非典型的固体类型先天性肺气道形 (CPAM) 1型的临床特征
Mizuho Igarashi1, Hidehiko Maruyama1, Osamu Miyazaki2
1Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, Japan.
Pediatrics and neonatology
|December 22, 2024
概括
非典型的1型先天性肺气道形 (CPAM) 在胎儿MRI上呈现为固体肺质量. 较大的CPAM体积比 (CVR) 与由于呼吸困难而需要早期新生儿手术相关.
科学领域:
- 儿科手术 儿科手术
- 胸部成像 胸部成像
- 遗传性肺部异常 遗传性肺部异常
背景情况:
- 第1型先天性肺气道形 (CPAM) 通常涉及大型,扩张的囊,容易在出生后迅速透气.
- 非典型的1型CPAM可以表现为固体肺质量,在胎儿MRI上具有异质信号强度.
研究的目的:
- 描述非典型的1型CPAM的临床,成像和组织学特征.
- 评估CPAM体积比率 (CVR) 与早期手术干预的需要之间的相关性.
主要方法:
- 在2002年3月至2022年12月期间被诊断患有非典型1型CPAM的婴儿的回顾性评估.
- 对胎儿T2加权MRI,产后胸部X射线和外科病理发现的分析.
主要成果:
- 确定了四名患有非典型的1型CPAM的婴儿,所有这些婴儿在胎儿MRI上都显示出固体肺质量,需要呼吸支持.
- 三名CVR≥1.6的婴儿因呼吸衰竭在2天内需要紧急手术.
- 组织学揭示了小型CPAM1型囊在类似于CPAM2型的固体成分内.
结论:
- 跨胎儿MRI,胸部X射线和组织学一致的发现特征这种固体型非典型的1型CPAM.
- 升高的CVR与新生儿早期呼吸衰竭和手术干预的风险增加有关.
- 由于其质量效应,建议对大型非典型的1型CPAM病变进行早期手术切除.
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