Akçıl Eren Fatma, Dilmen Özlem Korkmaz, Tunalı Yusuf
Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Turk J Anaesthesiol Reanim. 2015 Dec;43(6):427-30. doi: 10.5152/TJAR.2015.93546. Epub 2015 Dec 1.
Costello syndrome is a rare genetic disorder characterised by growth and mental retardation, macrocephaly, short neck and macroglossia. Cardiac involvement can also occur in Costello syndrome and is presented in the form of hypertrophic cardiomyopathy, tachyarrythmias and valvular dysfunction. Nervous system involvement including ventriculomegaly, hydrocephaly and Chiari type 1 malformation are also common. Predisposition of papillomata and malignant tumours are high. General anaesthesia practice in patients with Costello syndrome may be complicated by difficult airway because of macrocephaly, short neck, macroglossia and oral or laryngeal papillomas. The airway management and cardiac abnormalities are the major concerns of an anaesthesiologist in Costello syndrome. We report the anaesthetic management of ventriculo-peritoneal shunt replacement for hydrocephaly in an 18-month-old child with Costello syndrome.
科斯特洛综合征是一种罕见的遗传性疾病，其特征为生长发育迟缓和智力发育迟缓、巨头畸形、短颈和巨舌症。科斯特洛综合征也可出现心脏受累，表现为肥厚型心肌病、快速性心律失常和瓣膜功能障碍。神经系统受累包括脑室扩大、脑积水和 Chiari 1 型畸形也很常见。患乳头状瘤和恶性肿瘤的倾向很高。由于巨头畸形、短颈、巨舌症以及口腔或喉部乳头状瘤，科斯特洛综合征患者的全身麻醉操作可能因气道困难而变得复杂。气道管理和心脏异常是麻醉医生在科斯特洛综合征中主要关注的问题。我们报告了一名 18 个月大患有科斯特洛综合征的儿童因脑积水进行脑室 - 腹腔分流术置换的麻醉管理。
