The prenatal ultrasonic character and postnatal follow-up of 227 microcystic and macrocystic congenital cystic adenomatoid malformations.

The objective of our study was to compare microcystic and macrocystic congenital cystic adenomatoid malformation (CCAMs) through prenatal characteristics, perinatal outcome, postnatal management and development after consultation in our prenatal diagnosis centre. We conducted a retrospective cohort study of 227 cases prenatally diagnosed as CCAM in our hospital within three years. One hundred and eighty-one cases continued their pregnancy and 46 pregnancies were terminated. One hundred and fifteen of 227 cases were microcystic and 112 were macrocystic. The prenatal ultrasound characteristics of two types showed no statistically significant differences, despite that CVR of macrocystic CCAMs was mildly greater than microcystic. None of recorded cases received surgical intervention in utero. Compared with healthy foetuses, CCAMs presented with higher percentage of polyhydramnios and male foetuses, larger amount of postpartum blood loss and longer duration of NICU hospitalisation. Compared with 46 legal abortions, continued pregnancy cases had a smaller CVR, less polyhydramnios, cardiac shift and hydrops. One hundred and seventy-nine cases were followed up after birth and 174 babies were asymptomatic. Thirty one cases received successful surgical resection. In conclusion, the prenatally diagnosed CCAMs have a good short-term prognosis and conservative management is a reasonable option in asymptomatic fetuses. Microcystic and macrocystic CCAMs both presented similar prenatal courses and perinatal prognoses. Conservative management, prenatally and postnatally, could be accepted in selected cases.Impact statementWhat is already known on this subject? Congenital cystic adenomatoid malformation is a well-known developmental abnormality of the lung. The prognosis for most CCAMs is good, but the prenatal and postnatal management remains controversial.What do the results of this study add? This study is one of the largest case cohorts to conclude that microcystic and macrocystic CCAMs both presented similar prenatal courses and perinatal prognoses. Conservative management is a reasonable option in asymptomatic babies.What are the implications of these findings for clinical practice and/or further research? A comprehensive assessment by an experienced multidisciplinary team is necessary to forbid blinding abortions of CCAMs. Conservative management prenatally and postnatally could be accepted in most asymptomatic cases.

Prediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.

OBJECTIVES: To identify, in fetuses with a congenital lung malformation (CLM), prenatal predictors of the need for postnatal respiratory support and the need for surgery by calculating the CLM volume ratio (CVR), and to evaluate the concordance between the prenatal appearance and the postnatal type of CLM. METHODS: This was an analysis of prenatal, perinatal and postnatal data from fetuses diagnosed with a CLM at the Erasmus University Medical Center - Sophia Children's Hospital in Rotterdam, The Netherlands, between January 2007 and December 2016. For all included fetuses, CVR was measured retrospectively on stored ultrasound images obtained at 18 + 1 to 24 + 6 weeks (US1), 25 + 0 to 29 + 6 weeks (US2) and/or 30 + 0 to 35 + 6 weeks' gestation (US3). Postnatal diagnosis of CLM was based on computed tomography or histology. Primary outcomes were the need for respiratory support within 24 h and surgery within 2 years after birth. RESULTS: Of the 80 fetuses with a CLM included in this study, 14 (18%) required respiratory support on the first postnatal day, and 17 (21%) required surgery within 2 years. Only the CVR at US2 was predictive of the need for respiratory support, with a cut-off value of 0.39. Four of 16 (25%) fetuses which showed full regression of the CLM prenatally required respiratory support within 24 h after birth. The CVR at US1, US2 and US3 was predictive of surgery within 2 years. Overall, the prenatal appearance of the CLM showed low concordance with the postnatal type. Prenatally suspected microcystic congenital pulmonary airway malformation (CPAM) was shown on computed tomography after birth to be congenital lobar overinflation in 15/35 (43%) cases. Respiratory support within 24 h after birth and surgical resection within 28 days after birth were needed in all cases of macrocystic CPAM. CONCLUSIONS: CVR can predict the need for respiratory support within 24 h after birth and for surgery within 2 years. Regression of a CLM prenatally does not rule out respiratory problems after birth. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. - Legal Statement: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Fetal Congenital Pulmonary Airway Malformation: The Role of an Objective Measurement of Cardiomediastinal Shift.

OBJECTIVE: To examine the relationship between cardiomediastinal shift angle (CMSA) and adverse perinatal outcomes and hydrops in cases of congenital pulmonary airway malformation (CPAM). STUDY DESIGN: This retrospective study evaluated CPAM cases referred to our institution from 2008 to 2015. The primary outcome was a composite score for adverse perinatal outcome. CMSA was measured for each case and evaluated for its association with the primary outcome. The prediction accuracy of CMSA for adverse perinatal outcome was assessed using receiver operator characteristic (ROC) curves. RESULTS: Eighteen (21.2%) of the 85 cases experienced an adverse perinatal outcome. Increases in CMSA were associated with adverse perinatal outcomes and hydrops in bivariate analyses. Adjusted analyses found each 10-degree increase in CMSA to be associated with increased odds of an adverse perinatal outcome (adjusted odds ratio [aOR] 2.2, 95% confidence interval [CI]: 1.4-3.3) and hydrops (aOR 3.0, 95% CI: 1.5-6.1). CMSA performed well and was comparable to CPAM volume ratio in predicting adverse perinatal outcomes (area under the curve 0.81 and 0.84, respectively). CONCLUSION: We describe a novel measurement of mediastinal shift in cases of CPAM and its relationship with adverse perinatal outcomes and hydrops. These findings may shape the evaluation and management of CPAMs, improve our understanding of their prognosis, and influence patient counseling.

[Fetal therapy--evaluation of intrauterine therapy in congenital cystic adenomatoid malformation of the lung (CCAM)]. / Terapia plodu--ocena skutecznosci leczenia wewnatrzmacicznego wrodzonego zwyrodnienia gruczolakowato-torbielowatego pluc (CCAM).

OBJECTIVES: The aim of the study was to evaluate the efficiency of intrauterine treatment of large cysts in fetal lungs using thoracoamniotic shunts. MATERIAL AND METHODS: Our observational retrospective study was carried out on a series of 8 fetuses who under went thoracoamniotic shunting after sonographic statement of large macrocystic lesions in the lungs at the Department of Gynecology Fertility and Therapy of the Fetus, Polish Mother's Research Institute, between 2009-2014. RESULTS: Mean gestational age at shunt insertion was 26.6 (range 18-33) weeks. Marked mediastinal shift in the echocardiographic examination was observed in all of the investigated cases. Five fetuses had polyhydramion, with 4 hydropic cases. Out of the remaining 4 fetuses without impaired cardiac function, 3 had very large lesions at initial presentation and 1 had a lesion that was rapidly increasing in size. Shunt insertion was successful in all cases. Only one patient went into premature labor (at 36 weeks of gestation). Mean gestational age at delivery was 38.2 weeks. Cesarean section was necessary in the half of the patients due to obstetric complications. All newborns underwent resection of the lesions. Three of them were operated in the first month after birth. The rest of the operations were postponed. Prenatal diagnosis of congenital cystic adenomatoid malformations was confirmed by pathologists in all cases. CONCLUSION: Intrauterine therapy of macrocystic lesions in fetal lungs enables to achieve good perinatal outcome It needs to be considered in every case of a fetus with developing impaired cardiac function.

[Successful intrauterine therapy in a fetus with a large lung cyst in the third trimester of pregnancy--case report]. / Skuteczna interwencja wewnqtrzmaciczna u plodu z olbrzymiq torbiela pluca w trzecim trymestrze ciazy--opis przypadku.

Congenital cystic adenomatoid malformation is a rare disorder of the respiratory system which occurs with an incidence of 1/25,000-35,000. Depending on the size of the cysts, CCAM is classified into microcystic and macrocystic. Very large lesions carry a significant risk of causing both, pulmonary hypoplasia due to compression of the lung tissue and fetal hydrops, probably due to impaired cardiac function as a result of mediastinal shift and compression of the vena cava. In this report, we present a case of a fetus with prenatally diagnosed large cyst of the left lung. Despite advanced gestational age, the baby was treated by thoracoamniotic shunting owning to developing impaired cardiac function and polyhydramion. Spectacular effects of the therapy have been achieved.

Management of prenatally diagnosed lung lesions.

Prenatal diagnosis provides insight into the in utero evolution of fetal thoracic lesions such as congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestration (BPS), or hybrid lesions. Serial sonographic study of fetuses with thoracic lesions has helped define the natural history of these lesions, determine the pathophysiologic features that affect clinical outcome, and formulate in utero and postnatal management based on prognosis.

Intradiaphragmatic hybrid lesion in an infant: case report.

Hybrid lesions have elements of both congenital cystic adenomatoid malformation and bronchopulmonary sequestration. We report an unusual case of an infant treated for an intradiaphragmatic hybrid lesion. Although computed tomography remains the criterion standard imaging examination for planning the operation, the exact localization of such lesions may be discovered only at surgical exploration.

Factors associated with partial and complete regression of fetal lung lesions.

OBJECTIVE: A decrease in the volume of congenital pulmonary malformations (CPM) can be observed on prenatal ultrasonography, but the underlying mechanism for this phenomenon is unknown. Our objective was to identify factors associated with the prenatal reduction in size of cystic and/or hyperechoic lung lesions. METHODS: This was a retrospective study of cases with a prenatal diagnosis of hyperechoic and/or cystic lung lesion. The extent of reduction in lesion size was calculated from ultrasound measurements. Clinical, ultrasound, radiological and histological data were tested for their relationship with prenatal CPM reduction. RESULTS: In a 4-year period, 36 patients were referred with a cystic and/or hyperechoic fetal lung lesion diagnosed at a mean gestational age of 23.4 weeks. The lesions were cystic in 16 cases (44%), hyperechoic in 12 (33%) and both in eight (22%). The malformation was no longer visible before birth (apparent disappearance) in nine cases (25%), shrank by 18-90% in 15 (42%) and did not reduce in 12 (33%). Findings on postnatal computed tomography were always abnormal. Isolated hyperechoic lesions were significantly more likely to shrink in utero. The mean reductions were 79%, 35% and 19%, for isolated hyperechoic, cystic and mixed lesions, respectively (P=0.001). Only 8% of hyperechoic lesions demonstrated no volume reduction, as compared to 50% and 42% of cystic and mixed lesions, respectively (P=0.03). Greater gestational age at birth was also associated with a decrease in the incidence of malformations (P=0.02). In cases that underwent surgery, hyperechoic lesions were linked to a variety of pathological diagnoses, whereas cystic lesions were all described histologically as congenital cystic adenomatoid malformations. CONCLUSIONS: Prenatal size reduction of fetal lung malformations is associated with isolated hyperechogenicity and greater gestational age at birth. This might result from the resumption of normal lung development after local disruption of lung growth.

Bronchopulmonary foregut malformations presenting as mass lesions in children: spectrum of imaging findings.

Bronchopulmonary foregut malformations are a heterogeneous but interrelated group of abnormalities that may contain more than one histologic feature. Familiarity with the presentation and imaging features of bronchopulmonary foregut malformations presenting as a congenital mass or mass-like lesion is important. Moreover, imaging plays a central role in the evaluation of these lesions since, when symptomatic, clinical features are usually nonspecific. With imaging, the presence of and features of the lesion can be determined, facilitating appropriate management to prevent the potential complications including infection, respiratory compromise and, very rarely, malignancy.

Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls.

BACKGROUND: Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association. METHODS: Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors' institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively. RESULTS: In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case. CONCLUSIONS: Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.

Genetic analysis of congenital cystic adenomatoid malformation reveals a novel pulmonary gene: fatty acid binding protein-7 (brain type).

The pathogenesis of congenital cystic adenomatoid malformation (CCAM) is unknown and its natural history is unpredictable. Fatty acid binding protein-7 (FABP-7) has been previously described in brain and breast development, but never before in the lung. We investigate gene expression in CCAM, and hypothesize that CCAM results from an aberration in the signaling pathway during lung development. Under IRB approval, tissue specimens of fetal CCAM, fetal control, postnatal CCAM, and postnatal control were examined and microarray analysis was performed. Candidate differentially expressed genes were selected with log-odds ratio (B) >0 and false discovery rate <0.05. Validation of differential expression was achieved at the RNA and protein levels. FABP-7 was underexpressed in fetal CCAM compared with fetal lung in both the microarray and by RT-PCR. Findings were duplicated by Western Blot analysis and immunohistochemistry. This is the first description of FABP-7 in the human lung. Decreased expression of FABP-7 in fetal CCAM compared with normal fetal lung at both the RNA and protein levels suggests FABP-7 may have a role in pulmonary development and in the pathogenesis of CCAM.

Ex-Utero Intrapartum Treatment (EXIT) in a giant case of Congenital Cystic Adenomatoid Malformation (CCAM) of the lung.

Congenital Cystic Adenomatoid Malformations (CCAM) of the lung are a relatively rare anomaly. The majority of cases do not require any antenatal or perinatal interventions. However, selected cases need special and intensified management. This article discusses a case of a CCAM of the left pulmonary lobe covering 2/3 to 3/4 of the thoracic volume, causing mediastinal shift and hydrops. The enormous size required the planning of an EXIT (Ex Utero-Intrapartum Treatment) procedure, which was performed when the trial of intraoperative ventilation of the lungs was unsuccessful. A thoracotomy and resection of the left upper lobe were performed with the fetus on feto-placental circulation. After resection and closure of the thoracotomy, improvement of lung ventilation allowed the delivery of the child. The child and mother were discharged in a healthy condition. We conclude that the EXIT procedure is a feasible and potentially life-saving method for select cases of prenatally detected CCAM.

[Congenital cystic adenomatoid malformation of the lung--report of two cases].

Congenital cystic adenomatoid malformation (CCAML) is a rare anomaly of the fetal lung which can be diagnosed prenatally by ultrasound. We present two cases of CCAML with different pregnancy outcome diagnosed at 17 and 24 weeks gestation, respectively, and discuss the major aspects of the obstetrical and therapeutical management.

Spontaneous regression of congenital cystic adenomatoid malformation of the lung: longitudinal examinations by magnetic resonance imaging.

We report a case of large cystic adenomatoid malformation of the lung (CCAM), which occupied almost the entire left lung with a prominent mediastinal shift at 24 weeks of gestation. The volume of the lesion, determined by magnetic resonance imaging (MRI), was 27.0 cm3. Subsequent MRI and ultrasound examinations revealed a spontaneous resolution of the lesion at 32 and 36 weeks of gestation without a mediastinal shift, when the lesion volume was 12.8 cm3 and 5.6 cm3, respectively. At 37 weeks of gestation, a mature male baby weighing 2638 g with an Apgar score of 7 was delivered by elective cesarean section. A lobectomy of the left upper lobe of the lung was carried out at 3 days of age, due to an enlargement of the CCAM after birth.

Postnatal spontaneous resolution of congenital cystic adenomatoid malformations.

PURPOSE: Although antenatal resolution of congenital cystic adenomatoid malformations (CCAMs) is well documented, complete spontaneous postnatal resolution is rare, its existence even questioned by some. METHODS: All cases of antenatally diagnosed CCAMs over 7 years were retrospectively reviewed. Inclusion criteria were the following: (1) antenatal diagnosis of CCAM, (2) persistence on postnatal imaging, and (3) subsequent spontaneous resolution on postnatal imaging. RESULTS: Of 56 antenatally diagnosed CCAMs, 2 patients were identified. Both had macrocystic lesions. In case 1, the CCAM that filled the hemithorax on antenatal ultrasound was smaller on postnatal chest radiograph and disappeared by age 37 months on computed tomography. Case 2 had significant reduction of the CCAM at birth (persistence of the lesion on initial radiographs and ultrasound was documented). By 5 months, the lesion was not evident on computed tomography. Neither case was associated with symptoms, polyhydramnios, hydrops, or other abnormalities. CONCLUSIONS: In patients with an antenatal diagnosis of CCAM, spontaneous resolution may occur postnatally in 4% of cases. Significant reduction in CCAM size may portend possible disappearance and therefore warrants an observational period before resection.

Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral chest: common origin?

Extralobar pulmonary sequestration (ELS) and congenital cystic adenomatoid malformation have been reported to coexist in several variations. This suggests a common embryologic origin. A 6-month-old boy presented with a history of recurrent pneumonias. The patient was diagnosed with a right lower lobe congenital cystic adenomatoid malformation (CCAM) and a left lower lobe ELS/CCAM. The diagnosis was made with the aid of a multidetector computed tomography (CT). Three-dimensional CT reconstruction showed the presence of a right lower lobe CCAM, a left lower ELS with an aberrant arterial supply from the celiac axis, and possible venous drainage into the right CCAM. The patient underwent a right thoracotomy. Intraoperatively, the lesions were discovered to be connected by a band of tissue. The right lower lobe CCAM and the left ELS were removed from the right chest. Histologic analysis confirmed the presence of a CCAM within the right lower lobe. The ELS had involvement of a type II CCAM within the sequestration. The connection between the right CCAM and left ELS/CCAM showed an anomalous conducting airway, anomalous vein, and anomalous artery connecting the 2 lesions. The authors present the first case of a CCAM connected to an ELS/CCAM in the contralateral hemithorax. The unique anatomic configuration of these lesions suggests a common embryologic origin of ELS and CCAM.

Prenatal diagnosis of congenital cystic adenomatoid malformation.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare pulmonary lesion. The prognosis is variable ranging from perinatal death to spontaneous in utero regression, with no neonatal morbidity. We present a case of CCAM diagnosed at 23 weeks' gestation using results of prenatal sonograms. Both prenatal Two-Dimensional and Three-Dimensional sonograms revealed multiple cystic lesions at the right lower lung field. Regular serial antenatal sonograms revealed the fetus had persistent right lower lung multicyst lesions but had no hydrops fetalis or associated congenital anomalies. The results of the antenatal 50 g glucose diabetic screen at 24 weeks' gestation were normal. A live male baby was vaginally delivered smoothly at 40 weeks' gestation. Body weight was 4170 g. Apgar scores were 7 at 1 minute and 8 at 5 minutes. The newborn had no signs of respiratory distress at birth. Postnatal chest computed tomography (CT) also revealed multiple fluid-filled cysts in the right lower lobe compatible with congenital cystic adenomatoid malformation. Because of the potential risk of perinatal death, repeated lung infection and malignant change of CCAM, the newborn received right lower lung lobectomy 1 week after delivery. Pathology confirmed the diagnosis of CCAM type II. The recovery was smooth postoperatively and the baby still receives regular follow-up. We emphasize the importance of prenatal diagnosis of CCAM and early removal of the congenital cystic lesions of the lung to achieve a good outcome.