Subglottic Airway Stenosis affects Surgery in Goldenhar Syndrome patient: Case Report

A recent case presented in the journal BMC Anesthesiology reported the Goldenhar Syndrome is complicated due to the presence of subglottic airway stenosis. Subglottic airway stenosis (SGS), which causes difficulties in airway management during the perioperative period can pose a potential threat during endotracheal tube insertion. 

The congenital disease known as Goldenhar syndrome occurs due to multifactorial genetic and environmental conditions. It is characterized by more or less severe extracranial malformations as well as the absence or underdevelopment of structures that come from the first and second pharyngeal arches. Mandibular hypoplasia, mandibular asymmetry, and micrognathia are just a few examples of the various supraglottic anomalies that may be seen. Subglottic airway stenosis is one of the complications that are less described. Hence researchers reported the case of an 18-year-old female with Goldenhar Syndrome and its complications during anesthesia induction. 

An 18-year-old female with Goldenhar syndrome presented for placement of a right mandibular distractor, right retro auricular dilator, and stage I transfer of a prefabricated expanded flap under general anesthesia. But during tracheal intubation, the endotracheal tube (ETT) met resistance unexpectantly when attempting to pass through the glottis. Subsequently, it was attempted with a smaller size ETT but resistance was met again. With a fiberoptic bronchoscope, it was found that the whole segment of the trachea and bilateral bronchi were very narrow. Chest CT was done that showed narrowed trachea and bilateral bronchi. Considering the associated risks for further surgical proceedings due to the unexpected severe airway stenosis, the operation was canceled. ETT was removed after the patient woke up. The patient underwent the procedure later under local anesthesia after 4 days.  

Airway stenosis can be acquired or congenital. But the occurrence of congenital stenosis is rare. It is difficult to identify subglottic airway stenosis before surgery as tracheoscopy and chest CT are not routine examinations. Hence as Goldenhar syndrome patients not only have supraglottic complications but also subglottic airway stenosis, care should be taken while evaluating such cases for surgery. 

Further reading: Xing, F., Deng, X.m. & Yang, D. Goldenhar syndrome complicated with subglottic airway stenosis: a case report. BMC Anesthesiol 23, 210 (2023). https://doi.org/10.1186/s12871-023-02179-w