Prenatal diagnosis of cystic adenomatoid malformation of the lung

Two cases of congenital cystic adenomatoid malformation of the lung (CCAML) are described. In the light of recent literature the prenatal diagnosis and management are discussed.     

Prenatal diagnosis of congenital cystic adenomatoid lung malformation: case report and review of the literature

Congenital cystic adenomatoid malformation of the fetal lung is an extremely rare developmental abnormality characterized by excessive overgrowth of the terminal respiratory bronchioles at the expense of the saccular spaces. We present a case of a 33-year-old, gravida 2, para 1, woman with congenital cystic adenomatoid lung malformation-type II diagnosed by ultrasound at the 20th week of gestation. On the right side of the chest an area with a maximum diameter of 18.5 mm and with small cystic lesions was recognized. The maximum diameter of the cysts was 0.5 cm. There were no other fetal abnormalities. The pregnancy was terminated and the postmortem examination confirmed the ultrasonographic findings. The cysts had the appearance of bronchiolus-like structures and were lined with cuboidal and columar epithelium. Distended alveoli were present, while the airways were normal in structure. No other congenital anomalies were found. In conclusion, in this study we describe the ultrasonographic and pathologic findings of an unusual case of congenital cystic adenomatoid malformation of the fetal lung.     

Outcome of congenital cystic adenomatoid malformation of the lung after antenatal diagnosis.

OBJECTIVE: We evaluated the outcome of fetuses diagnosed with having congenital cystic adenomatoid malformation (CCAM) on ultrasonographic examination and managed conservatively. METHODS: A retrospective study of 19 cases of CCAM diagnosed antenatally in our hospital was conducted between 1990 and 2001. Complete clinical information was available for all patients, with a mean follow-up of 62 months. RESULTS: The median gestational age at which CCAM was diagnosed was 23 weeks and there were eight live births. With conservative postnatal management, seven neonates had no major complications and one developed bronchopneumonia. CONCLUSION: Taken together, the findings of the present study and a review of the literature strongly support the conservative management of selected neonates with CCAM.     

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。     

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.     

Congenital cystic lesions of the lung: congenital cystic adenomatoid malformation and bronchopulmonary sequestration

Congenital cystic lesions of the lung in fetuses are rare. The most common malformations of the lower respiratory tract are congenital cystic adenomatoid malformation and bronchopulmonary sequestration. With the increased use of obstetric ultrasound, cystic lung lesions are detected more often antenatally, which allows for proper planning of peripartum and neonatal management. This article discusses a range of diagnostic and management options.     

Waxing and waning hyperinflation in congenital cystic adenomatoid malformation of the lung.

This report describes a newborn with respiratory distress, waxing and waning left lung hyperinflation, and pulmonary hemorrhage. This atypical presentation of congenital cystic adenomatoid malformation of the lung has not been previously reported, posed a major diagnostic problem, and delayed potentially lifesaving surgery.     

Prenatal diagnosis of fetal congenital cystic adenomatoid malformation of the lung using three-dimensional ultrasound: Comparison between the 20th and 21st centuries

ObjectiveCongenital cystic adenomatoid malformation of the lung (CCAML) is one of the most common lung lesions diagnosed prenatally. In order to compare the trends and improvements of prenatal diagnosis of CCAML, we herein retrospectively reviewed our cases of fetal CCAML detected by three-dimensional ultrasound (3-D US) between two centuries.Materials and MethodsWe reviewed our computer database of prenatal diagnosis of CCAML in National Cheng Kung University Hospital from October 1994 to November 2011. All of the fetuses were initially scanned by two-dimensional (2-D) US to locate the region-of-interest (ROI). Then, the 3-D probe was used to scan all of the ROI systematically and mechanically, and the images were stored in the laser discs for further 3-D visualization and reconstruction. To compare the characteristics at prenatal diagnosis of CCAML between the 20th and 21st centuries in our hospital, Chi-square tests were undertaken. A p value <0.05 was considered as statistically significant.ResultsIn total, 58 fetuses with CCAML were depicted by 3-D US in utero (12 cases were diagnosed in the 20th century and 46 cases in the 21st century). The ranges of gestational age at prenatal diagnosis of CCAML by 3-D US in the 20th century were between 15 and 36 weeks (mean = 24 weeks), and were between 16 and 31 weeks (mean = 22 weeks) in the 21st century. Moreover, nine cases (75%) were diagnosed at the second trimester in the 20th century, whereas 44 cases (96%) were diagnosed at the second trimester in the 21st century.ConclusionThe advancement of 3-D US has remarkable advantages in adding novel visual depiction of a 3-D lesion of a 3-D fetus in 3-D US after reconstruction, and thus assists substantially in the prenatal diagnosis and genetic consultation of CCAML. Furthermore, the trend analysis in this series showed a significantly earlier gestational age at prenatal diagnosis of CCAML in the 21st century than that in the 20th century.     

Sonographic diagnosis of cystic adenomatoid malformation in-utero

Routine ultrasound examination at 30 weeks gestation revealed an intrapulmonary cystic mass in an otherwise normal fetus. Following delivery at term, the diagnosis of congenital cystic adenomatoid malformation of the right lung was confirmed, and an elective right middle lobectomy successfully performed at nine days of age.     

Evaluating the association between congenital cystic adenomatoid malformation of the lung and abdominal wall laxity

We report a male fetus with congenital cystic adenomatoid malformation of the lung, who was treated by laser with resolution of hydrops. Postnatally, the infant had the typical abdominal wall laxity and undescended testes characteristic of prune-belly syndrome. The sonographic diagnosis of cystic adenomatoid malformation was confirmed histologically after postnatal resection. The relationship between cystic adenomatoid malformation and prune-belly syndrome is evaluated through analysis of the present and previously reported cases. We show that there does appear to be a true association between cystic adenomatoid malformation and prune-belly syndrome. We also, however, demonstrate that the traditional explanation (cystic adenomatoid malformation-related hydrops causes ascitic abdominal distension resulting in prune-belly syndrome) is unlikely to be straightforwardly correct.     

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.     

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.     

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.