Zhang Peilin, Herring Dan, Cook Linda, Mertz Heather
Department of Pathology, Obstetrics and Gynecology, West Virginia University, Morgantown, USA.
J Perinatol. 2005 Jun;25(6):426-8. doi: 10.1038/sj.jp.7211329.
Congenital high airway obstructive syndrome (CHAOS) is a rare but fatal disease with predictably characteristic features including stenotic or atretic upper airway, hyperplastic lungs, elevated diaphragm, massive fetal ascites and fetal hydrops. Diagnosis of CHAOS by ultrasound scan is possible and clinically important since advanced intrauterine surgery to correct the defect is possible. We report a case of fetus of CHAOS with massive ascites, pulmonary hyperplasia and laryngeal stenosis/atresia. We feel that it is important to recognize the entity both by ultrasound scan and by the pathologist so that some cases can be corrected by intrauterine fetal surgery.
先天性高气道梗阻综合征(CHAOS)是一种罕见但致命的疾病,具有可预测的特征,包括上气道狭窄或闭锁、肺增生、膈肌抬高、大量胎儿腹水和胎儿水肿。通过超声扫描诊断CHAOS是可行的,且具有重要的临床意义,因为可以进行先进的宫内手术来纠正缺陷。我们报告一例患有大量腹水、肺增生和喉狭窄/闭锁的CHAOS胎儿病例。我们认为,通过超声扫描和病理学家识别该疾病很重要,以便一些病例能够通过宫内胎儿手术得到纠正。