Echogenic Lung Research Articles

Antenatal diagnosis of congenital pulmonary airway malformation – two case report

BackgroundCongenital Pulmonary Airway Malformation (CPAM), is the most common Congenital Thoracic Malformation, accounting for 95% of congenital echogenic lung lesions detected antenatally on ultrasonography (USG).
 Antenatally diagnosed CPAM may regress or result in hydrops fetalis. Management options include expectant fetal follow-up, maternal administration of steroids and fetal surgery. We present two cases of antenatally diagnosed CPAM with both mothers consenting to and receiving antenatal steroids.
 Case 1
 A 31-year-old primigravida was noted with homogeneous highly hyperechoic right lung mass measuring 3.0 x 3.1 x 2.5cm at 24 weeks gestation on USG. The mass-to-thorax ratio (MTR) was 0.72, with no systemic feeding artery noted, stomach bubble was normally situated, there was no hydrops. An initial diagnosis of CPAM of the right lung was made. At 38 weeks’ gestation, USG showed mass was unmeasurable. Delivery was via CS at 39 weeks. Baby was well at birth and discharged home after 48 hours of monitoring, baby is 4 months old and growing well
 Case 2
 A 33-year-old G4P3AA was diagnosed with CPAM on USG at 22 weeks’ gestation. There was displacement of fetal heart to the right hemi-thorax. Colour Doppler did not show a systemic feeding arterial supply, or hydrops, and no associated structural anomaly. Serial USG for MTR at 28- and 32-weeks’ gestation showed increase of 0.72 and 0.86 respectively, with polyhydramnios. Further USG noted MTR of 0.57. Delivery was by emergency caesarean section (CS) for prolonged labour at term. Baby required admission due to hypoxia and respiratory distress on the first day of life, baby was discharged on day 14 post-delivery. Chest computed tomographic angiogram noted a feeding vessel to part of the right lung. Patient is 6 months old and has undergone a successful surgery.
 Conclusion
 Antenatal USG diagnosis and multidisciplinary approach to management in Congenital Thoracic Malformations is essential and improves antenatal and post-natal outcomes.

Ultrasound Differential Diagnosis and Prognosis of Right Main Bronchus Atresia in Fetus: Clinical Case

Background. Obstructive lesion of upper respiratory tract in fetus is extremely rare pathology with adverse perinatal outcomes.Clinical Case Description. Ultrasound examination of fetus (gestational age 21 weeks 6 days) has revealed one-sided isolated main bronchus atresia. Atresia was presented as enlargement of right lung due to mucus accumulation, its increased echogenicity, mediastinal displacement to the left and left lung size reduction. We performed differential diagnosis with cystic-adenomatous lung malformation type III, congenital lobar emphysema and pulmonary sequestration. These conditions are also characterized by lung echogenicity and volume increase but they are usually limited to one lung lobe or segment, and pulmonary sequestration has a systemic blood supply. The use of high-frequency transducers, lung vessels Doppler imaging and volume echography allow us to investigate in detail the lung structure of the fetus, correctly calculate the lungs volume and timely the main bronchus atresia at the second trimester of pregnancy.Conclusion. The ultrasound diagnosis of isolated main bronchus atresia is based on revealing of increased lung volume on ipsilateral side, its increased echogenicity, presence of hypoechogenic linear tubular structures (bronchocele), mediastinal displacement to the opposite side and extreme hypoplasia of the contralateral lung. Prognosis for the life is unfavorable.

Values and validity of fetal parameters by ultrasound and Doppler as markers of fetal lung maturity

BackgroundIn this study, we combined ultrasound and Doppler parameters to conclude the most accurate and applicable method for fetal lung maturity assessment. Thepurpose is to reduce risk of neonatal respiratory distress by assessment of fetal lung maturity through ultrasound and Doppler instead of amniocentesis.ResultsA total of 120 women were included. The ultrasound examination was performed at a gestational age ranging between 30 and 37 weeks and delivery occurred within 72 h from ultrasound. After birth, 19 fetuses were diagnosed with RDS.The ultrasound parameters assessed for fetal lung maturity showed varying sensitivity and specificity but presence of proximal tibial epiphyses showed the highest sensitivity (91%) and specificity (95%) followed by the presence of distal femoral epiphyses with 90% sensitivity and 84% specificity. The placental grade III maturity showed a sensitivity of 81% and 74% specificity followed by the presence of the amniotic fluid free-floating particles with an 83% sensitivity and 63% specificity, while echogenic fetal thalamus showed 77% and 79% for sensitivity and specificity, respectively, for prediction of fetal lung maturity. By using the fetal biometry, we could report that a BPD between 82.8 and 93.5 mm, AC between 295 and 322 mm, and FL (62.7-72.1 mm) correlated with mature fetal lungs. Regarding Doppler indices, increased resistive index of umbilical artery > 0.69 and of pulmonary artery > 0.78 correlated with neonatal RDS while fetuses with UtA RI values between 0.43 and 0.66 noticed in RDS vs (0.42-0.57) in the non-RDS. MCA RI values are also higher in RDS 0.77–0.88 vs 0.74–0.79 in non-RDS.On the other hand, analysis of colon grading and fetal lung echogenicity in relation to liver showed no significant value in the assessment of fetal lung maturity.ConclusionUltrasound and Doppler showed good predictive value and accuracy and is considered a valuable non-invasive procedure in assessment of fetal lung maturity. However, no single parameter alone could show a definitive sign

Fetoscopic Balloon Dilation and Cricotracheal Resection for Laryngeal Atresia in CHAOS.

View Supplementary Video 1 Laryngoscope, 131:660–662, 2021

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.

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.

Congenital Lobar Overinflation: A Rare Enigmatic Lung Lesion on Prenatal Ultrasound and Magnetic Resonance Imaging.

To report the ultrasound (US) features in prenatal cases of suspected congenital pulmonary airway malformation or unspecified lung lesions with a final surgical pathologic diagnosis of congenital lobar overinflation (CLO). Institutional Review Board-approved radiology and clinical database searches from 2001 to 2017 were performed for prenatally diagnosed lung lesions with a final diagnosis of CLO. All patients had detailed US examinations in addition to magnetic resonance imaging (MRI). Size, echotexture, and vascularity were assessed with US, and the signal and vascularity were assessed with MRI. Follow-up prenatal US scans, postnatal imaging, and postnatal outcomes were reviewed. The study population consisted of 12 patients. The median gestational age was 23.3 weeks. The median congenital pulmonary airway malformation volume-to-head circumference ratio was 0.66. Lesion locations were 6 in the lower lobes (4 right and 2 left), 5 in the upper lobes (3 left and 2 right), and 1 in the right middle lobe. The texture was homogeneously echogenic relative to the normal lung in 100% with no visualized macrocysts. Hypervascularity by color Doppler US was observed in 5 cases (41.7%). A T2 hyperintense lung lesion was identified by MRI in 12 of 12 cases (100%), with elongated vessels identified in 11 of 12 cases (91.7%). All 12 cases had pathologically proven CLO. Congenital lobar overinflation should be considered in cases of prenatal echogenic lung lesions without macrocysts or classic findings of bronchial atresia. Hypervascularity may be an important imaging feature of a subset of CLO. Most cases become less conspicuous, decrease in size without overt hydrops, and are asymptomatic postnatally.

Congenital High Airway Obstruction Syndrome: Antenatal Ultrasound Findings

Abstract Objective: The objective of this study was to describe the antenatal ultrasound appearances of congenital high airway obstruction syndrome (CHAOS), which typically manifests as large echogenic lungs acquiring whole of the thorax and dilatation of the trachea distal to the obstruction and causing intrinsic laryngeal/tracheal obstruction. Case report: A 30-year-old pregnant female with 24 weeks gestation come for routine absolute neutrophil count (ANC) scan. Ultrasonography (USG) was performed, and large echogenic lungs occupying whole of the thoracic cavity compressing the heart in between and causing inversion of the bilateral domes of the diaphragm were found. There was dilatation of the tracheal distal to the lesion and polyhydramnios. Conclusion: The described USG finding of CHAOS may be useful in deciding when to include this entity in the differential diagnosis of enlarged echogenic lungs.

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.

CHAOS: Prenatal imaging findings with post mortem contrast radiographic correlation.

Congenital high airway obstruction syndrome is a rare fetal anomaly with characteristic constellation of prenatal findings on ultrasound and MRI. The typical triad of imaging features are enlarged and echogenic lungs, flattening or inversion of diaphragm and fetal hydrops. Early prenatal recognition of congenital high airway obstruction syndrome by ultrasound and/or MRI is mandatory for the appropriate perinatal management. We report a case of a male fetus with typical imaging findings of congenital high airway obstruction syndrome on ultrasound and MRI at 19 weeks of gestation. The role of contrast radiographs of fetal airways, including retrograde laryngogram, in confirming the postnatal diagnosis of this fetal condition is demonstrated. The prenatal imaging findings were correlated with contrast radiographs of upper airways, sonography of aborted fetus and fetal autopsy findings.

Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes.

The congenital pulmonary airway malformation volume ratio (CVR) is a widely used sonographic measure of relative mass size in fetuses with lung malformations. The purposes of this study were to examine serial CVR measurements to understand longitudinal growth patterns and to determine correlation with postnatal imaging. An institutional review board-approved retrospective review was performed on fetuses referred for an echogenic lung malformation between 2002 and 2014. For each fetus, the CVR was prospectively calculated using 2D ultrasound and followed with advancing gestation. Based on 40 fetuses, the mean initial CVR was 0.51 ± 0.07 at 20.5 ± 0.3 weeks of gestation. The CVR increased after 24 weeks of gestation (p = 0.0014), peaking at a CVR of 0.96 ± 0.11 at 25.5 ± 0.05 weeks, followed by a significant decrease in the CVR to 0.43 ± 0.07 prior to term (p < 0.0001). However, approximately one third showed no appreciable increase in size. The mean CVR was significantly correlated with postnatal chest computed tomography (CT) size dimensions (p = 0.0032) and likelihood for lung resection (p = 0.0055). Fetal lung malformations tend to follow one of two distinct growth patterns, characterized by either (1) a maximal CVR between 25 and 26 weeks of gestation or (2) minimal change in relative growth. The mean CVR correlates with postnatal CT size and operative management.

Congenital high airway obstruction syndrome (CHAOS): discussing the role and limits of prenatal diagnosis starting from a single-center case series.

we aimed to report our experience about congenital high airway obstruction syndrome (CHAOS) that is a rare and fatal congenital anomaly; laryngeal atresia is the most frequent cause. Sonographic findings are enlarged echogenic lungs, dilated trachea, and ascites. we performed a single-center case series analysis collecting antenatally through ultrasound examination, and some of them confirmed by autopsy. we report six cases of CHAOS diagnosed by antenatal ultrasonography between 2007 and 2013. to date literature provides very few individual case reports. This work describes typical sonographic findings of this syndrome and it underlines the importance of early prenatal diagnosis to improve prognosis thought an ex utero intrapartum treatment, that seems to be the only chance of survival for the affected fetus.

Prenatal diagnosis of Fraser syndrome: a matter of life or death?

BackgroundFraser Syndrome is a rare, autosomal recessive syndrome. It’s characterized primarily by cryptophthalmos, syndactyly and urogenital malformation. Respiratory malformations are frequently present and not taken into account. To better manage childbirth at the time of delivery it is crucial to get prenatal diagnosis early on in the pregnancy.Case presentationWe are reporting a female infant born by natural birth with 46,XX. She was characterized phenotypically by cryptophthalmos, syndactyly, bilateral microtia and ambiguous genitalia. A prenatal ultrasound didn’t revealed or raised any suspects for the Fraser Syndrome. It only discovered a unilateral kidney agenesis. At birth the infant showed a severe respiratory distress, intubation was attempted but it failed. The baby was transferred to Santobono-Pausilipon III level hospital. A tracheostomy was performed successfully and saved her life. Computerized Tomography revealed left microphthalmos and a malformation like-coloboma into right ocular globe with cysts and a small calcification parietal anterior. Genetic test revealed the typical mutations in the gene FREM2 confirming the diagnosis of Fraser Syndrome. In her fourth month, after birth, the infant was subjected to an operation to reconstruct eyelids with a mucous membrane graft. The left renal function was normal. The baby showed a delay in motor milestones for visual impairment. At the 19th month fallow-up, during a magnetic resonance it was revealed: a normal morphologic brain development, a thin presence in the right optic nerve and the visual cortex were developing.ConclusionsThe prenatal diagnosis of Fraser Syndrome is frequently possible. The prenatal ultrasound can reveal features like polyhydramnios or oligohydramnios, echogenic lungs, renal abnormalities or agenesis and cryptophthalmos that are pathognomonic of the Fraser Syndrome. The health providers must keep in mind that if there are suspects of the Fraser Syndrome during prenatal exams, the infants could have a severe malformation in the respiratory tract.

P17.08: Outcome of prenatal diagnosis of multilobar peripheral bronchial atresia mimicking large echogenic lung mass

P17.08: Outcome of prenatal diagnosis of multilobar peripheral bronchial atresia mimicking large echogenic lung mass

EP09.08: Prenatal diagnosis and outcome of fetuses with echogenic lung masses

To describe the antenatal ultrasound findings and outcome of fetuses with echogenic lung masses. Ten fetuses with echogenic lung masses suspected prenatally by ultrasound were observed in a total of 2916 examinations performed in our unit from 2011 up to 2014. Eleven cases of congenital diaphragmatic hernia were excluded. There were three cases of congenital pulmonary airway malformation (CPAM). Five cases of pulmonary sequestration (PS), three of them were considered to have also CPAM. One case of congenital high airway obstruction syndrome (CHAOS) and one case of unilateral bronchial obstruction (UBO). In all cases, a displacement of the heart and abnormal lung was observed and presence of an aberrant vessel rising from the descending aorta was seen in all PS cases. In 8 cases, magnetic resonance imaging (MRI) was performed, in 7/8 helped to clarify the diagnosis. The prenatal management in all cases was expectant and in cases of CPAM and PS + CPAM, a course of corticosteroids was administered. We observed prenatal remission of the lung mass in 6 cases (complete: 4 and partial: 2). One case of PS had hydrothorax, which decreased completely along gestation. The fetus with CHAOS develop hydrops and died in utero. The fetus with UBO had a right aortic arch and during pregnancy the obstruction decreases and the lung recovered to its normal size. The fetuses with echogenic lung masses is a rare congenital abnormality (1/1,000 birth). Clinical diagnosis can be suspected by US, but the final classification may be difficult and others diagnostic tools (MRI and histology) should be employed. In most cases prognosis is good (even in a case with PS and hydrothorax), except in CHAOS. Corticosteroid therapy should be offered in cases of CPAM and PS (mixed).

Term Infant with Echogenic Fetal Lung Mass on Prenatal Sonogram

Postnatal management of infant with prenatally diagnosed fetus with Echogenic fetal lung depends on the infant’s system at the time of delivery. Infant with large lesions can present with symptomatology at birth whereas a symptomatic infant with normal X-ray requires CT scan or more advance MRI studies between 4-6 weeks prior to discharge from birth facility.

Congenital High Airway Obstruction Syndrome (CHAOS) � A Rare Fetal Malformation Diagnosed on Antenatal Ultrasonography

Introduction Congenital high airway obstruction syndrome is rare fetal malformation caused by obstruction of fetal airway because of laryngeal or tracheal atresia, subglottic stenosis, laryngeal cyst or laryngeal web. This clinical condition was brought into notice first by Hedrick in the late 1900 [1]. CHAOS is usually caused by atresia or stenosis of the larynx or trachea. The true incidence of CHAOS is unknown. It is important to diagnose it antenatally as it is uniformly lethal. If the syndrome is unrecognized during the prenatal period, it usually results in stillbirth or death shortly after delivery [2]. The prenatal ultrasound findings of CHAOS include large echogenic lungs, flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops [3]. Here we present a rare case of CHAOS diagnosed on the antenatal scan. Case Blog A 22 year old primi-gravida was referred for routine anomaly scan at 18 weeks of gestation. There was no history of consanguinity and the family history was unremarkable. Ultrasound examination revealed a single live intrauterine fetus corresponding to a gestational age of 18 weeks. Amniotic fluid was within normal limits. Bilateral lungs were echogenic and enlarged with small heart compressed in between the lungs (Figure 1), dilated bronchi and trachea (Figure 2), mild ascites (Figure 3), and flattened diaphragm (Figure 4). Based on these findings, the diagnosis of CHAOS was made. Patient and her family members were counselled regarding the possible unfavourable outcome of the pregnancy. The patient opted for termination of pregnancy.

English

Congenital high airway obstruction syndrome (CHAOS) results in a predictable constellation of findings: large echogenic lungs flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops. 1 The finding of CHAOS on prenatal ultrasound examination is diagnostic of complete or near-complete obstruction of the fetal upper airway, most likely caused by laryngeal atresia. A greater understanding of the natural history of CHAOS may permit improved prenatal and perinatal management. INTRODUCTION: Congenital chest malformations are rare, and may involve the lung parenchyma, bronchi, arterial supply, and venous drainage. They include congenital cystic adenomatoid malformation (CCAM), congenital diaphragmatic hernia (CDH), bronchopulmonary sequestration (BPS), congenital hydrothorax, and congenital lobar emphysema. Rarer entities include congenital high airway obstruction syndrome (CHAOS), congenital bronchogenic cyst, bronchial atresia, pulmonary Arterio Venous malformation (PAVM), congenital pulmonary lymphangiectasia, pulmo- nary hypoplasia-aplasia, mediastinal teratoma, and mediastinal lymphangioma.2 Sometimes there may be compromise in normal pulmonary development, causing hypoplastic lung formation. Pulmonary abnormalities are not mutually exclusive, since abnormalities frequently occur together as hybrid conditions. MATERIALS AND METHODS: Here we present a case of a fetus post-delivery found to have large hyperechoic lungs, flattened diaphragm and hydrops in the prenatal scan, which form a triad in the detection of CHAOS. A brief review of literature is discussed. CASE PRESENTATION: In our case we had a twenty six years old pregnant lady with twenty two weeks history of amenorrohea who had come for a routine antenatal scan. She was the mother of a two year old child which was born normally and was doing well. On antenatal ultrasound of the present pregnancy, the fetus was found to be of approximately twenty weeks gestation. The fetus was found to have large hyperechoic lungs, flattened diaphragm and hydrops which form a triad in the detection of CHAOS. (Fig: 1) The patient and her husband were counseled regarding the risks involved on progression of the fetus till birth. The parents agreed to terminate the pregnancy voluntarily. A dead male fetus was delivered and sent for autopsy subsequently. An interesting finding noted during dissection of neck and thoracic organs was an abrupt ending of the trachea approximately 11 mm caudal to the lower end of larynx. (Fig: 2 & Fig: 3). The subsequent histopathology showing swollen cells confirmed the diagnosis (Fig: 4)

Review on Current Management and Outcome Data of Echogenic Lung Lesions and Hydrothorax of the Fetus

This review focuses on the prenatal management and outcome of echogenic lung lesions and isolated hydrothorax of the fetus. We give an overview of the most common forms of echogenic lung lesions like cystic adenomatoid malformation of the lung and bronchopulmonary sequestration as well as of congenital high airway obstruction sequence. We review the occurrence, appearance, pathophysiology and natural history of these lesions. Furthermore we discuss selection criteria for intrauterine treatment and algorithms for prenatal surveillance of affected fetuses.

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.