摘要胎儿肺部先天性囊性腺瘤样畸形（congenital cystic adenomatoid malformation，CCAM）的病程、进展及预后取决于肿块大小、纵隔是否移位、胎儿血流动力学是否改变，以及是否发生胎儿水肿，其中巨大CCAM相对少见。本例孕27周疑诊胎儿巨大CCAM合并胎儿水肿及心功能不全，进行多次影像学评估及多学科会诊，制定了分娩方式及新生儿抢救预案后，孕31周 +4行胎儿胸腔穿刺引流术。术后胎儿CCAM较前缩小，同时母体胸闷、憋气等情况均得以改善。术后3 d（孕32周）孕妇胎膜早破，阴道分娩一男婴。生后5 d新生儿进行了肺部囊腺瘤切除术并明确诊断。生后2年持续随访，母儿均预后良好。孕期发现CCAM时，应充分进行宫内评估，并根据围产期可能出现的情况，制定相应的监测方案，避免不必要的终止妊娠。若继续妊娠，应进行多学科评估，为围产期相关手术做准备。

abstractsThe course, progression, and prognosis of fetal congenital cystic adenomatoid malformation (CCAM) depend on the size of the mass, whether the mediastinum is displaced, changes in fetal hemodynamics, and the occurrence of fetal hydrops, with large CCAM being relatively rare. In this case, a fetus was suspected of having a large CCAM with fetal hydrops and cardiac insufficiency at 27 weeks of gestation. After multiple imaging evaluations and multidisciplinary consultations, a delivery plan and neonatal resuscitation plan were formulated. At 31 +4 weeks of gestation, in-utero thoracentesis and drainage were performed. Postoperatively, the fetal CCAM was reduced compared to before, and the maternal symptoms of chest tightness and shortness of breath were also improved. Three days after the procedure, the mother experienced premature rupture of membranes and delivered a male infant vaginally at 32 weeks of gestation. On the fifth day after birth, the newborn underwent resection of the pulmonary cystic adenomatoid malformation, confirming the diagnosis. Follow-up for two years post-birth showed good prognosis for both the mother and the child. Therefore, when CCAM is detected during pregnancy, thorough in-utero evaluation should be conducted, and a monitoring plan should be developed based on potential perinatal conditions to avoid unnecessary termination of pregnancy. If the pregnancy continues, multidisciplinary evaluation and preparation for perinatal surgery are necessary.