Williams Christol
AANA J. 2014 Apr;82(2):108-13.
Costello syndrome is a rare genetic disorder with an estimated 300 medical cases worldwide. Typical features that characterize this syndrome include short stature, macrocephaly, developmental delay, loose skin folds, distinctive coarse facial features, and multiorgan system anomalies. The following case report discusses the anesthetic management for a 3-year-old boy undergoing general anesthesia for a scheduled dental restoration, hydrocelectomy, inguinal hernia repair, and bilateral myringotomy with placement of pressure equalization tubes. A scarcity of literature for the anesthetic management of Costello syndrome (also known as faciocutaneoskeletal syndrome) exists. Utilizing an overview of the pertinent literature, clinical practice recommendations are suggested for the anesthetic implications of managing a pediatric patient with this rare syndrome.
科斯特洛综合征是一种罕见的遗传性疾病,全球估计有300例医学病例。该综合征的典型特征包括身材矮小、巨头畸形、发育迟缓、皮肤褶皱松弛、面部特征明显粗糙以及多器官系统异常。以下病例报告讨论了一名3岁男孩在接受全身麻醉进行预定的牙齿修复、鞘膜积液切除术、腹股沟疝修补术以及双侧鼓膜切开置管术时的麻醉管理。关于科斯特洛综合征(也称为面部皮肤骨骼综合征)麻醉管理的文献很少。利用相关文献综述,针对管理患有这种罕见综合征的儿科患者的麻醉问题提出了临床实践建议。