PDF(Pubmed)
Abstract：
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.
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.
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.
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.
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.
摘要：
背景：Goldenhar综合征是一种先天性疾病，涉及由第一和第二咽弓以及或多或少严重的颅外异常引起的结构缺失或发育不足。可以观察到各种声门上畸形，包括下颌骨发育不全,下颌不对称和微下颌畸形。声门下气道狭窄(SGS),这可能导致围手术期气道管理困难，在对Goldenhar综合征的文献描述中很少强调，但可能具有临床意义。
方法：一名18岁女性，有Goldenhar综合征病史，因放置右下颌牵引器而出现，右耳后扩张器,并在全身麻醉下转移预制扩张皮瓣。气管插管时,当试图通过声门时，气管内导管（ETT）意外地遇到阻力。随后，我们尝试使用较小尺寸的ETT进行手术，但再次遇到阻力。用纤维支气管镜，我们发现气管和双侧支气管的整个节段明显狭窄。考虑到意外的严重气道狭窄的发现以及进行手术的相关风险，手术被取消了。一旦患者完全清醒，我们就取出了ETT。
结论：麻醉医师在评估Goldenhar综合征患者的气道时，应注意这一临床发现。计算机断层扫描（CT）和三维图像重建的冠状和矢状测量可用于评估声门下气道狭窄的程度并测量气管的直径。
方法：一名18岁女性，有Goldenhar综合征病史，因放置右下颌牵引器而出现，右耳后扩张器,并在全身麻醉下转移预制扩张皮瓣。气管插管时,当试图通过声门时，气管内导管（ETT）意外地遇到阻力。随后，我们尝试使用较小尺寸的ETT进行手术，但再次遇到阻力。用纤维支气管镜，我们发现气管和双侧支气管的整个节段明显狭窄。考虑到意外的严重气道狭窄的发现以及进行手术的相关风险，手术被取消了。一旦患者完全清醒，我们就取出了ETT。
结论：麻醉医师在评估Goldenhar综合征患者的气道时，应注意这一临床发现。计算机断层扫描（CT）和三维图像重建的冠状和矢状测量可用于评估声门下气道狭窄的程度并测量气管的直径。
