Kim Tae Hee, Kim Young Uk, Song Jun Gol, Hwang Jai Hyun
Department of Anesthesiology and Pain Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Anesthesiol. 2009 Jun;56(6):717-719. doi: 10.4097/kjae.2009.56.6.717.
Seckel syndrome, also called bird-headed dwarfism, is an extremely rare, inherited, autosomal recessive disorder. The patients with Seckel syndrome are characterized by growth retardation, microcephaly with mental retardation, proportional dwarfism, bird like faces, and beak-like triangular nose. A literature review reveals that they have multiple anesthetic problems such as difficult airway management, difficult venous cannulation and concomitant medical diseases. We describe our experience in anesthetic management of a 21-month-old male patient with Seckel syndrome associated with pneumonia who underwent orchiopexy for bilateral cryptochidism.
塞克尔综合征,也称为鸟头侏儒症,是一种极其罕见的遗传性常染色体隐性疾病。塞克尔综合征患者的特征是生长发育迟缓、小头畸形伴智力障碍、比例性侏儒症、鸟样面容和喙状三角形鼻子。文献综述显示,他们存在多种麻醉问题,如气道管理困难、静脉穿刺困难以及合并内科疾病。我们描述了对一名21个月大患有塞克尔综合征并伴有肺炎的男性患者进行麻醉管理的经验,该患者因双侧隐睾症接受了睾丸固定术。