Department of Anesthesiology, West China Hospital, Sichuan University, Chengdu, China.
Department of Burn Plastics, West China Hospital, Sichuan University, Chengdu, China.
Anaesthesist. 2020 Jun;69(6):432-435. doi: 10.1007/s00101-020-00778-7. Epub 2020 May 6.
Crouzon syndrome (CS) is a rare autosomal dominant inherited disorder caused by mutations in the fibroblast growth factor receptor 2 (FGFR2) gene. The disease is characterized by premature fusion of the coronal and sagittal sutures of the skull, resulting in clinical manifestations of midfacial hypoplasia, shallow orbit, maxillary dysplasia, and occasional upper respiratory obstruction. This article presents the case of a child aged 2 years and 7 months with CS scheduled for bilateral tonsillectomy and adenoidectomy. The patient had a difficult procedure of extubation and was reintubated and the tracheal intubation was removed 2 days after surgery. The CS is a rare condition with physical characteristics that can result in difficult airway manipulation. It is important for anesthesiologists to recognize and avoid potential airway complications in the management of such patients through detailed preoperative evaluation and careful observation after surgery to reduce perioperative risks.
克鲁宗综合征（CS）是一种罕见的常染色体显性遗传性疾病，由成纤维细胞生长因子受体 2（FGFR2）基因突变引起。该疾病的特征为颅骨冠状缝和矢状缝过早融合，导致中面部发育不全、眼眶浅、上颌骨发育不良以及偶尔发生上呼吸道阻塞。本文介绍了一例 2 岁 7 个月的 CS 患儿，拟行双侧扁桃体切除术和腺样体切除术。该患者拔管困难，重新插管，并在手术后 2 天取出气管插管。CS 是一种罕见疾病，具有可导致气道操作困难的特征。对于麻醉师来说，通过详细的术前评估和术后仔细观察，识别和避免此类患者潜在的气道并发症非常重要，以降低围手术期风险。
