Lupariello Francesco, Di Vella Giancarlo, Botta Giovanni
University of Turin, Torino, Italy.
Fetal Pediatr Pathol. 2020 Apr;39(2):179-183. doi: 10.1080/15513815.2019.1644688. Epub 2019 Jul 25.
Congenital High Airway Obstruction Syndrome (CHAOS) is an uncommon anomaly. Prenatal ultrasonography allows a prenatal diagnosis to prepare for immediate surgical correction at birth. If the obstruction is severe and a correct therapeutic approach is not planned, CHAOS can cause neonatal death shortly after delivery from a potentially surgically correctable lesion. This neonate died unexpectedly shortly after delivery due to CHAOS. Ultrasonographic findings of enlarged echogenic lungs, dilated airways distal to the obstruction, flattened or inverted diaphragms, or ascites were absent. This was due to a type-II laryngeal-atresia and a type-C esophageal-atresia, with a resultant distal fistula that allowed intrauterine decompression of the fluid in the lungs. The absence of prenatal ultrasonographic findings of CHAOS may be due to a lower fistula between respiratory and gastrointestinal tracts. This set of associations may not be a surgically correctable cause of CHAOS.
先天性高气道梗阻综合征（CHAOS）是一种罕见的异常情况。产前超声检查可进行产前诊断，为出生时立即进行手术矫正做好准备。如果梗阻严重且未规划正确的治疗方法，CHAOS可导致新生儿在出生后不久因潜在可手术矫正的病变而死亡。该新生儿在出生后不久因CHAOS意外死亡。超声检查未发现肺回声增强、梗阻远端气道扩张、膈肌扁平或倒置或腹水等表现。这是由于II型喉闭锁和C型食管闭锁，导致远端瘘管形成，从而使肺内液体在宫内得以减压。产前超声未发现CHAOS表现可能是由于呼吸道和胃肠道之间存在较低的瘘管。这一系列关联可能不是CHAOS的可手术矫正病因。
