Prenatal diagnosis of cystic adenomatoid malformation of the lung

Cystic adenomatoid malformation of the lung (CAM) is an uncommon malformation in which normal tissue is replaced by cysts of various sizes. This malformation may be diagnosed prenatally with ultrasound by visualization of cystic masses in the fetal chest, which may occur in association with fetal hydrops. Three cases of fetal hydrops secondary to cystic adenomatoid malformation of the lung are described; in one case cystic lesions in the chest were clearly demonstrated.     

胎儿先天性肺囊腺瘤样畸形超声声像图特征及预后分析

目的：探讨胎儿先天性肺囊腺瘤样畸形（CCAM）超声声像图特征,为胎儿临床结局评估提供依据。方法分析21例产前超声诊断为胎儿CCAM的超声声像图特征,并与产后CT、手术及随访结果进行对照。结果（1）21例胎儿CCAM产前超声声像图特征：CCAMⅠ型2例（10%,2/21）,CCAMⅡ型13例（62%,13/21）,CCAMⅢ型6例（28%,6/21）;位于一侧胸腔20例（95%,20/21）,双侧胸腔1例（5%,1/21）;首次检出胎儿CCAM的孕周为18＋4~25＋4周,中位孕周为22＋6周;胎儿CCAM体积与头围比（CVR）＜1.6者16例（76%,16/21）,CVR＞1.6者5例（24%,5/21）;动态观察病灶达最大体积后维持不变8例（38%,8/21）,达最大体积后逐渐缩小13例（62%,13/21）（其中消失5例）。（2）合并症：19例（90%,19/21）出现伴发症状或合并其他结构畸形,均伴发纵隔移位,羊水过多6例,膈疝1例,肺隔离症1例,胎儿水肿1例。（3）随访结果：16例产前超声检查与出生后胸部CT结果相符,其中9例经手术及病理证实;1例引产者与尸体解剖病理诊断符合;余4例产前超声动态复查提示肿块缩小,无合并症,出生后未行检查及治疗。（4）临床预后：19例（90%,19/21）预后良好,1例合并胎儿水肿者孕妇选择性终止妊娠,1例合并膈疝者出生后1d死亡。结论产前超声检查是诊断胎儿肺囊腺瘤的首选和可靠的方法,产前动态超声检查是评估及预测胎儿CCAM预后的关键,单纯CCAM预后良好。     

胎儿肺囊腺瘤畸形的产前超声诊断与分型

目的探讨胎儿肺囊腺瘤畸形(CAML)产前超声分型特征及临床价值.方法 对41例产前超声诊断为肺囊腺瘤畸形胎儿的超声声像图表现进行回顾性分析并分型,41例均随访至引产或出生.结果 (1)肿瘤部位:41例胎儿肺囊腺瘤畸形发生在左侧22例,右侧15例,双侧4例.(2)产前超声分型诊断:Ⅰ型3例,Ⅱ型14例,Ⅲ型24例.(3)病理诊断:41例中32例引产,尸检病理诊断为肺囊腺瘤29例,3例隔离肺产前超声误诊为肺囊腺瘤Ⅲ型.(4)41例中9例随访至出生(3例肿块逐渐减小消失,6例出生后CT证实为肺囊腺瘤),产前超声分型与产后诊断均符合.(5)产前超声诊断胎儿肺囊腺瘤畸形与病理诊断和出生儿随访诊断符合率为92.7%(38/41),误诊率为7.3%(3/41).结论 胎儿肺囊腺瘤畸形产前超声诊断及分型诊断准确率高,是产前早期发现胎儿肺囊腺瘤畸形的首要检查方法,有重要临床意义,但隔离肺易误诊为肺囊腺瘤Ⅲ型,应注意鉴别.     

Prenatal diagnosis of bilateral cystic adenomatoid malformation of the lung

Bilateral cystic adenomatoid malformation of the lung (CAML), solid type (Stocker III), is a rare malformation that we observed sonographically in two fetuses at 23 weeks. The ultrasonic image showed an echogenic mass compressing the heart and occupying the whole thoracic cavity. Massive ascites, probably as a result of heart failure, was apparent. The prognosis is fatal. Prenatal diagnosis enables termination of pregnancy and saves the mother from invasive procedures in case of fetal distress. In unilateral, more favorable lesions, the managing physician is prepared for respiratory distress of the newborn and an emergency operation.     

产前超声在诊断先天性肺囊腺瘤畸形及预后分析中的价值

目的:探讨产前超声在胎儿先天性肺囊腺瘤畸形(congenital cystic adenomatiod malformation of the lung,CCAM)诊断中的价值及临床意义。方法:对产前超声疑诊为CCAM的30例胎儿的声像图特征进行回顾性分析,并随访其临床结果。结果:超声疑诊的30例CCAM胎儿中,单侧病变为28例,双侧病变为2例;CCAMⅠ型6例,Ⅱ型6例,Ⅲ型18例;有8例孕妇选择引产,余22例活产;所有活产病例产后随访至12个月,婴儿均无异常。结论:超声检查是发现胎儿肺内CCAM病灶的重要方法,在发现该病灶后,可应用超声观察病灶变化,单纯CCAM且不合并胎儿水肿的患儿出生后预后较好。     

Antenatal ultrasound detection of cystic adenomatoid malformation of lung: report of a case and review of the recent literature

We have described a case of cystic adenomatoid malformation of the lung, detected before birth. The ultrasound findings demonstrated in this case correlated well with both the radiographic appearance of the lesion and with the gross pathology. Though we do not feel that the ultrasonic findings described are specific for this entity, they are believed to be characteristic of at least one type of cystic adenomatoid malformation. The prognostic significance of associated polyhydramnios and fetal anasarca is discussed. The importance of detecting this lesion antenatally is highlighted in this case in which the malformation would most likely have gone undetected in the perinatal period were it not for the antenatal ultrasonic findings.     

Prenatal diagnosis and fetal outcome of cystic adenomatoid malformation of the lung: case report and historical survey.

Diagnostic ultrasound allows prenatal diagnosis of cystic adenomatoid malformation of the lung from the second trimester onwards throughout pregnancy. If the diagnosis is made before the 24th week of pregnancy, counselling often results in termination of pregnancy. Whether or not this attitude is in agreement with good medical practice is discussed on the basis of our own experience and a review of the literature.     

In utero resolution of microcystic congenital cystic adenomatoid malformation after prenatal betamethasone therapy: A report of three cases and a literature review

Fetal congenital cystic adenomatoid malformation (CCAM) can progress to nonimmune hydrops, and the mortality rate of CCAM with hydrops is reported to be nearly 100%. We describe three microcystic CCAM cases in which the fetal condition improved after maternal betamethasone therapy. The median gestational age at steroid administration was 23 5/7 weeks' gestation. The CCAM decreased in size in all cases. Our series showed a 100% hydrops resolution rate (2/2) and a 100% survival rate (3/3). Our experience suggests the efficacy of betamethasone treatment on fetuses with microcystic CCAM who have fluid collection or are at risk of developing hydrops. © 2014 Wiley Periodicals, Inc. J Clin Ultrasound 43 :451–457, 2015     

Early prenatal diagnosis of type III bilateral congenital cystic adenomatoid malformation of the lung.

We present a case of type III fetal cystic adenomatoid malformation with bilateral total involvement of the lungs. The characteristic sonographic findings are echogenic homogeneous lung mass, fetal ascites and placentomegaly. The early diagnosis at 16 weeks' gestation allows the elective termination of pregnancy because of the likely fatal prognosis. To our knowledge, this is the earliest reported case of congenital cystic adenomatoid malformation of the lung that has been detected prenatally.     

先天性肺囊性腺瘤样畸形的CT表现

先天性肺囊性腺瘤样畸形（CCAM）是一种少见的肺发育畸形，临床有急性或反复性肺感染，窒息，进行性呼吸困难，常误诊为先天性肺囊肿及支气管扩张等疾病。本文搜集经手术病理证实的CCAM7例，回顾性分析其CT表现特征，旨在提高对本病的认识。     

Presumptive intrahepatic congenital cystic adenomatoid malformation.

Congenital cystic adenomatoid malformation (CCAM) and extralobar pulmonary sequestration (EPS) are 2 rare types of bronchopulmonary malformations that typically appear in infancy and childhood. A CCAM is a hamartomatous lesion of the lung, thought to be the result of the cessation of bronchiolar maturation with overgrowth of mesenchymal elements. An EPS is a discrete mass of lung tissues separate from the tracheobronchial tree and is supplied by a systemic vascular source (usually aortic). As well, it forms outside the normal pleural investment. Conran and Stocker analyzed 50 cases of EPS and found that 50% (23 of 46) were associated with a coexistent CCAM. Rare cases of intra-abdominal EPS (IEPS) have been reported in the literature, and in these cases, CCAMs commonly occur within the pulmonary sequesterant. We present an interesting case of CCAM manifesting within the liver of a patient treated prenatally for a large pulmonary type II CCAM and review the reported cases of IEPS with CCAM.     

肺先天性囊性腺瘤样畸形4例临床病理分析

目的 探讨肺先天性囊性腺瘤样畸形（CCAM）的临床病理特征。方法 对4例经手术、病理证实的CCAM病例进行临床病理分析，并系统复习文献，讨论其病理分型、临床特点、治疗和预后及其发病机制。结果 4例均累及单侧单叶肺，其中CCAMⅠ型3例，Ⅱ型1例。除1例为胎儿行引产外，其余3例行部分肺切除或肺叶切除术。结论 CCAM是一种少见的肺先天性畸形，影像学是发现该病的有效手段，确诊有赖于病理检查。CCAM患者有一定的恶变风险，手术切除病变肺组织是有效的治疗方法。     

超声对胎儿肺囊性腺瘤样畸形的诊断价值

肺的良好发育是胎儿出生后能够成活的决定因子.实际上,肺未发育成熟和肺发育不良及其合并症是孕24周以前胎儿不能成活的主要原因.肺发育不良将导致产后新生儿呼吸功能不全,常威胁生命.