大头型先天性肺气道畸形胎儿的产前与围产结局：胸腔羊膜腔分流与期待治疗比较
该回顾性队列研究纳入1998—2024年安大略胎儿中心123例孤立性大头型CPAM胎儿，比较胸腔羊膜腔分流（TAS）与期待治疗的产前影像、围产及出生后结局。TAS组30例（24%），其CVR、囊肿直径、纵隔移位及胎儿水肿比例均更高；TAS后80%病例病灶缩小，但生存率更低（85% vs. 100%）、早产更常见（24% vs. 7%），新生儿呼吸支持需求更高（86% vs. 25%）。
要点
- 大头型CPAM中约24%需TAS，TAS组基线CVR、囊肿直径及水肿比例更高。
- TAS后80%病灶缩小，但生存率85% vs. 100%，早产24% vs. 7%。
- TAS组新生儿呼吸支持86% vs. 25%，出生后切除更早（0.1 vs. 7.0月）。
核心结论
- 大头型CPAM是先天性肺畸形的高危亚型，相当比例需胎儿干预、新生儿呼吸支持和出生后手术切除。
- 研究结果支持此类胎儿在三级中心进行监测和分娩。
研究设计与样本
- 回顾性队列研究，纳入1998—2024年安大略胎儿中心123例孤立性大头型CPAM胎儿。
- 比较接受TAS（30/123，24%）与期待治疗胎儿的产前超声特征、围产结局及出生后手术数据。
- 在TAS组中评估死亡预测因素。
主要发现
- TAS组与期待组相比：CVR 1.5 vs. 0.5，囊肿直径3.4 vs. 1.0 cm，纵隔移位58% vs. 7%，胎儿水肿16.7% vs. 0%。
- TAS后80%病例病灶缩小，但生存率更低（85% vs. 100%），早产更频繁（24% vs. 7%）。
- TAS组新生儿呼吸支持需求更高（86% vs. 25%），出生后切除更早（0.1 vs. 7.0个月）。
- 在TAS组中，CVR > 2或持续性胎儿水肿与围产期死亡相关。
关键数值与推荐
- TAS组CVR > 2或持续性胎儿水肿与围产期死亡相关。
- TAS后80%病例病灶缩小。
- TAS组生存率85%，期待组100%；TAS组早产率24%，期待组7%。
- TAS组新生儿呼吸支持率86%，期待组25%。
- 出生后切除时间：TAS组0.1个月，期待组7.0个月。
临床怎么用
- 大头型CPAM胎儿应在三级中心监测和分娩，因需胎儿干预、新生儿呼吸支持及出生后手术切除的比例较高。
- 对考虑TAS的胎儿，CVR > 2或持续性胎儿水肿提示围产期死亡风险较高。
- TAS后病灶缩小但早产和新生儿呼吸支持需求增加，出生后切除时机更早。
以上解读依据论文摘要（全文未开放获取或许可不允许翻译转载）。
原文
OBJECTIVE: Describe prenatal imaging characteristics, perinatal and postnatal outcomes in fetuses with isolated macrocystic congenital pulmonary airway malformations (CPAM) undergoing thoracoamniotic shunting (TAS) and expectant management. METHODS: Retrospective cohort study of 123 fetuses with isolated macrocystic CPAM managed at the Ontario Fetal Center (1998-2024). Prenatal ultrasound features, perinatal outcomes, and postnatal surgical data were described for fetuses undergoing TAS and expectantly managed. Predictors of mortality were assessed in the TAS group. RESULTS: TAS was performed in 30/123 (24%) of cases. Shunted fetuses had higher CPAM volume ratio (CVR) (1.5 vs. 0.5), larger cyst diameter (3.4 vs. 1.0 cm), mediastinal shift (58% vs. 7%), and hydrops (16.7% vs. 0%). Lesion size decreased after TAS in 80% of cases; however, survival was lower (85% vs. 100), and preterm delivery was more frequent (24% vs. 7%). Among shunted cases, CVR > 2 or persistent hydrops were associated with perinatal mortality. Neonatal respiratory support was required more often following TAS (86% vs. 25%). Most infants underwent postnatal resection, occurring earlier after TAS (0.1 vs. 7.0 months). CONCLUSIONS: Macrocystic CPAM represents a high-risk subtype of congenital lung malformations, with a substantial proportion requiring fetal intervention, neonatal respiratory support, and postnatal surgical resection. These findings support tertiary surveillance and delivery.
引用
- Carmant LS, Kelly A, Lee S, et al. Prenatal and Perinatal Outcomes in Fetuses With Macrocystic Congenital Pulmonary Airway Malformations: Thoracoamniotic Shunting Versus Expectant Management. Prenat Diagn. 2026. PMID: 42178491. doi:10.1002/pd.70172
供执业医师工作参考，不替代临床判断；数值与分级以指南原文为准。