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Lung ultrasound (LU) is a noninvasive, bedside imaging technique that is attracting growing interest in the evaluation of neonatal respiratory diseases. We conducted a nationwide survey of LU usage in Italian neonatal intensive care units (NICUs).A structured questionnaire was developed and sent online to 114 Italian NICUs from June to September 2021.The response rate was 79%. In the past 4 years (range: 2-6), LU has been adopted in 82% of Italian NICUs. It is the first-choice diagnostic test in 23% of the centers surveyed. The main LU diagnostic applications reported were: pneumothorax (95%), respiratory distress syndrome (89%), transient tachypnea of the newborn (89%), plural effusion (88%), atelectasis (66%), pneumonia (64%), bronchopulmonary dysplasia (43%), congenital pulmonary airway malformation (41%), and congenital diaphragmatic hernia (34%). Thirty percent of participating centers calculated LU score routinely, but only seven units used it to predict the need for surfactant replacement. Sixty-six percent of respondents learned the LU technique via a self-training process, while 34% of them visited an expert in the field for one-to-one tuition.LU has a widespread use in Italian NICUs. However, the use of LU is extremely heterogeneous among centers. There is an urgent need to ensure standardization of clinical practice guidelines and to design and implement a formalized and accredited training program.

Little is known about differentially expressed genes (DEGs) and alternative splicing (AS) landscapes in congenital lung malformations (CLMs). We applied reference-based assembly of sequencing reads from RNA sequencing (RNA-seq) libraries to identify DEGs and AS landscapes in the lesions and normal lung tissue from the most common types of CLMs, including congenital pulmonary airway malformation-Ⅰ (CPAM-Ⅰ), CPAM-Ⅱ, intralobar sequestration (ILS), and ILS with CPAM (ILS-CPAM). We analyzed the expression profiles and related biological functions of AS events (ASEs). We further constructed a co-expression regulatory network between RNA binding protein (RBP) genes and corresponding ASEs to explore the related pathways in the regulated network. Ten DEGs were identified in the four types of CLMs, including eight upregulated genes and two downregulated genes. Additionally, 16 differential ASEs were detected, including the genes MACF1, RFX2, and FBXL4. Gene ontology (GO) enrichment was mainly observed in embryonic visual malformation and apoptotic process, and the KEGG pathway mainly enriched in the PI3K/AKT signaling pathway. We also detected 13 differentially expressed RBPs among 1979 DEGs in CPAM-I, in which ASEs in the MACF1 gene and RBP genes TLR8 and PTRH1 were closely associated. Moreover, we confirmed that the expression levels of PTRH1, NSUN7, and DZIP1L abundantly increased and the expression levels of TLR8, MEF2A, and NIPBL decreased in the CPAM-I lung tissue compared with the controls. It is suggested that ASEs in different types of CLMs is prominently different from normal controls, and ASEs differences occurring in CPAM-I malformation tissue are dramatically different from other types, which demonstrates the complex pathogenesis of CLMs and provides foundations for future studies to elucidate the mechanisms of developing CLMs.

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.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.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.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 ObstetricsGynecology published by John WileySons 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.

OBJECTIVE To investigate the relationship between mild congenital pulmonary airway malformation (CPAM) and its long-term prognosis in childhood and to explore whether surgery is necessary. METHODS We conducted a retrospective cohort of fetuses with mild CPAM diagnosed prenatally with available long-term outcomes in childhood from 2004 to 2016. The patients were divided into two groups according to the fetal CPAM-to-volume ratio (CVR) of less than 1.0 and 1.0-1.6. The primary outcome was a postnatal composite outcome including CPAM-associated respiratory symptoms and surgical resection of the lesion. The secondary outcomes included neonatal asphyxia, perinatal morbidity and mortality. RESULTS Forty-two fetuses were identified as having CVR

OBJECTIVES To compare the prognostic factors of fetuses with microcystic and macrocystic congenital pulmonary airway malformations (CPAMs). METHODS We retrospectively evaluated fetuses with CPAMs at Asan Medical Center. The CPAM size, mass effect, and maximum cyst size in macrocystic CPAMs were evaluated prenatally. The adverse postnatal outcomes, including respiratory symptoms, mechanical ventilation, and surgery, were evaluated. RESULTS In 118 cases, 2 fetal deaths and 1 neonatal death occurred. All cases of fetal hydrops and complete regression after birth were in the macrocystic and microcystic CPAM groups, respectively. Twenty-four neonates (20.7%) had respiratory symptoms, and 18 (15.5%) required mechanical ventilation. Sixty-three neonates (54.3%) underwent surgery, of whom 21 (33.3%) required surgery in the neonatal period. The maximum congenital pulmonary airway malformation volume ratio was significantly associated with all postnatal outcomes (P < .05), and the optimal cutoff values were lower for respiratory symptoms, mechanical ventilation, and neonatal surgery in the macrocystic CPAMs. The maximum cyst size was also associated with all postnatal outcomes in macrocystic CPAMs (P < .05). CONCLUSIONS Different cutoff values for the maximum congenital pulmonary airway malformation volume ratio should be applied according to the CPAM type for the prediction of postnatal outcomes. The maximum cyst size can also be a useful prognostic factor in macrocystic CPAMs.

Background The increased and earlier use of prenatal ultrasound has facilitated the detection of congenital thoracic malformations (CTMs). Our Pediatric Pulmonology Institute follows an increasing number of patients with CTMs. Thus, we aimed to evaluate and describe prenatal sonographic findings of CTM, and to estimate changes in detection rates of CTMs over a period of 16 years. Methods A retrospective, cross-section analysis of prenatal ultrasound (US) screening tests carried out in a large community-based clinic, comparing two periods: 2001-2007 and 2007-2017. Results A total of 34 716 prenatal US were performed at a median gestational age of 15.4 weeks (range, 11.6-23.9) and 15.7 weeks (range, 12-33.6) in 2001-2007 and 2007-2017, respectively. In 2001-2007, 12 016 prenatal US tests detected 19 CTMs, compared to 30 CTMs in 22 700 tests in 2007-2017. Detection rates did not change (1.58/1,000 in 2001-2007 versus 1.32/1,000 in 2007-2017, P = 0.64). The most common abnormality was congenital pleural effusion (CPE) (17 cases, 34.7%), followed by congenital pulmonary airway malformation) and congenital diaphragmatic hernia; 13 cases each, 26.5%. Twenty CTMs, mainly congenital diaphragmatic hernia and CPE, were associated with other fetal lesions. Conclusions Congenital diaphragmatic hernia and CPE tend to appear with multiple lesions and warrant further attention. The incidence rates stayed stable when comparing the last decade to previous years. Thus, the increased referral of CTM can be attributed to an increase in the number of prenatal screening studies performed, rather than a true higher incidence.

International audience; AimOesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition.MethodThis was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist.ResultsMore than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation.ConclusionIntrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms.

International audience; Objectives To assess prenatal changes in the volume of congenital pulmonary malformations (CPM) and examine whether these changes differ in lesions that appear cystic on ultrasound compared with hyperechoic lesions, and to study the relationship between CPM volume and risk of fetal compression. Methods We conducted a nationally representative, multicenter, prospective cohort study, which included 579 ultrasound examinations in 176 pregnant women with a diagnosis of fetal CPM, between March 2015 and November 2016. Several ultrasound examinations were performed between diagnosis and delivery, including measurement of CPM volume. We modeled changes in CPM volume ratio (CVR) as a function of gestational age, overall and for cystic/mixed vs hyperechoic malformations, and examined the association between CVR and signs of compression during pregnancy. Results When modeling CVR changes over time, there was a statistically significant decrease in CVR with increasing gestational age (P 0.4 vs CVR ≤ 0.4 cm2. Conclusions Predicted changes in CVR during pregnancy differ between cystic and hyperechoic malformations. This may be the result of different pathophysiological mechanisms or differences in the timing of occurrence of these different types of CPM. CVR measured at the initial diagnostic ultrasound examination was strongly associated with the odds of subsequent compression. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Aims Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lung. Here, we report a case of mucinous adenocarcinoma arising in CPAM. A 23-month-old boy underwent a thoracoscopic lobectomy of the left upper lobe of the lung based on a presumptive diagnosis of asymptomatic CPAM, found in antenatal sonogram. Methods and results Histologically, the lesion was consistent with CPAM, Stocker type I. In addition, multiple foci ranging from mucinous epithelial hyperplasia to mucinous adenocarcinoma were detected. All lesions shared the same immunoprofile with the expression of cytokeratin (CK) 20, MUC5AC, and human epidermal growth factor receptor2 (HER2), but were negative for CK7, transcription factor 1 (TTF-1), MUC1, CDX2, BRAF (VE1) and anaplastic lymphoma kinase (ALK). K-RAS point mutation (G12V) was also detected in all micro-dissected mucinous lesions but EGFR mutation was not found. All lesions were consistent with mucinous adenocarcinoma. The patient's clinical course has been uneventful during the 12-months follow-up period. Conclusions This interesting case demonstrated that multiple foci in CPAM can synchronously transform into malignancies.

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. Copyright  2011 ISUOG. Published by John Wiley & Sons, Ltd.

Background and objective: This study evaluated the accuracy of prenatal MRI and postnatal CT imaging in the identification of congenital cystic adenomatoid malformation and bronchopulmonary sequestration by comparison with histological analysis. Methods: Over a 3-year period, 15 patients with lung malformations diagnosed prenatally by ultrasound were referred for prenatal MRI, and all were investigated postnatally by chest CT. All asymptomatic newborns with unresolved lesions underwent elective surgery by thoracoscopy. All surgical specimens were analysed histologically. Results: Among the 15 patients with an abnormality diagnosed by ultrasound, prenatal MRI findings differed from the final histological diagnosis with respect to extent (n = 3), type of lesion (n = 1) and aberrant vessel identification (n = 4). Postnatal chest CT failed to visualize the aberrant vessel in one patient. Complete regression of the lesion was noted in two patients with bronchopulmonary sequestration, and in one patient with congenital cystic adenomatoid malformation and was confirmed by CT. Elective thoracoscopic lobectomy of the affected lobe was performed for 12 patients. Two conversions to thoracotomy were required. All operated patients had an uneventful hospital course. Conclusions: Prenatal MRI is less accurate than postnatal CT scan, which remains the most reliable diagnostic modality to specify the location and extent and kind of lesions.

Objective To describe the antenatal findings and outcome of fetuses with echogenic lung lesions. Methods This was a retrospective study of the prenatal sonographic features, antenatal management and outcome of 193 fetuses with an echogenic lung lesion diagnosed at 18–35 weeks of gestation. There were nine cases of congenital high airway obstruction syndrome (CHAOS), 170 cases of cystic adenomatoid malformation (CAM) and 14 cases of pulmonary sequestration (PS). A literature search was also carried out to compare our data with those of previous series. Results The prognosis in our series of fetuses with CHAOS was invariably poor, but the literature describes a handful of survivors after delivery by Cesarean section and ex-utero intrapartum therapy (EXIT). Of the cases in our series with PS and no pleural effusions, more than 95% survived; in half of these cases the lesion resolved antenatally and in the other half sequestrectomy was carried out postnatally. In cases with PS and pleural effusions, successful treatment was provided by the placement of thoracoamniotic shunts or occlusion of the feeding blood vessel by ultrasound-guided laser coagulation or injection of sclerosants. In cases with CAM and no hydrops, there was more than 95% survival and in up to half of the cases there was sonographic evidence of spontaneous antenatal resolution of the hyperechogenic lesion, which was confirmed by postnatal imaging in about 60% of the cases. Of the cases with CAM with hydrops managed expectantly, more than 95% died before or after birth. Of the cases with macrocystic CAM with hydrops, two-thirds survived after placement of a thoracoamniotic shunt. In cases with microcystic CAM with hydrops, there is some evidence that open fetal surgery with lobectomy could improve survival but such treatment is highly invasive for the mother. Conclusions CHAOS is a severe abnormality, whereas CAM and PS are associated with a good prognosis. In a high proportion of fetuses with hyperechogenic lung lesion, there is spontaneous antenatal resolution and the underlying pathology may be transient bronchial obstruction. Copyright  2008 ISUOG. Published by John Wiley & Sons, Ltd.

We report a case of a congenital cystic adenomatoid malformation of the lung (CCAM). At 12 weeks of gestation, an echogenic lung associated to a hydrothorax was detected. Despite the results of the combined test that informed of a high risk of chromosomopathy, normal karyotype was confirmed after an amniocentesis. The three-dimensional ultrasound inversion mode depicted all the cysts within the fetal lungs as opaque areas that were seen concurrently together, which was compatible with CCAM. After parental counseling, the patient opted to terminate the pregnancy at 18 weeks. Pathological analysis of the lungs confirmed the diagnosis of a CCAM type II. The recent advent of the three-dimensional ultrasound inversion mode opens many diagnostic options until now unavailable or difficult to attain sonographically and therefore it may help us to increase the sonographic detection rates of congenital cystic adenomatoid malformation.

Aim: To evaluate the usefulness of magnetic resonance imaging (MRI) in predicting the risk of lethal pulmonary hypoplasia in fetuses. Methods: The subjects consisted of 15 fetuses (29–40 weeks' gestation), including fetuses with major malformation diagnosed on prenatal ultrasonography. MRI using a 1.5 T magnet and half-Fourier acquisition single-shot fast spin-echo sequences were applied to all fetuses at 29–36 weeks. Fetal lung-to-liver signal intensity ratio (LLSIR) was calculated by medians of region-of-interest analysis; estimated fetal bodyweight (FBW), by ultrasonography; and estimated fetal lung volume (FLV), by planimetric measurement of total lung volume. FLV/FBW was also calculated. The presence of the pulmonary hypoplasia in neonates was identified based on clinical and anatomico-pathological findings. Differences in LLSIR and FLV/FBW were analyzed for surviving and non-surviving neonates. Results: Ten surviving neonates had a median LLSIR of 3.00, range: 1.60–4.40, while that in seven non-surviving neonates was 2.21, range: 0.70–3.72; no significant difference was found between the groups. Surviving neonates had a median FLV/FBW of 11.4, range: 7.1–15.7, while that in non-surviving neonates was 4.4, range: 3.1–5.7. FLV/FBW in non-surviving neonates was significantly lower than that of the FLV/FBW for surviving fetuses (P

Purpose. To correlate prenatal sonographic diagnosis of cystic lung malformations with fetopathologic findings after termination of pregnancy. Methods. We retrospectively analyzed the data of 16 terminated cases in which a cystic lung lesion was diagnosed pre- or postnatally. Results. On average, prenatal diagnosis was established on the 21st gestational week (range, 19–26 weeks). The cause of termination was severe polyhydramnios in 4 cases, nonimmune fetal hydrops in 4 cases, other congenital malformation in 5 cases (renal malformation, 2 cases; congenital diaphragmatic hernia, 3 cases), and obstetrical conditions (intrauterine death, placental abruption, spontaneous abortion) in 3 cases. In 11 cases, congenital cystic adenomatoid malformation (CCAM) was the presumptive prenatal diagnosis. Autopsy confirmed the prenatal diagnosis in 6 of them, while in the other 5 cases, an enteric cyst, a laryngeal atresia, an unidentified tumor, a pulmonary hypoplasia, and an extralobar pulmonary sequestration were found on histologic examination. On the other hand, the autopsy revealed CCAM in those 5 cases in which other malformations were suggested prenatally. Conclusion. The prenatal sonographic diagnosis of CCAM is difficult. Our cases emphasize the important role of fetopathology even today in the verification of prenatal diagnosis based on sonographic examinations. © 2007 Wiley Periodicals, Inc. J Clin Ultrasound, 2007

Objectives In the fetus with a structurally normal heart, two conditions – giant chest mass, such as congenital cystic adenomatoid malformation (CCAM), and twin–twin transfusion syndrome (TTTS) – alter ventricular loading conditions and may result in cardiovascular compromise. The aim of this study was to elucidate the mechanism of cardiovascular dysfunction by comparing geometryindependent, Doppler flow-derived measures of ventricular performance in fetuses with altered loading conditions vs. those in normal fetuses. Methods Doppler flow-derived measures of myocardial performance index (MPI) as described by Tei, ventricular ejection force as described by Isaaz, and combined cardiac output (CCO) were obtained by echocardiography in fetuses with a normal cardiovascular system (n = 76) or CCAM (n = 36) and fetal partners with TTTS (n = 22). Results In the CCAM group, systolic performance as evidenced by the ejection forces was preserved, right ventricular (RV) MPI was increased and CCO diminished, suggesting diastolic dysfunction and poor filling secondary to cardiac compression and a tamponade effect. In TTTS, recipient twins exhibited greater left ventricular (LV) ejection forces and higher CCO than donor twins, and had abnormal RV and LV MPI, reflecting increased preload, preserved left systolic performance, but diastolic dysfunction. Donor twins had diminished ejection forces and CCO in comparison with normal controls and recipient partners, reflecting hypovolemia. Conclusions In both CCAM and recipient twins of the TTTS, diastolic dysfunction plays a significant role in the pathophysiology of each disorder and precedes changes in systolic performance. Measures of ventricular performance can help elucidate poorly understood mechanisms of cardiovascular compromise in the developing fetus. Copyright  2007 ISUOG. Published by John Wiley & Sons, Ltd.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality that usually presents in childhood. Some associated malignancies have been reported. This study aimed to describe the clinical and multidetector CT (MDCT) image characteristics of CCAM of the lung in adults. Adult patients with congenital cystic lung diseases in association with surgery for CCAM were evaluated over a five and a half year period. Seven (four women, age range 17-64 years) of 109 congenital cystic lung disease patients were histologically confirmed as having CCAM. The most frequent symptom was productive cough (n = 5) and one patient was admitted with haemoptysis. The diagnosis was based on clinical and radiological findings and one patient was not diagnosed until surgery. MDCT images consisted of having a multiple loculated unilobar cystic mass in six patients and a cavitary mass in one and/or normal systemic arteries. The lesion was present in the right lung in four and in the left lung in three patients. The involved lobe was the upper in three, lower in three and middle lobe in one. Six patients underwent lobectomy and there was no associated malignancy or mortality. The mean length of hospital stay was 17.5 +/- 7.3 days. In adult patients who suffer from a recurrent productive cough and who have a multiloculated cystic mass in one lobe and normal vascular images in MDCT, CCAM, although rare, should be considered.

We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks' gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks' gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.

ongenital lobar emphysema (CLE), also known as congenital lobar overdistension, is a usually postnatally diagnosed malformation of the lung, which can be characterized by air trapping and overdistension of segments and lobes. In some cases, there is respiratory distress, a mediastinal shift, and wheezing due to spontaneous overinflation of the affected areas.1 Prenatal diagnosis has been made in a number of cases (Table 1),2–10 but in most cases, the diagnosis was made postnatally. Such fetuses have a congenital echogenic mass, including a mediastinal shift and displacement of the heart, resulting in compression of the contralateral lung.2,4–6 Also, macrocystic and microcystic lesions have been reported.3,7 In a series of 94 cases of congenital masses of the lung, it was only possible to make the correct diagnosis of CLE in 6 patients after birth.11 This condition is diagnosed at birth in about 25% and at the age of 1 month in about 50%. The diagnosis is sporadic after 6 months.1,12 Standard treatment for the acute neonatal respiratory distress in this diagnosis is surgical removal of the affected lung area.1,13 In some cases of neonatal respiratory distress, however, there is spontaneous resolution.14,15 The diagnosis of a congenital mass of the lung includes congenital cystic adenomatoid malformation (CCAM), CLE, pulmonary sequestration, and bronchogenic cysts.11 We report a case of a congenital echogenic mass of the lung diagnosed as CCAM after imaging in the fetus and neonate. Only histologic examination, however, revealed the correct diagnosis of CLE. Although treatment of CCAM should involve postnatal resection because of the potential of malignancy, CLE can be treated expectantly in most cases. We discuss the problems of correct diagnosis of congenital lung masses and the implications for pediatric treatment. Received May 17, 2004, from the Departments of General Pediatrics (T.A.), Pediatric Radiology (H.C.O.), Pediatric Surgery (S.E.), Pediatric Pathology (I.L.), and Obstetrics and Gynecology (C.S.v.K.), University of Schleswig-Holstein, Kiel, Germany. Revision requested June 20, 2004. Revised manuscript accepted for publication June 29, 2004. We thank William D. Travis, MD, Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC, and Klaus M. Muller, MD, Institute of Pathology, Berufsgenossenschaftliche Kliniken, University Hospitals of Bochum, Bochum, Germany, for expert advice on the histologic specimens. Address correspondence and reprint requests to Tobias Ankermann, MD, Department of General Pediatrics, University of SchleswigHolstein, Campus Kiel, Schwanenweg 20, 24105 Kiel, Germany. E-mail: ankermann@pediatrics.uni-kiel.de. Abbreviations CCAM, congenital cystic adenomatoid malformation; CLE, congenital lobar emphysema; CT, computed tomography; MRI, magnetic resonance imaging

For safer and simpler neonatal ECMO management, the authors have been developing an innovative, automatically-controlled ECMO machine using self-regulating blood pumps. The newest model is an air-driven, tube-type blood pump. A pair of blood pumps are placed in parallel and actuated alternately with compressed air. The pump flow is automatically regulated in accordance with hemodynamic changes of the body. The need for a venous reservoir is eliminated because the circuit does not generate excessive negative pressure when there are shortages of draining blood. Therefore, the priming volume of the circuit is only 85 ml. This ECMO apparatus has three driving modes, one of which is a "delay" mode. This enables the pump flow to easily be varied from 10 to 500 ml/min under automatic control, and it can be used when the pump flow is decreased for weaning. This newest ECMO apparatus was clinically used in 3 neonates with severe lung hypoplasia and persistent fetal circulation. The ECMO duration was 139 h to 168 h, and the maximum ECMO flow 71.3 to 109.0 ml/min/kg. Thanks to the self-regulation, intensive observation of the circuit was not necessary during ECMO. Damage to blood cells was less significant, and the use of blood products was only minimal (15.7 ml/kg/day) because a large volume of blood to stabilize the pump performance was not necessary. In conclusion, the automatically-controlled ECMO apparatus worked well without complications by changing the ECMO flow automatically in accordance with hemodynamic changes. This automatic ECMO system required fewer personnel expenditures and was also favorable in terms of hematological findings.

Congenital cystic adenomatoid malformation of the lung can be detected with antenatal ultrasound as hyperechogenic areas in the fetal chest. Associated extrapulmonary malformations as well as chromosomal aberrations are described as very rare. We present a case report of a fetus in the 23rd week of gestation who showed in the course of a routine ultrasound screening a large number of malformations: holoprosencephaly, arrhinencephaly, cleft palate, CCAM type III of the right inferior pulmonary lobe, ventricular septal defect and bilateral clubfeet. Chromosome analysis confirmed the suspicion of trisomy 13. The present case shows how important it is—even with malformations that are rarely accompanied by associated anomalies and which have a very good prognosis—to carry out a directed diagnosis including a fetal karyotyping. Copyright © 2003 John Wiley & Sons, Ltd.

Objectives Most available data regarding accuracy of prenatal diagnosis, prevalence and outcome of congenital cystic adenomatoid malformation (CCAM) are derived largely from tertiary referral centres and may not reflect general population rates. We aimed to describe the accuracy of prenatal diagnosis, ascertain the population prevalence and post-natal outcome for cases of suspected CCAM. Methods Retrospective collection of prenatal and paediatric data for cases of suspected CCAM notified to the Trent Congenital Anomalies Register 1997 to 2001. Results Thirty-seven cases of CCAM were suspected prenatally. Twenty-one cases were confirmed post-natally as having a CCAM (positive predictive value 57%). Eighteen of the 21 cases were delivered at term as live births, 15 of which have undergone successful surgery to date. Thirteen of the 37 cases had apparently resolved by delivery. Three further cases were subsequently found to be cases of lung sequestration or lobar emphysema. Five cases of CCAM were detected after delivery (sensitivity of prenatal detection 81%). The population prevalence at delivery was 9.0 per 1 00 000 total births. Five confirmed cases of CCAM developed hydrops, three required in utero intervention and delivered as live births at term, one was terminated and one died in utero. The overall mortality in the confirmed cases of CCAM was 23% of which the majority were terminations of pregnancy. Conclusions Problems of diagnostic accuracy and apparent resolution of CCAM render counselling difficult, although our data suggest that the prognosis is better than others have reported. Confirmation of the diagnosis in the neonatal period is vital in order to obtain the true population prevalence figures and to interpret outcome data. Copyright © 2003 John Wiley & Sons, Ltd.

A 9-day-old boy presented in respiratory distress and with failure to thrive. The chest X-ray showed a hyperlucent area of the left lung. A resection of the markedly emphysematous segment 2 of the left upper lobe was performed assuming the emphysematous tissue was due to congenital lobar emphysema (CLE). Histological examination of the lung tissue, however, revealed a pattern consistent with congenital cystic adenomatoid malformation (CCAM) type 4. The therapy for CLE as well as for CCAM is similar, i.e., resection of the emphysematous tissue. As far as the prognosis is concerned, it is important to diagnose the exact type of malformation in order to exclude associated anomalies, as well as the risk of development of malignancies in later life. The frequency of associated malformations of CCAM type 4 is unknown. Although the risk for development of malignancies from CCAM type 4 is not clear at the moment, the possible development of malignancies justifies prompt resection shortly after diagnosis, even in asymptomatic patients. A life-long follow-up in those patients who had a resection of CCAM in early childhood is recommended.

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.

A small infant with congenital cystic adenomatoid was scheduled for thoracoscopic resection of the lung cyst. During carbon dioxide insufflation, there was a sharp rise of endtidal carbon dioxide which was followed by marked hypoxemia and bradycardia due to occlusion of the tracheal tube with blood. The plan changed to open thoracotomy and total pneumonectomy. Despite several reports, which addressed successful thoracoscopic cystic lung resection, its safety remain to be determined.

Congenital cystic adenomatoid malformation of the lung (CCAML) is uncommon, and usually presents in children with respiratory distress. We describe a girl presenting with pneumonia at age 10 years. She had been previously healthy and active, and with no previous hospitalizations or X-rays. A chest CT scan showed right lower lobe cystic lesions suggestive of CCAML.

The prenatal diagnosis of an echogenic fetal lung (EFL) is now often made in the early second trimester using high-resolution ultrasound. This ultrasound appearance is usually caused by a congenital cystic adenomatoid lung malformation (CCAM), an intrapulmonary lung sequestration or obstruction of a major airway. In order to provide prognostic guidelines to parents who may be considering termination of a fetus with these findings, we have analysed a series of 11 cases diagnosed in our centre over the past 2 years in conjunction with 60 cases from major published series. The data suggest that in the absence of non-immune hydrops fetalis (NIHF) or other anomalies, the outcome for the fetuses is excellent, with over 90 per cent survival. Neither early diagnosis (24 weeks) nor the presence of mediastinal shift is a poor prognostic indicator. In addition, it appears that if NIHF is absent at diagnosis, the chance that it will develop as the pregnancy continues is small (6 per cent). Furthermore, there is a significant (up to 30 per cent) chance that this ultrasound finding will resolve in utero. The development of in utero fetal surgical techniques may be the only hope for those hydropic fetuses who appear to have a dismal prognosis.

Few reports concerning intrauterine shunting are available. We investigated the impact of this method. In order to evaluate intrauterine shunting and the complication rate for different indications, we sent a questionnaire to all German-speaking level 3 centres. In four level 3 centres, 52 intrauterine catheters were inserted in 34 fetuses. The indications were uni- or bilateral hydrothorax in nine cases, cystic adenomatoid malformation of the lung in four cases, infravesical stenosis in 13 cases, and fetal ascites in eight cases. In three cases (6 per cent), difficulties occurred when the drain was inserted. In 15 cases (29 per cent), the function of the drain was reduced by dislocation or occlusion. The mortality rate caused by shunting was as high as eight per cent (four cases). The application of an intrauterine shunt currently represents a rarely performed ultrasound-guided therapeutic intervention in the fetus. In all cases, the indication for shunting is to avoid compression of normal tissue by cystic structures. A high complication rate restricts the application of drainage to selected cases.

During an 8-year period (1984-1992), we made the ultrasonographic diagnosis of cystic adenomatoid malformation (CAM) of the lung in 58 fetuses at 17-39 weeks' gestation. We reviewed the records of these fetuses and combined the data from 74 cases reported in the literature to determine the incidence of the different types of CAM, associated malformations, and outcome. The lesions were macrocystic in 78 (59 per cent) and microcystic in 54 (41 per cent) of the cases. CAM was left-sided in 51 per cent, right-sided in 35 per cent, and bilateral in 14 per cent of the fetuses. In 15 (11 per cent) of the fetuses there were additional malformations and 57 (43 per cent) were hydropic. The pregnancy was electively terminated in 44 (33 per cent) of the cases, including all those with bilateral CAM. There were six (5 per cent) intrauterine deaths, five in association with hydrops, and one with growth retardation and heart defect. Of the 82 (62 per cent) infants that were liveborn, 21 (26 per cent) died in the neonatal period, 15 before and six after surgery. Of the 61 survivors, 16 (26 per cent) did not require surgery. In the 88 cases where the pregnancy was not terminated, survival was better if the CAM was macrocystic (74 per cent versus 58 per cent for microcystic), if there was no hydrops (92 per cent versus 21 per cent for hydrops), and if the amniotic fluid volume was normal or decreased (82 per cent versus 53 per cent for polyhydramnios).

Pulmonary disorders in infants are intimately related to the pulmonary development during the antenatal, neonatal and infantile period. In this review, normal pulmonary development is considered and several pulmonary disorders such as pulmonary hypoplasia, pulmonary sequestration, congenital cystic adenomatoid malformation, hyaline membrane disease, bronchopulmonary dysplasia, Wilson-Mikity syndrome and perinatal pneumonia are presented in light of the current literature.

Six cases of macrocystic and one case of microcystic congenital adenomatoid lung malformation were diagnosed by ultrasound between 20 and 31 weeks of gestation. Combined polyhydramnios and fetal hydrops was present in three cases, polyhydramnios alone in one case, and isolated fetal hydrops also in one case. In the remaining two cases, both polyhydramnios and fetal hydrops were absent. Fetal outcome was poor, i.e., two terminations of pregnancy, three early neonatal deaths, and two survivors.

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.

Congenital cystic adenomatoid malformation of the lung is a developmental abnormality characterized by abnormal proliferation of terminal bronchioles forming cysts of varying sizes. Extensive lesions are associated with a poor prognosis due to the development of nonimmune hydrops and/or pulmonary hypoplasia. The advent of high-resolution ultrasonography has made it possible to identify these lesions during the antenatal period. With the prenatal identification of cystic adenomatoid malformations comes the potential for in utero intervention to modify the natural history of this process and prevent the development of hydrops and pulmonary hypoplasia. An instance of successful intrauterine treatment of congenital cystic adenomatoid malformation is presented.