Sleesman Jay B, Tobias Joseph D
Departments of Anesthesiology and Child Health, University of Missouri, Columbia, MO 65212, USA.
Paediatr Anaesth. 2003 Sep;13(7):629-32. doi: 10.1046/j.1460-9592.2003.01132.x.
Robinow syndrome, originally described in 1969, consists of mesomelic brachymelia, short stature, genital hypoplasia and characteristic facies. Associated organ system involvement may include developmental delay, congenital heart disease, obstructive disorders of the urinary tract and renal cysts. As there is a potential for airway problems related to the facial features, such as midface hypoplasia and micrognathia, perioperative management of these patients is important. The authors present an 8-year-old boy, previously diagnosed with Robinow syndrome, who was admitted for an emergency surgical procedure. The perioperative implications of this disorder are reviewed.
罗宾诺综合征最初于1969年被描述，其特征包括四肢短小的短肢侏儒症、身材矮小、生殖器发育不全以及特殊面容。相关器官系统受累可能包括发育迟缓、先天性心脏病、泌尿道梗阻性疾病和肾囊肿。由于存在与面部特征相关的气道问题的可能性，如面中部发育不全和小颌畸形，因此对这些患者进行围手术期管理非常重要。作者介绍了一名8岁男孩，此前被诊断为罗宾诺综合征，因紧急外科手术入院。本文对该疾病的围手术期影响进行了综述。
