Peker Kevser, Ergil Julide, Öztürk İbrahim
Clinic of Anaesthesiology, Dışkapı Yıldırım Beyazıt Training and Reseacrh Hospital, Ankara, Turkey.
Turk J Anaesthesiol Reanim. 2015 Oct;43(5):360-2. doi: 10.5152/TJAR.2015.52714. Epub 2015 Aug 21.
Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%-3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance. Patients with Waardenburg syndrome stand for difficult airway. We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation.
瓦登伯革氏综合征是一种罕见的常染色体显性疾病，可导致听力丧失、色素异常、神经嵴病变和部分白化病。据估计，在先天性耳聋病例中发病率为2%-3%，在普通人群中为1/42000。患有瓦登伯革氏综合征的儿童通常在幼年时需要进行人工耳蜗植入手术的麻醉。该综合征可能对麻醉管理具有重要意义的特征包括喉软化、多处肌肉挛缩、颈部活动受限、青紫型心脏病和电解质失衡。瓦登伯革氏综合征患者代表着困难气道。我们旨在报告一名接受人工耳蜗植入手术的瓦登伯革氏综合征患儿的麻醉管理情况。
