Tsukamoto Masanori, Yamanaka Hitoshi, Yokoyama Takeshi
Department of Dental Anesthesiology, Kyushu University Hospital, Fukuoka, Japan.
Department of Dental Anesthesiology, Faculty of Dental Science, Kyushu University, Fukuoka, Japan.
J Dent Anesth Pain Med. 2017 Sep;17(3):231-233. doi: 10.17245/jdapm.2017.17.3.231. Epub 2017 Sep 25.
Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management. In patients with short stature, the tracheal tube size may differ from what may be predicted based on age. The appropriate size ( internal diameter ) of tracheal tubes for children has been investigated. Congenital heart disease is frequently associated with Wolf-Hirschhorn syndrome. Depending on the degree and type of heart disease, careful monitoring of hemodynamics is important.
沃尔夫-赫希洪综合征是一种由4号染色体缺失引起的罕见遗传性疾病。该综合征患者的特征为颅面发育异常、癫痫发作、生长发育迟缓、智力障碍和先天性心脏病。尽管已有多例报道,但关于沃尔夫-赫希洪综合征患者麻醉管理的信息却非常少。我们遇到了一例需要对一名患有沃尔夫-赫希洪综合征的2岁女童进行麻醉管理的病例。选择合适尺寸的气管导管并在术中维持稳定的血流动力学可能是麻醉管理中的关键问题。对于身材矮小的患者,气管导管尺寸可能与根据年龄预测的尺寸不同。已对儿童气管导管的合适尺寸(内径)进行了研究。先天性心脏病常与沃尔夫-赫希洪综合征相关。根据心脏病的程度和类型,仔细监测血流动力学很重要。