Goldenhar syndrome complicated with subglottic airway stenosis: a case report

Abstract

Background: Goldenhar syndrome is a congenital disease that involves an absence or underdevelopment of structures that arise from the first and second pharyngeal arches and more or less severe extracranial anomalies. A variety of supraglottic malformations may be observed, including mandibular hypoplasia, mandibular asymmetry and micrognathia. Subglottic airway stenosis (SGS), which can cause difficulties in airway management during the perioperative period, is seldom emphasized in literature descriptions of Goldenhar syndrome, but can be clinically significant.

Case presentation: An 18-year-old female with a history of Goldenhar syndrome presented for placement of a right mandibular distractor, right retroauricular dilator, and stage I transfer of a prefabricated expanded flap under general anesthesia. During tracheal intubation, the endotracheal tube (ETT) met resistance unexpectantly when attempting to pass through the glottis. Subsequently, we attempted the procedure with a smaller size ETT but again met resistance. With fiberoptic bronchoscope, we found that the whole segment of the trachea and bilateral bronchi were obvious narrow. Given the finding of unexpected severe airway stenosis and the associated risks with proceeding with the surgery, the operation was cancelled. We removed the ETT once the patient was fully awake.

Conclusions: Anesthesiologists should be aware of this clinical finding when evaluating the airway of a patient with Goldenhar syndrome. Coronal and sagittal measurements on computerized tomography (CT) and three-dimensional image reconstruction can be used to evaluate the degree of subglottic airway stenosis and measure the diameter of the trachea.

Keywords: Chest computerized tomography; Goldenhar syndrome; Subglottic airway stenosis.

Fig. 1

Physical examination of the patient showed right-sided facial asymmetry, microtia, a short mandible, and non-palpable temporomandibular joint

Fig. 2

 A: The cranial CT imaging of the patient shows that the right mandibular is shorter than the left. The arrow shows that right sided mandible hypoplasia. B: Right side of this patient

Fig. 3

The tracheal mucosa protrudes from the lateral hole of the ETT. The outer diameter of 5-mm ETT is 6.8 mm

Fig. 4

Three-dimensional image reconstruction of chest CT. The minimum diameter of the trachea was only 5.4 mm

Fig. 5

Coronal and sagittal measurements on CT and three-dimensional image reconstruction shows that the whole segment of the trachea and bilateral bronchi were all narrow. The minimum diameter of the trachea was only 5.4 mm. The maximum was 8.8 mm. The airway diameter in normal adults is approximately 15 mm ~ 20 mm