胎儿肺囊性腺瘤的宫内诊断和治疗

胎儿肺囊性腺瘤(CCAM)可以在中孕期通过超声检出,多数可在妊娠期消失或缩小,伴有其他畸形者预后不良。超声动态观察有助于判断预后。CCAM伴发胎儿水肿是胎儿窘迫、胎死宫内的前兆。对于CCAM伴发胎儿水肿,可行宫内治疗。对于没有呼吸症状的新生儿,产后CT检查有助于明确病变及确定随访或择期手术。     

胎儿先天性肺囊性腺瘤22例手术治疗疗效分析

目的:回顾性分析胎儿先天性肺囊性腺瘤(CCAM)的病例特点及临床治疗情况。方法:收集佛山市妇幼保健院2011年5月15日至2018年12月30日分娩的所有CCAM患胎的医疗记录,纳入22例,汇总病例特征、B超扫描特征、CCAM类型、患胎预后等,采用Stata 13进行数据分析。结果:连续监测到14例CCAM患胎肺头比(CVR),3例孕期增大,11例孕期减小或无明显增大,其中1例大囊性CCAM产后完全消失;5例CVR1.6的患胎中有3例CVR2.0,其中有1例出现水肿,CVR2.0患胎预后较好。22例胎儿CCAM中,2例未进行手术干预,4例开放式胎儿手术和7例产时子宫外手术,余下9例行产后切除。2例(CVR3.0)开放式胎儿手术患胎早产肺部发育不完善死亡,对治疗完成并成功存活的20例出现新生儿肺炎、呼吸窘迫症状、气胸等呼吸系统并发症的患儿,采取早期呼吸机通气和住院治疗,预后恢复良好。结论:CCAM治疗时要对胎儿进行预后监测,CVR阈值为2.0时可能会提高监测的准确性。治疗应根据胎儿的情况和多学科讨论后共同合作完成。     

产前超声诊断胎儿先天性肺囊腺瘤一例

患者 ,2 4岁 ,孕 2 5 5周 ,产前常规超声检查 ,胎儿符合孕周 ,羊水池深 9 4cm ,胎儿左肺明显增大 ,回声增强 ,左肺内见 1 2cm× 1 1cm囊腔 ,心脏被挤向右侧 ,胎儿胸腔内液性暗区厚径 0 5cm ,腹腔内液性暗区厚径 0 9cm ,超声提示胎儿左肺类腺样囊腺瘤 (cysticadenomatoidmalformation ,CAM ) ,右肺发育不良 ,胸腹水 ,羊水过多。引产后病理诊断 :胎儿左肺囊性腺瘤样畸形 ,Ⅲ型 Ⅰ型。讨论　胎儿肺囊性腺瘤样畸形是一种罕见的先天畸形 ,它是由于支气管末端过度增生而形成的良性先天性肺实质内的病变 ,可发生在双肺 ,也可为单侧或只影响…     

胎儿腹水的产前诊断与预后

目的 探讨胎儿腹水的病因、诊断和预后.方法 对79例超声诊断为单胎胎儿腹水的产前诊断结果及妊娠结局进行回顾性分析.结果 79例中,超声提示56例(70.9%)为胎儿水肿综合征表现,23例(29.1%)为单纯性腹水.56例胎儿水肿综合征中29例(51.8%)伴心脏扩大.病因诊断显示79例中,30例为α-重型地中海贫血,9例宫内感染,3例母胎Rh血型不合,Turner综合征和13一三体各1例,胎儿脏器畸形引起的腹水19例,16例病因不明,病因的诊断率79.7%.随访中64例孕妇选择终止妊娠(5例胎死宫内),13例足月分娩(10例健存,包括7例胎粪性腹膜炎和3例特发性腹水),2例失访.结论 胎儿腹水病因复杂,α-重型地中海贫血、胎儿脏器结构畸形和宫内感染为主要病因.建议通过系统方案进行产前诊断并对预后作出评估.     

胎粪性腹膜炎产前超声追踪1例

孕妇36岁,平素健康,月经周期规律,本次妊娠早期无感染及药物接触史。2009-09-15孕30周时来我院进行产前常规检查,超声检查显示:腹部切面腹腔内见大量液性暗区,前后径最深处约4.1cm,,肠管漂浮其中;余各器官未发现明显异常,羊水指数:26.1cm。超声提示:宫内妊娠,单活胎,头位,胎盘Ⅰ级,胎儿大小约31周;胎儿腹水;羊水过多;建议复查。2d后(9月17日)再次超声复诊:胎儿腹     

羊水过多的病因与诊断

据文献报道,羊水过多的发病率为0.5～1%。我院1978～1987年间住院分娩23441例中,羊水过多49例,发病率为0.21%。羊水过多的病因:羊水过多者其羊膜组织及羊水化学成分均无明显改变,但与胎儿和母体的一些疾病有关,最多见的首先为胎儿畸形,文献报告25%羊水过多者合并有胎儿畸形。我院49例中合并胎儿畸形者25例占畸形几51%。25例畸形儿中,中枢神经系统畸形18例占72%,其中无脑儿最多占14例,脊柱裂3例,脑积水1例。其他畸形共7例:十二指肠闭锁、先天性心脏病、巨腹双肾积水及巨大膀胱、唇裂腭裂(为双胎儿中之一)、尿道下裂肝大胸水以及肝脾大腹水各1例。畸形儿羊水过多的发病机制:无脑儿及脊柱裂系由于脑脊髓神经外露缺乏覆盖,易受刺激     

妊娠11～13~(+6)周超声结构筛查在胎儿中枢神经系统畸形诊断中的应用

目的:探讨规范化早孕期超声结构筛查在胎儿中枢神经系统畸形诊断中的临床价值。方法:对6902例(8336胎)孕11~13~(+6)周胎儿(单胎妊娠5468例,双胎妊娠1434例)行规范化早孕期超声结构筛查,并追踪妊娠过程和临床结局。结果:6902例(8336胎)11~13~(+6)孕周胎儿中产前超声检出中枢神经系统畸形13例(单胎妊娠10例,双胎妊娠3例),其中露脑畸形5例,无脑儿4例,前脑无裂2例,脑膜膨出1例,开放性脊柱裂1例。合并其他结构异常4例,染色体核型异常3例。13例胎儿随访结果:除3例双胎畸形胎儿经选择性减胎术后正常胎儿继续妊娠,余胎儿引产前超声与引产后尸检结果相符合。结论:规范化早孕期超声结构筛查可及早发现胎儿中枢神经系统畸形,对降低畸形胎儿的出生率及指导产科处理均有重要临床价值。     

胎儿骶尾部畸胎瘤的产前诊断和预后影响因素分析

目的探讨胎儿骶尾部畸胎瘤(SCT)的产前诊断和预后因素。方法回顾性分析2014年1月至2021年9月就诊于浙江大学医学院附属妇产科医院并经产前超声诊断(诊断孕周≤28周)为胎儿SCT且继续妊娠的孕妇41例, 分析其产前影像学特征和妊娠结局, 包括肿瘤体积与胎儿体重比(TFR)、肿瘤实性部分占比、肿瘤生长速率(TGR)、是否存在胎儿水肿、胎盘增厚以及羊水过多。采用受试者工作特征(ROC)曲线分析确定TFR、TGR预测胎儿不良结局的临界值。结果 (1)41例妊娠SCT胎儿孕妇的超声诊断孕周为(24.2±2.9)周(范围:18～28周), 其中1例孕22周进展为胎儿水肿引产, 1例孕29周发生胎儿宫内死亡引产, 39例继续妊娠直至分娩。39例继续妊娠的孕妇中, 1例因妊娠晚期发生恶性羊水过多、胎儿心胸比增大于孕31周行剖宫产术, 1例因胎儿心力衰竭于孕32周行剖宫产术, 1例因胎儿心力衰竭合并水肿于孕32周行剖宫产术;其余36例均于新生儿出生后3周内行手术切除肿瘤, 预后均良好。(2)妊娠28周前TFR>0.12可预测胎儿预后不良, 敏感度为100.0%, 特异度为86.1%, 曲线下...     

非免疫性胎儿水肿临床分析

目的:探讨非免疫性胎儿水肿的特点、临床处理及预后。方法:回顾分析2010年7月至2015年6月就诊于南京医科大学第一附属医院产科的20例胎儿水肿孕妇资料、胎儿情况及新生儿情况。结果:20例胎儿水肿均为非免疫性胎儿水肿。发现时间:妊娠14+~37+周;早孕发现2例,中孕发现4例,晚孕发现14例。有胸腔积液表现17例,腹水表现9例,皮肤水肿表现11例,羊水过多9例;脐动脉舒张期血流缺失3例,超声发现胎儿畸形4例。胎儿水肿综合征12例:7例流产、引产;其余为死胎、死产、新生儿死亡;不典型胎儿水肿8例:2例新生儿死亡,2例失访,4例预后良好。结论:部分非免疫性胎儿水肿的确切原因不明确;临床表现多样,胎儿水肿综合征预后不良,孕产妇对胎儿期望值各不相同,需针对不同情况,采取不同的监测、分娩、救治措施。     

妊娠晚期羊水过多57例临床分析

目的 探讨妊娠晚期羊水过多的妊娠结局及无激惹试验的临床应用价值。方法 对妊娠晚期羊水过多孕妇５７例进行回顾性分析和总结,以比较羊水过多的发生时间、性质、程度与胎儿畸形的相关性,及无激惹试验正常和异常与围生儿预后的关系。结果 羊水过多发生在妊娠中晚期（２９～３６＾＋６周）胎儿畸形发生率明显高于妊娠足月（３７～４２周,Ｐ〈０．０５）;急性或重度羊水过多和无激惹试验异常的胎儿窘迫、新生儿窒息及胎儿畸形发     

Percutaneous laser ablation of fetal congenital cystic adenomatoid malformation: too little, too late?

OBJECTIVE: Congenital cystic adenomatoid malformation, type III (CCAM III) lesions are large, bulky tumors which can cause mediastinal shift, prevent normal pulmonary growth, and compress the esophagus, thus leading to complications of nonimmune hydrops, pulmonary hypoplasia and polyhydramnios. Because the mortality rate of untreated fetuses with CCAM and hydrops is high, early delivery or intrauterine resection of the enlarged pulmonary lobe (lobectomy) is indicated; however, open fetal resection of CCAM at less than 30 weeks is associated with perioperative mortality that approaches 40%, as well as the usual maternal and fetal morbidity of open fetal surgery. As an alternative, percutaneous laser ablation of a CCAM III lesion with hydrops was attempted. METHODS: A 30-year-old G3 P1011 with CCAM III in the left fetal hemithorax developed mediastinal shift, hydrops and polyhydramnios at 23 weeks' gestation. After pregnancy termination and open fetal resection were declined, an 18-gauge needle was placed into the fetal tumor percutaneously under real-time ultrasonographic guidance, using sterile technique with light sedation. A cleaved 400-microm Nd:YAG laser fiber was passed through the needle lumen, and using a power setting of 15 W, a total of 2,943 J of laser energy was delivered in pulses of 1.0 s at 0.2-second intervals over two sessions one week apart. RESULTS: Although tumor size decreased, the hydrops worsened and fetal death occurred. CONCLUSIONS: The fetus with CCAM complicated by hydrops is already so compromised by the advanced state of the disease that insufficient time is available for necrotic tissue reabsorption after minimally invasive therapy with laser energy. Until earlier markers for intervention are determined, percutaneous laser debulking of CCAM is unlikely to be successful.     

Prognostic factors associated with congenital cystic adenomatoid malformation of the lung

This study presents 18 cases of prenatally diagnosed congenital cystic adenomatoid malformation (CCAM) to identify potential factors that could predict prognosis. Comparisons of prenatal parameters were made between fetuses that survived and those that died perinatally. It was found that microcystic lesion, bilateral lung involvement and hydrops were each highly correlated with poor prognosis, while neither polyhydramnios nor mediastinal shift was significantly associated with had outcome. Fetal interventions were indicated only in two of the surviving cases: a thoracocentesis and a cysto-amniotic shunt. A therapeutic amniocentesis was performed in one case of polyhydramnios. The diagnosis of CCAM was histologically confirmed in all cases by necropsy or by postnatal lobectomy.     

Successful intrauterine therapy for congenital cystic adenomatoid malformation of the lung. A case report

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare abnormality which is amenable to be diagnosed by prenatal ultrasonography. In general, CCAM associated with non-immune hydrops has a poor prognosis unless a fetal intervention is performed. In some series almost 100% of either prenatal or early neonatal deaths are observed without intervention. Recently the cystic adenomatoid malformation volume ratio (CVR) has been proposed as an index to predict the development of hydrops in this condition. If the CVR is >1.6, the risk of hydrops is approximately 75%. We report a case of CCAM (macrocystic type) of the left lung referred to our center at 21 weeks of gestation. Fetal therapy was considered owing to enlargement of the CCAM volume, severe mediastinal shift, right lung compression, polyhydramnios and ascites from 21 to 26 weeks. Thoracoamniotic shunting was performed successfully at 26 weeks with resolution of the cystic mass. At 37 weeks a male infant was born vaginally weighing 3,210 g with Apgar scores of 5 and 10. Resection of the mass was performed uneventfully on day 3. The infant is currently 22 months of age, asymptomatic and in good condition.     

Cystic adenomatoid malformation of the lung: an obstetric and ultrasound perspective.

Between 1986 and 1990, seven women were found to have fetuses with an abnormal antenatal ultrasound appearance of the chest and lungs suggestive of congenital cystic adenomatoid malformation of the lung (CCAM). All were confirmed by pathology. Cystic spaces were only visible in those women with macrocystic disease (Stocker types 1 and 11) whilst increased echogenicity of affected lung tissue and mediastinal deviation were seen in all types. Cystic adenomatoid malformation of the lung was also found in one woman with a raised maternal serum alpha-fetoprotein. The appearances are distinctive, and should be sought in all cases of polyhydramnios, fetal ascites and raised maternal serum alpha-fetoprotein.     

Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience

Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. METHOD: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. RESULTS: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. CONCLUSION: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.     

Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung

OBJECTIVE: Our purpose was to review the management and outcome of pregnancies with a prenatal diagnosis of fetal congenital cystic adenomatoid malformation of the lung (CCAM). STUDY DESIGN: A retrospective review was performed of all cases since 1995 with a prenatal diagnosis of fetal CCAM from the sole tertiary perinatal referral center in Western Australia. RESULTS: Twenty-one pregnancies with CCAM were identified. The gestational age at diagnosis was <22 weeks in 86% of cases. Macrocysts were seen in 76% of cases during ultrasound examination. Seventeen pregnancies continued until term. Regression of the sonographic appearances was observed in 19% of cases. Fetal hydrops complicated two cases. One neonate died within 24 hours of delivery because of pulmonary hypoplasia. Twelve children have required pulmonary lobectomies. No adverse sequelae are evident in surviving children. CONCLUSION: Most cases of prenatally diagnosed CCAM have had a good outcome. This review has positively influenced the counseling of women with this diagnosis.     

Early prenatal diagnosis of congenital cystic adenomatoid malformation of the lung (Stocker's type I); a case report

A congenital cystic adenomatoid malformation was diagnosed by ultrasound examination at 20 week's gestation. The entire right lung was cystic and elements of poor prognosis such as hydrops fetalis and polyhydramnios were present. After verification of the karyotype, abortion was performed and autopsy confirmed prenatal findings and Stocker's type I. Cystic congenital adenomatoid malformation of the lung is a rare form of congenital pulmonary disease. Our case shows that this malformation can be accurately diagnosed during the midtrimester of pregnancy by ultrasound examination. Ultrasound examination permits an evaluation of the three types described by Stocker and may reveal certain lesions associated with poor prognosis, such as anasarca or polyhydramnios. Also, it offers the possibility to save some fetuses by surgical decompression in the immediate postnatal period, or to terminate earlier pregnancies by abortion.     

Congenital cystic adenomatoid malformation in the fetus: a hypothesis of its development

We present a case of congenital cystic adenomatoid malformation of the lung diagnosed at 34 weeks of gestation in the setting of polyhydramnios. The fetus had CCAM in the L lung, with mediastinal shift to the right and ascites. The neonate underwent drainage of cysts and subsequent left lung lobar resection with improvement in respiratory function. The pathology of CCAM is reviewed in detail. We speculate the role of alcohol as a teratogen through retinoic acid at 8-10 weeks of gestation when fetal lungs are actively developing.     

Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations

OBJECTIVE: To determine whether fetuses that underwent thoracoamniotic shunt placement for treatment of pleural effusion (PE) or macrocystic congenital cystic adenomatoid malformation (CCAM) have an improved outcome as compared with an untreated population. METHODS: A retrospective review from a single tertiary center was performed using thoracoamniotic shunt placement to treat PE or macrocystic CCAM between 1998 and 2001. Thoracoamniotic shunts were used on 26 occasions in 19 pregnancies. RESULTS: The average gestational age at the diagnosis of PE and CCAM was 22 + 4 and 20 + 0 weeks, respectively. Shunts were offered in pregnancies complicated by hydrops or at significant risk for pulmonary hypoplasia. Shunts were placed at 26 + 2 weeks (average) and 23 + 1 weeks (average) in the PE and CCAM groups, respectively. In CCAM patients, the mean pre- and postshunting mass volumes were 50.5 and 25.7 cm(3), representing a 51% reduction in mass volume following shunt placement. In the PE group, the average delivery age was 33 + 5 weeks, with an average shunt placement to delivery time of 7 + 3 weeks. In the CCAM group, the average delivery was 33 + 3 weeks, with an average shunt placement to delivery time of 10 + 2 weeks. The postnatal survival rates were 67% (6/9) and 70% (7/10) in the PE and CCAM groups, respectively. CONCLUSIONS: (1) Thoracoamniotic shunts should be considered as a treatment option for selected PE or macrocystic CCAM fetuses with hydrops or a significant risk for pulmonary hypoplasia; (2) the neonatal survival with shunting was similar for PE and CCAM groups and was improved as compared with literature reports, and (3) fetuses with CCAM presented earlier with hydrops than those with PE. Successful shunting resulted in a prolongation of pregnancy into the 3rd trimester in both groups.