesthetic plan focused around maintaining spontaneous ventilation and obtaining a clear view of the glottis on the first attempt. The anesthetic plan included intravenous induction with mask and sevoflurane, followed by intravenous induction with dexmedetomidine and ketamine. Due to his many previous surgeries and cooperative mother, she was able to accompany him into the OR and aid in the mask induction. Airway was first attempted with a video laryngoscope D blade and fiberoptic. Grade I view was obtained and the patient was intubated on the first attempt with a 5.5 cuffed endotracheal tube. The patient safely proceeded through surgery and was able to be extubated swiftly after the scar band release. 

Discussion: Goldenhar Syndrome causes airway anatomy that is diverse and can make intubation difficult especially if no prior airway documentation is available to assess. Visualizing equipment, whether it be through video laryngoscope or fiberoptic use (or both) is a mainstay in intubating Goldenhar patients, and significantly increase the intubation success rate. Mandibular distraction procedures, which are performed to lengthen the small or recessed jaw, can improve future success in intubation attempts, and a clear understanding of previous surgeries should be documented to understand potential airway changes since prior attempts. Maintenance of spontaneous ventilation can help avoid a “can’t ventilate, can’t intubate” situation. Mallampati classification is a poor predictor of difficult intubation due to characteristic small mouth openings, however interincisor gap, forward protrusion of the mandible, and thyromental distance are all strong predictors.