Fetal Lung Lesions Research Articles

Maternal "mirror" syndrome: Evaluating the benefits of fetal therapy.

To evaluate maternal and perinatal outcomes following fetal intervention in the context of maternal "mirror" syndrome. A multicenter retrospective study of all cases of fetal hydrops complicated by maternal "mirror" syndrome and treated by any form of fetal therapy between 1995 and 2022. Medical records and ultrasound images of all cases were reviewed. "Mirror" syndrome was defined as fetal hydrops and/or placentomegaly associated with the maternal development of pronounced edema, with or without pre-eclampsia. Fetal hydrops was defined as the presence of abnormal fluid collections in ≥2 body cavities. Twenty-one pregnancies met the inclusion criteria. Causes of fetal hydrops and/or placentomegaly included fetal lung lesions (n=9), twin-twin transfusion syndrome (n=6), severe fetal anemia (n=4), and others (n=2). Mean gestational age at "mirror" presentation was 27.0±3.8weeks. Maternal "mirror" syndrome was identified following fetal therapeutic intervention in 14 cases (66.6%). "Mirror" symptoms resolved or significantly improved before delivery in 8 (38.1%) cases with a mean interval from fetal intervention to maternal recovery of 13.1days (range 4-35). Three women needed to be delivered because of worsening "mirror" syndrome. Of the 21 pregnancies treated (27 fetuses), there were 15 (55.5%) livebirths, 7 (25.9%) neonatal deaths and 5 (18.5%) intra-uterine deaths. Following successful treatment and resolution of fetal hydrops, maternal "mirror" syndrome can improve or sometimes completely resolve before delivery. Furthermore, the recognition that "mirror" syndrome may arise only after fetal intervention necessitates hightened patient maternal surveillance in cases of fetal hydrops.

Perinatal outcomes of prenatal diagnosis of congenital pulmonary airway malformation: an experience.

This study aimed to assess the perinatal outcomes of pregnancies with a prenatal diagnosis of congenital cystic adenomatoid malformation. We conducted a retrospective cohort study based on information contained in the medical records of pregnant women whose fetuses had been prenatally diagnosed with congenital cystic adenomatoid malformation by ultrasonography. Sample analysis was based on 21 singleton pregnancies with confirmed isolated fetal congenital cystic adenomatoid malformations. The mean maternal±standard deviation age was 28±7.7 years. Types I, II, and III congenital cystic adenomatoid malformation were detected in 19% (4/21), 52.4% (11/21), and 28.6% (6/21), respectively. All fetuses presented with unilateral congenital cystic adenomatoid malformation (21/21) without associated anomalies, and 52.3% (11/21) were in the right lung. In total, 33.3% (7/21) of fetuses presented a "congenital cystic adenomatoid malformation volume ratio" >1.6 and were managed with maternal betamethasone administration. The mean gestational age at the time of steroid administration was 28.5±0.9 weeks, with a reduction in the lesion dimensions of 9.5% (2/21) (Types I and III of congenital cystic adenomatoid malformation). The mean gestational age at delivery was 38.7±2.4 weeks, and a cesarean section was performed in 76.2% (16/21) cases. Postsurgical resection was necessary for 23.8% (5/21) of the patients, and 4.7% (1/21) of them died because of respiratory complications after surgery. Pulmonary hypoplasia occurred in 9.5% (2/21) of the patients, and 4.7% (1/21) of them died because of respiratory insufficiency. The survival rate was 90.5% (19/21), and 57.2% (12/21) remained asymptomatic. Despite the isolated prenatal diagnosis of congenital cystic adenomatoid malformation, which showed good survival, congenital cystic adenomatoid malformation is associated with significant perinatal morbidity. Maternal betamethasone administration did not significantly reduce fetal lung lesion dimensions.

In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: A single center experience

BackgroundSevere primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS). ObjectivesTo compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH. MethodA retrospective analysis was performed for all fetuses treated by TAS, between the years 2004–2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires. Results38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups. ConclusionTAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

Pulmonary Cystic Adenomatoid Malformation, Prenatal Diagnosis and Case Management

The Cystic Adenomatous Malformation (CAM) is a rare fetal lung lesion. Its frequency is about 1 in 4000 births and is characterized by an overgrowth of terminal respiratory bronchioles that forms cysts of various sizes. It can be associated with polyhydramnios and pulmonary hypoplasia. We describe here a case of Adenoid Cystic Pulmonary Malformation type II with prenatal diagnosis at 20 weeks of gestational age, as well as its subsequent management. Morphological ultrasound describes multiple small cysts associated with a slight mediastinal deviation. The prognosis depends mainly on the associated injuries. Prenatal ultrasound allows early diagnosis and follow-up of CCAM to monitor the appearance of possible pathophysiological complications that may worsen its prognosis.

Interstitial Laser Occlusion of the Systemic Feeding Vessel in a Hybrid Lung Lesion: Technique, Clinical Course, Perinatal Outcome and a Review of Literature

AbstractThe two most common fetal lung lesions include Bronchopulmonary sequestration (BPS) and Congenital pulmonary airway malformation (CPAM).Their co-existence is known as a hybrid lesion. Fetal hydrops in such cases is associated with very high morbidity and mortality. Several fetal interventions have been recommended over the past years to treat the same, with varying success rates. We describe a case of a hybrid CPAM complicated by hydrops which was successfully treated by interstitial laser coagulation of the systemic feeder artery. We also review current literature on the available interventions and conclude that interstitial laser has emerged as the preferred modality of treatment over the years.

Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions.

Fetal lung lesions include common lesions such as congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS) and combined CPAM-BPS hybrid lesions, as well as less common entities including congenital lobar emphysema/obstruction, bronchial atresia, bronchogenic cysts and rare malignant pulmonary lesions such as pleuropulmonary blastoma. Fetal lung lesions occur in approximately 1 in 15,000 live births and are thought to arise from a spectrum of abnormalities related to airway obstruction and malformation, with the lesion type depending on the timing of insult, level of bronchial tree involvement, and severity of obstruction. Lesions vary from small and asymptomatic to large and symptomatic with significant mass effect on surrounding structures. Accurate diagnosis and characterization of these anomalies is crucial for guiding patient counseling as well as perinatal and postnatal management. The goal of this review is to provide an overview of normal fetal lung appearance and imaging features of common and uncommon lesions on both ultrasound and MR imaging, and to discuss key aspects in reporting and evaluating the severity of these lesions.

EP17.13: Prenatal sonographic presentation, diagnosis and outcome of fetal echogenic lung lesion

To describe fetal echogenic lung lesion in terms of presenting features on prenatal ultrasound, establishing diagnosis, predicting prognosis and outcome. This is a 5yr retrospective study from Jan' 13 to Dec'17. Total 40 fetuses were diagnosed with echogenic lung lesion. The GA was from 16 to 29 wks. This included 6 cases of CHAOS, 28 cases of CPAM and 6 cases of PulSequestration (PS) The US presentation of CHAOS was unmistakenly similar and classical in all cases with bilateral enlarged, echogenic lung with dilated trachea and anteriorly pushed heart. All the 3 cases of extrathoracic PS, delivered with normal postnatal USG abdomen and x-ray chest. 2 of 3 fetuses with intrathoracic PS regressed. In one fetus the lesion regressed but persisted. Postnatal x-ray confimrd the lesion. but as the child is asymptomati no intervention done. Out of 28 CPAM cases, 4 macrocystic, type II lesion in 3 and microcystic lesion in remaining 21 cases. Only 1 fetus had hydrops at diagnosis. Three fetuses had CVR > 1.6. Out of four macrocystic CPAM cases two terminated, one had IUFD due to hydrops and in one fetus the lesion increased in size, baby underwent thoracoscopic excision of the cyst at 2 yrs of life and doing well now. Out of 3 type II CPAM, two fetuses delivered and postnatal MRI is normal. In one fetus, the lesion became macrocystic during follow up, but regressed at 30 weeks with normal postnatal x-ray. 21 fetuses of microcystic CPAM, one with other systemic malformation terminated. In all 20 cases the postnatal x-ray was normal and babies are doing well. Fetal lung lesions are easily diagnosed during level II USG. Among fetal echogenic lung lesions the most common are CPAM. CHAOS are mostly fatal. CPAM and BPS are relatively benign lesions. Tracing the blood supply is the key to diagnosis. CPAM and BPS though look deceptive generally regress with increasing gestation. Diagnosing the lesion with accuracy, hydrops and the CVR are useful in predicting the outcomes.

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions.

To assess the diagnostic performance of the fetal cardiac axis (CA) and/or cardiac position (CP) versus the congenital pulmonary malformation volume ratio (CVR) in predicting any and severe neonatal respiratory morbidity in fetal congenital lung lesions. This work was an 11-year retrospective cohort study. The sensitivity, specificity, positive predictive value, and negative predictive value of CA and/or CP assessment in prediction of respiratory morbidity were calculated before 24 weeks' gestation and between 24 and 32 weeks and compared to CVR cutoffs obtained from the literature. Fifty-three patients were included. CA and/or CP abnormalities were present in 45% and 38% of patients before 24 weeks and between 24 and 32 weeks and were significantly more common in left- versus right-sided lesions (60% versus 17%; P = .003). The sensitivity, specificity, positive predictive value, and negative predictive value of an abnormal CA and/or CP for any and severe respiratory morbidity were 0.67, 0.61, 0.33, and 0.86 and 0.8, 0.58, 0.17, and 0.97 before 24 weeks and 0.75, 0.73, 0.45, and 0.91 and 0.8, 0.67, 0.20, and 0.97 between 24 and 32 weeks, respectively. An abnormal CA and/or CP had higher sensitivity for any respiratory morbidity compared to the CVR at 0.5 and 0.8 cutoffs both before 24 weeks and between 24 and 32 weeks (P < .05). An abnormal CA and/or CP before 24 weeks and between 24 and 32 weeks has higher sensitivity for the detection of any respiratory morbidity at birth compared to the CVR at both 0.5 and 0.8 cutoffs. A normal CA and CP have a high negative predictive value for excluding any respiratory morbidity at birth both before 24 weeks and between 24 and 32 weeks.

Fetal chest malformations

Congenital anomalies of the fetal chest typically consist of lung lesions that are echogenic on ultrasound. These lesions comprise congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), bronchial atresia, bronchogenic cysts, congenital lobar emphysema, and congenital high airway obstruction syndrome (CHAOS). CPAM and BPS account for a majority of these anomalies. Although rare, the apparent incidence of these echogenic lung lesions has increased recently, with CPAMs now occurring at a rate of 0.94 per 10,000 live births. This increase is likely a product of the improved resolution of ultrasound equipment, and the increasing uptake of routine fetal anomaly scanning in the second trimester. In utero, fetal echogenic lung lesions can result in mediastinal shift, fluid collections and hydrops. Following birth, they can cause substantial neonatal respiratory compromise. Determining the type of lesion prenatally can be challenging, even with complementary imaging such as MRI scanning, and the final histopathological diagnosis – where available – is often different to that suspected prior to birth. Of greater importance to prospective parents, however, is the likelihood of a favourable outcome, and the need for fetal intervention and/or intensive neonatal care. A range of sonographic predictors has been proposed for this purpose, but the best studied index is the CPAM-volume ratio – the ratio of the volume of the lesion (calculated as a prolate ellipse) to the fetal head circumference, to normalise for gestation. This presentation will review the performance of the CVR in the prediction of a range of fetal and neonatal concerns, including in a series from the author's own institution. It will also compare this index with other predictors, none of which has shown sufficient utility to replace the CVR at this stage. It will conclude with suggestions regarding future research in this field.

Prenatally diagnosed congenital lung malformations—A long‐term outcome study

To report the natural history of a cohort of children with prenatally diagnosed congenital lung malformations (CLM) which we set out to manage expectantly. Retrospective review of children born between 1995 and 2013 with a CLM identified on prenatal ultrasound. Prenatal ultrasound data were analyzed along with patient medical records, radiology, and pathology. One hundred fifty-nine newborns with a fetal lung lesion were identified during the study period. All infants were live born. Twenty-eight (18%) newborns were symptomatic, 17 with transient symptoms, and 11 with persistent symptoms. The latter all underwent lobectomy in the neonatal period with two postoperative deaths. One hundred thirty-one asymptomatic newborns plus the 17 babies with transient symptoms (148 total) were followed during childhood for a median of 6.0 years (0.1-19.2 years). Twenty-one children (13% of the original cohort of 159) became symptomatic at a median age of 2.5 years (9 months-5 years 8 months) with infection and underwent CLM resection. No child became symptomatic after the age of 5 years 8 months. One hundred twenty-seven children remained symptom free during follow-up for a median of 5.75 years (1 month-19 years). We saw no instance of malignancy in the resected specimens. This study adds further evidence that most children born with CLM identified prenatally are asymptomatic at birth and the majority will remain asymptomatic during childhood. We recommend follow-up to the age of 10 years.

The rare solid fetal lung lesion with T2-hypointense components: prenatal imaging findings with postnatal pathological correlation.

At fetal MR, congenital lung lesions are usually T2 hyperintense with respect to normal lung parenchyma. Some lesions, however, demonstrate unusual patterns of T2 hypointensity, sometimes in a rosette-like pattern. These lesions usually present a diagnostic conundrum. To evaluate the imaging findings and pathological characterization of fetal solid lung lesions with elements showing T2-hypointense signal with respect to lung. This is a retrospective study of lung lesions with elements showing T2 hypointensity treated prenatally and postnatally at our center and with available pathological evaluation. Prenatal imaging evaluation included US and MR; postnatal evaluation consisted of pathological examination of the lesion. We also performed prenatal and postnatal chart review. Six cases met study criteria. Areas of decreased echogenicity/T2-hypointense signal were more conspicuous at MR than US. At pathology, these areas correlated with immature parenchymal development and increased mesenchymal tissue. Five of these lesions were congenital pulmonary airway malformations (CPAM); one was a congenital peribronchial myofibroblastic tumor (CPMT). The lesions did not significantly change in size after steroid administration. They were all large in volume and were associated with increased amniotic fluid. All cases of CPAM underwent premature delivery (one of them weeks after fetal surgical resection of the lesion for worsening hydrops); the fetus with CPMT was delivered at term. The neonate with CPMT succumbed shortly after birth secondary to lung hypoplasia; the remaining five neonates survived. The differential diagnoses of prenatal lung lesions that contain unusual T2-hypointense elements include CPAM and CPMT. The T2-hypointense areas appear to correlate with increasing degree of immaturity at histology. None of the lesions significantly changed in size after prenatal administration of steroids. All cases with CPAM lesions did well despite persistent polyhydramnios and premature birth. The single case of CPMT, however, resulted in neonatal demise shortly after birth secondary to pulmonary hypoplasia. It is important that fetal radiologists, obstetricians and fetal surgeons alike are aware of these lesions so that appropriate diagnosing and parental counseling can be reached.

Antenatal assessment and postnatal outcome of fetal echogenic lung lesions: a decade’s experience at a tertiary referral hospital

Introduction: Fetal echogenic lung lesions (ELL) are the commonest pulmonary pathology diagnosed on antenatal sonography, and include congenital pulmonary airway malformations (CPAMs) and bronchopulmonary sequestrations. This study aimed to evaluate the predictive utility of the CPAM volume ratio (CVR) at presentation in a series of fetuses with ELLs at a tertiary Australian referral hospital.Material and methods: Retrospective cohort study of all pregnancies with a prenatal diagnosis of an isolated fetal echogenic lung lesion managed at the Royal Women’s Hospital, Victoria, Australia, between 2005 and 2015. Data were obtained from electronic ultrasound image databases and medical records.Results: Sixty-five cases were included in the final analysis. The mean gestation at presentation was 22 weeks and 6 d, and the mean CVR was 0.66. Hydrops was evident in four cases at presentation, and did not develop subsequently in any other case. Significant perinatal concerns – fetal/neonatal demise, hydrops, requirement for neonatal intubation/ventilation, or surgery in the first year of life – did not occur with or following a CVR at presentation of <0.45. The survival rate at 1 year was 95%.Discussion: The CVR is a potentially useful tool to assess all fetal ELLs, and not just those presumed to be CPAMs. A CVR at presentation of <0.45 was associated with favourable outcomes.

Fetal Lung Surgery

ABSTRACT Advances in technology and wide used of obstetric ultrasound have allowed an increase in the antenatal identification of fetal problems, such as fetal lung lesions. Technological advances in transducer and software technology have remarkably improved visualization of structures in the fetus. Additionally, newer understanding of the natural course of several malformations and new methods of in utero treatment, the evolution of fetal surgical techniques and anesthesia have made fetal surgery possible and have changed the course of evolution of lung defects before and after birth. Therefore, there is now an increasing demand for an accurate and timely diagnosis, counseling, and planning of appropriate management of the cases whether expectant management or in utero therapy at perinatal care centers with timely perinatal transfer. How to cite this article Sen C, Yayla M, Api O, Goynumer G. Fetal Lung Surgery. Donald School J Ultrasound Obstet Gynecol 2016;10(3):271-296.

Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan.

The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. Fetal hydrops was visualized using MRI in 9.2% of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8% of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04±1.71 vs. 0.98±0.50, P<0.00071), and decreased to a greater degree in non-CCAM patients compared with CCAM patients during the late gestational period (from 1.37±1.28 to 1.14±0.84 in CCAM and from 1.08±0.47 to 0.46±0.64 in non-CCAM). An estimated 8-9% of prenatally diagnosed patients carry the highest risk of perinatal respiratory distress. Fetal LVR remaining at a high level during the late gestational period seems to predict a high risk.

Diagnóstico prenatal y manejo de malformación pulmonar fetal

La malformación adenomatosa quística (MAQ) es una rara lesión pulmonar fetal y se caracteriza por un crecimiento exagerado de los bronquiolos terminales, los cuales forman quistes. Describimos un caso de diagnóstico y manejo prenatal y postnatal de MAQ a 3,400 msnm (Cusco).

Fetal laser therapy: applications in the management of fetal pathologies

Fetoscopic coagulation of placental anastomoses is the treatment of choice for severe twin-to-twin transfusion syndrome. In the present day, fetal laser therapy is also used to treat amniotic bands, chorioangiomas, sacrococcygeal teratomas, lower urinary tract obstructions and chest masses, all of which will be reviewed in this article. Amniotic band syndrome can cause limb amputation by impairing downstream blood flow. Large chorioangiomas (>4 cm), sacrococcygeal teratomas or fetal hyperechoic lung lesions can lead to fetal compromise and hydrops by vascular steal phenomenon or compression. Renal damage, bladder dysfunction and lastly death because of pulmonary hypolasia may be the result of megacystis caused by a posterior urethral valve. The prognosis of these pathologies can be dismal, and therapy options are limited, which has brought fetal laser therapy to the forefront. Management options discussed here are laser release of amniotic bands, laser coagulation of the placental or fetal tumor feeding vessels and laser therapy by fetal cystoscopy. This review, largely based on case reports, does not intend to provide a level of evidence supporting laser therapy over other treatment options. Centralized evaluation by specialists using strict selection criteria and long-term follow-up of these rare cases are now needed to prove the value of endoscopic or ultrasound-guided laser therapy.

Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases

PurposeHydrops and pulmonary hypoplasia are associated with significant morbidity and mortality in the setting of a congenital lung lesion or pleural effusion (PE). We reviewed our experience using in utero thoracoamniotic shunts (TA) to manage fetuses with these diagnoses. MethodsA retrospective review of fetuses diagnosed with a congenital lung lesion or pleural effusion who underwent TA shunt placement from 1998–2013 was performed. ResultsNinety-seven shunts were placed in 75 fetuses. Average gestational age (± SD) at shunt placement and birth was 25±3 and 34±5weeks. Shunt placement resulted in a 55±21% decrease in macrocystic lung lesion volume and complete or partial drainage of the PE in 29% and 71% of fetuses. 69% of fetuses presented with hydrops, which resolved following shunt placement in 83%. Survival was 68%, which correlated with GA at birth, % reduction in lesion size, unilateral pleural effusions, and hydrops resolution. Surviving infants had prolonged NICU courses and often required either surgical resection or tube thoracostomy in the perinatal period. ConclusionTA shunts provide a therapeutic option for select fetuses with large macrocystic lung lesions or PEs at risk for hydrops and/or pulmonary hypoplasia. Survival following shunting depends on GA at birth, reduction in mass size, and hydrops resolution.

Retrospective study of prenatal diagnosed pulmonary sequestration

To describe the prenatal findings, treatments and outcomes of fetuses with pulmonary sequestrations (PS), which were retrospectively studied. From May 2010 to January 2013, 292 women were referred to the Guangdong Women and Children Hospital, Guangzhou because obstetric ultrasound had demonstrated fetal lung lesions. In 68 fetuses, the echogenic lung masses were pulmonary sequestrations deriving arterial blood supply from clearly identifiable systemic arteries rather than the pulmonary artery. We examined records of the 68 fetuses and measured the lesions and congenital cystic adenomatoid malformation volume ratio (CVR), provided prenatal counseling and treatment, documented the CVR, location of lesion, gestational age at diagnosis, need for fetal intervention, perinatal clinical course (including the development of hydrops, effusions, and neonatal respiratory distress), gestational age at delivery, postnatal ultrasound and CT, operation treatment, survival and pathology. There were 68 PS in our study. All of 56 cases with CVR ≤ 1.6, survived without prenatal hydrops or postnatal respiratory symptoms. In 12 cases with CVR >1.6, 7 cases (58.3 %, 7/12) had hydrops, 10 cases (83.3 %, 10/12) survived, 2 cases (16.7 %, 2/12) of induced abortion, 8 cases (66 %, 8/12) were postnatal symptomatic. There was statistical significant difference in the incidence rate of hydrops and postnatal respiratory symptoms between the PS with CVR ≤ 1.6 and that with CVR >1.6. 66 cases postnatal survived, two cases of induced abortion. 64 cases had no prenatal treatment, two cases underwent thoracoamniotic shunt. The mean gestational age at birth was 38 weeks (range 34-40 weeks). 21 asymptomatic cases had no surgery. 45 cases (8 symptomatic and 37 asymptomatic) underwent surgical resections, 43 cases without postoperative complications, two cases of postoperative pneumothorax, no wound infection. Postoperative follow-up showed good growth in all surgery cases. PS specific absence of hydrop was a congenital disorder with an excellent prognosis. CVR >1.6 was associated with fetal hydrops and postnatal symptoms. However, absolute CVR value cannot be used to select fetuses for fetal treatment before the development of hydrops. Surgery of PS in neonates or infants presented a good outcome.

Malformations adénomatoïdes kystiques du poumon : diagnostic, prise en charge, hypothèses physiopathologiques

Congenital cystic adenomatoid malformations (CCAM) of the lung are the most frequent congenital lung malformations. Their diagnosis is based on histological features. CCAM consist of bronchopulmonary cystic lesions which are classified according to the presence and cysts size. Type I CCAM are composed of large cysts (>2 cm) lined by a columnar pseudostratified epithelium. Type II CCAM contain multiple small cystic lesions (<1 cm) lined by a flattened cuboidal epithelium. Type III CCAM are more solid and contain immature structures resembling the pseudoglandular stage of lung development. Ultrasonography (US) allows early detection during the second trimester of pregnancy as cystic, and/or hyperechoic fetal lung lesions. Although most CCAM remain asymptomatic, CCAM can cause polyhydramnios or fetal hydrops, respiratory distress at birth, infections and pneumothoraces during infancy, and may give rise to malignancies. Serial US allow detection of complications, and planification of delivery. Complicated forms require an urgent treatment. In fetuses with a macrocystic life-threatening lesion, a thoraco-amniotic shunt can be placed. Microcystic compressive forms may respond to prenatal steroids. Post-natal symptomatic lesions require early surgery. The treatment of asymptomatic forms remains controversial. Some recommend a non-operative approach with a long-term clinical and radiological following, whereas other favour a preventive surgical excision. The origin of CCAM remains unknown. Recent advances suggest a transient and focal abnormality in lung development which may result from an airway obstruction. This article reviews the diagnosis, treatment, and pathophysiology of CCAM.

Echogenic and Cystic Fetal Lung Lesions: Evaluation by Prenatal Sonography and Postnatal Imaging

Echogenic and Cystic Fetal Lung Lesions: Evaluation by Prenatal Sonography and Postnatal Imaging