背景:患有包括先天性囊性腺瘤样畸形(CCAM)在内的大型肺部病变的胎儿存在心肺功能受损的风险。产前产妇倍他米松和囊肿引流分别用于微囊性病变和巨囊性病变的结局有所改善,但一些病变仍然很大,需要在出生前进行切除(开放式胎儿手术,OFS),在通过产前子宫内治疗(EXIT)分娩时,或剖宫产后立即(切段至切除,STR).我们试图比较接受OFS的胎儿的产前特征和结局,退出,或STR告知决策和产前咨询。
方法:对接受OFS的患者进行了单机构回顾性审查,退出,或STR用于2000年至2021年的产前诊断肺部病变。解剖病理学家对标本进行了审查。病变分为“CCAM”(最大病理组)和“所有肺部病变”,因为在做出护理决定时,在产前评估期间不可能进行病理诊断。产前变量包括初始,最伟大的,和最终CCAM体积比(CVR),倍他米松使用/频率,囊肿引流,和积水的存在。结果包括生存,ECMO利用率,NICU住院时间(LOS),产后使用一氧化氮,和呼吸机日。
结果:69%(85例患者中的59例)的肺部病变切除为CCAM。在病理诊断为CCAM的患者中,最初,最大,OFS患者的最终CVRs最大,其次是EXIT和STR患者。同样,OFS组的积液发生率显著较高,积液消退率较低.虽然囊肿引流率在组间没有差异,母体倍他米松的使用差异显著(OFS60.0%,出口100.0%,STR74.3%;p=0.0378)。值得注意的是,所有OFS都发生在2014年之前。生存率没有差异,呼吸机日,一氧化氮,NICULOS,或群体之间的ECMO。在多变量逻辑建模中,在病理诊断为CCAM的接受切除手术的患者中,NICU出院生存的决定因素包括初始CVR<3.5和需要<3次母体倍他米松剂量.
结论:对于尽管母体给予倍他米松或囊肿引流仍较大的CCAM,通过OFS进行手术切除,退出,或STR是可行的选择,组间生存有利且相当。在现代时代,对于具有持续较大肺部病变的胎儿,已经从OFS和EXIT程序转变为STR。在研究期间,母体倍他米松的使用增加和特殊分娩单元的引入以及对STR与类似的胎儿和新生儿结局的赞赏推动了这种转变。EXIT和OFS与STR相关的产妇发病率降低。因此,努力优化肺部大病变胎儿的多学科围产期护理对于告知患者选择标准和促进STR作为现代首选手术方法非常重要.
方法:四级。
BACKGROUND: Fetuses with large lung lesions including congenital cystic adenomatoid malformations (CCAMs) are at risk for cardiopulmonary compromise. Prenatal maternal betamethasone and cyst drainage for micro- and macrocystic lesions respectively have improved outcomes yet some lesions remain large and require resection before birth (open fetal surgery, OFS), at delivery via an Ex Utero Intrapartum Treatment (
EXIT), or immediately post cesarean section (section-to-resection, STR). We sought to compare prenatal characteristics and outcomes in fetuses undergoing OFS,
EXIT, or STR to inform decision-making and prenatal counseling.
METHODS: A single institution retrospective review was conducted evaluating patients undergoing OFS, EXIT, or STR for prenatally diagnosed lung lesions from 2000 to 2021. Specimens were reviewed by an anatomic pathologist. Lesions were divided into \"CCAMs\" (the largest pathology group) and \"all lung lesions\" since pathologic diagnosis is not possible during prenatal evaluation when care decisions are made. Prenatal variables included initial, greatest, and final CCAM volume-ratio (CVR), betamethasone use/frequency, cyst drainage, and the presence of hydrops. Outcomes included survival, ECMO utilization, NICU length of stay (LOS), postnatal nitric oxide use, and ventilator days.
RESULTS: Sixty-nine percent (59 of 85 patients) of lung lesions undergoing resection were CCAMs. Among patients with pathologic diagnosis of CCAM, the initial, largest, and final CVRs were greatest in OFS followed by
EXIT and STR patients. Similarly, the incidence of hydrops was significantly greater and the rate of hydrops resolution was lower in the OFS group. Although the rate of cyst drainage did not differ between groups, maternal betamethasone use varied significantly (OFS 60.0%,
EXIT 100.0%, STR 74.3%; p = 0.0378). Notably, all OFS took place prior to 2014. There was no difference in survival, ventilator days, nitric oxide, NICU LOS, or ECMO between groups. In multiple variable logistic modeling, determinants of survival to NICU discharge among patients undergoing resection with a pathologic diagnosis of CCAM included initial CVR <3.5 and need for <3 maternal betamethasone doses.
CONCLUSIONS: For CCAMs that remain large despite maternal betamethasone or cyst drainage, surgical resection via OFS, EXIT, or STR are viable options with favorable and comparable survival between groups. In the modern era there has been a shift from OFS and EXIT procedures to STR for fetuses with persistently large lung lesions. This shift has been fueled by the increased use of maternal betamethasone and introduction of a Special Delivery Unit during the study period and the appreciation of similar fetal and neonatal outcomes for STR vs.
EXIT and OFS with reduced maternal morbidity associated with a STR. Accordingly, efforts to optimize multidisciplinary perinatal care for fetuses with large lung lesions are important to inform patient selection criteria and promote STR as the preferred surgical approach in the modern era.
METHODS: Level IV.