Policlinico Universitario Agostino Gemelli, "A. Gemelli" University Polyclinic Foundation, Catholic University of the Sacred Heart, Department of Anesthesiology and Intensive Care Medicine, Rome, Italy.
Policlinico Universitario Agostino Gemelli, "A. Gemelli" University Polyclinic Foundation, Catholic University of the Sacred Heart, Department of Anesthesiology and Intensive Care Medicine, Rome, Italy.
Braz J Anesthesiol. 2021 Mar-Apr;71(2):178-180. doi: 10.1016/j.bjane.2020.10.013. Epub 2021 Feb 6.
Cardiofaciocutaneous syndrome is a rare syndrome characterized by particular craniofacial features, cardiac abnormalities, and multiple organ diseases. Patients present with pulmonary stenosis, hypertrophic cardiomyopathy, short neck, micrognathia, laryngomalacia, and tracheomalacia. These conditions may strongly influence patient perioperative outcomes. We describe a 15-year-old child with cardiofaciocutaneous syndrome presenting for a dentistry procedure. She had an uneventful perioperative and postoperative course except for difficult airway management.
心面皮肤综合征是一种罕见的综合征，其特征为特定的颅面特征、心脏异常和多种器官疾病。患者表现为肺动脉瓣狭窄、肥厚型心肌病、短颈、小颌畸形、喉软化和气管软化。这些情况可能强烈影响患者的围手术期结果。我们描述了一名 15 岁的心面皮肤综合征患儿，该患儿因牙科手术就诊。她的围手术期和术后过程均无异常，仅在气道管理方面存在困难。
