Jeong Sang-Hee, Lee Mi-Young, Kang Ok-Ju, Kim Rina, Chung Jin-Hoon, Won Hye-Sung, Lee Pil-Ryang, Jung Euiseok, Lee Byong Sop, Choi Woo-Jong, Lee Yoon Se
Department of Obstetrics and Gynecology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Division of Neonatology, Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Obstet Gynecol Sci. 2021 Jan;64(1):52-61. doi: 10.5468/ogs.20266. Epub 2020 Dec 7.
To report our experience with management of fetuses with congenital high airway obstruction syndrome (CHAOS).
We retrospectively reviewed the cases of fetuses who were prenatally diagnosed and postnatally confirmed with CHAOS between 2010 and 2019 at Asan Medical Center, Seoul, Korea.
Of 13 fetuses prenatally diagnosed with CHAOS, 7 were lost to follow-up and 6 were postnatally confirmed as having CHAOS. All fetuses, except one were delivered via cesarean section with an ex utero intrapartum treatment (EXIT) procedure. Two patients had coexisting congenital heart diseases requiring several cardiac surgeries following birth. Both of these patients demonstrated developmental delay; however, the remaining 4 had a normal development except for expressive language. Two infants died of respiratory complications, and the remaining 4 were alive at the end of the follow-up period. All 4 live patients underwent tracheostomy with planned reconstruction surgery. Three children are now able to phonate, and 1 can maintain a conservation.
The proper management of CHAOS using the EXIT procedure results in high survival and low hypoxemia-induced complication rates. Therefore, an accurate prenatal diagnosis is necessary for an appropriate perinatal management.
报告我们对先天性高气道梗阻综合征（CHAOS）胎儿的管理经验。
我们回顾性分析了2010年至2019年在韩国首尔峨山医学中心产前诊断并产后确诊为CHAOS的胎儿病例。
13例产前诊断为CHAOS的胎儿中，7例失访，6例产后确诊为CHAOS。除1例胎儿外，所有胎儿均通过剖宫产及产时宫外治疗（EXIT）手术分娩。2例患者合并先天性心脏病，出生后需要多次心脏手术。这2例患者均表现出发育迟缓；然而，其余4例除语言表达外发育正常。2例婴儿死于呼吸并发症，其余4例在随访期末存活。所有4例存活患者均接受了气管造口术及计划中的重建手术。3名儿童现在能够发声，1名儿童能够保持交流。
采用EXIT手术对CHAOS进行恰当管理可获得高生存率和低缺氧诱导并发症发生率。因此，准确的产前诊断对于适当的围产期管理是必要的。
