Echogenic Lung Research Articles

PF.67 The Increased Incidence of Echogenic Lung Lesions – an 18-Year Review from the Wessex Region

IntroductionEchogenic lung lesions (ELL) are a heterogeneous group of lung abnormalities that display a variety of features and are inherently difficult to diagnose and characterise antenatally. Included in this group...

Prediction of pulmonary hypoplasia in mid‐trimester preterm prelabor rupture of membranes: research or clinical practice?

Prediction of pulmonary hypoplasia in mid‐trimester preterm prelabor rupture of membranes: research or clinical practice?

A case of congenital high airway obstruction syndrome caused by complete tracheal obstruction with associated anomalies

An obstruction of the fetal upper airway in utero due to various reasons may cause a fatal condition congenital high airway obstruction syndrome (CHAOS). The prenatal ultrasound fi ndings of CHAOS include large echogenic lungs, fl attened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops [1]. If it is associated with severe hydrops and/ or associated anomalies, its prognosis is fatal. Here we present a case of antenatally diagnosed CHAOS with associated anomalies and a literature review.

Congenital lobar emphysema with cystic lung abnormality: Antenatal ultrasound appearance

+2 weeks of gestational age was referred for consultation due to the findings of a cyst in the right lung of the fetus. Prenatal ultrasound showed a 3 cm-sized hypoechogenic cyst, suggesting congenital cystic adenomatoid malformation. As the cyst grew and compressed the heart, serial cyst aspirations were performed. The cyst was proved to be congenital lobar emphysema on postnatal histopathologic exam. No other anomaly was found. Only few cases of congenital lobar emphysema (CLE) have been reported up to date and the prenatal diagnosis is extremely rare. Typically, fetal sonographic features of CLE have a bright echogenic lung and, to date, only 3 cases have been reported to have a cystic lesion. We report a case of a fetus with a cystic lung abnormality and postnatally proved congenital lobar emphysema.

Prenatal Diagnosis and Pathology of Laryngeal Atresia in Congenital High Airway Obstruction Syndrome

Congenital high airway obstruction syndrome is a rare but life-threatening condition. Therefore, prenatal diagnosis is important. The obstruction can be due to laryngeal/tracheal atresia or external compression. While a differential diagnosis with congenital cystic adenomatoid malformation (CCAM) type III may be difficult, it is still possible with ultrasonography. In this study, we report a case of bilateral echogenic lungs with hydrops fetalis. After the prenatal diagnosis of laryngeal atresia, the couple opted to have an elective termination of pregnancy performed at 20 weeks of gestation. The diagnosis was confirmed by a complete pathological examination.

Alveolar capillary dysplasia associated with duodenal atresia: Ultrasonographic findings of enlarged, highly echogenic lungs and gastric dilatation in a third‐trimester fetus

We report a case of alveolar capillary dysplasia, wherein duodenal atresia was diagnosed during the third trimester. A 36-year-old mother was referred to our hospital for polyhydramnios at 31 weeks' gestation. Duodenal atresia was suspected from the ultrasonographic findings, which showed gastric dilation. Other findings noted were enlarged, highly echogenic lungs, a spherical heart and an increased lung-thorax transverse area ratio. A male infant was born at 37 weeks' gestation. The findings of serial radiography of the infant's upper gastrointestinal tract were compatible with the diagnosis of duodenal atresia; however, he developed persistent pulmonary hypertension of the newborn eight hours after birth and died at five days of age. The autopsy revealed alveolar capillary dysplasia and duodenal obstruction. We propose that the detection of duodenal atresia should prompt the physician to conduct a thorough ultrasonographic examination to rule out associated anomalies, such as alveolar capillary dysplasia, which can be detected by the presence of highly echogenic lungs.

Challenges in the diagnosis of fetal non‐chromosomal abnormalities at 11–13 weeks

To examine the performance of the 11-13 weeks scan in detecting non-chromosomal abnormalities. Prospective first-trimester screening study for aneuploidies, including basic examination of the fetal anatomy, in 45 191 pregnancies. Findings were compared to those at 20-23 weeks and postnatal examination. Aneuploidies (n = 332) were excluded from the analysis. Fetal abnormalities were observed in 488 (1.1%) of the remaining 44 859 cases; 213 (43.6%) of these were detected at 11-13 weeks. The early scan detected all cases of acrania, alobar holoprosencephaly, exomphalos, gastroschisis, megacystis and body stalk anomaly, 77% of absent hand or foot, 50% of diaphragmatic hernia, 50% of lethal skeletal dysplasias, 60% of polydactyly, 34% of major cardiac defects, 5% of facial clefts and 14% of open spina bifida, but none of agenesis of the corpus callosum, cerebellar or vermian hypoplasia, echogenic lung lesions, bowel obstruction, most renal defects or talipes. Nuchal translucency (NT) was above the 95th percentile in 34% of fetuses with major cardiac defects. At 11-13 weeks some abnormalities are always detectable, some can never be and others are potentially detectable depending on their association with increased NT, the phenotypic expression of the abnormality with gestation and the objectives set for such a scan.

P20.09: Mesenchymal tumor of the liver and placental mesenchymal dysplasia in a patient with pregnancy induced hypertension: a rare case report

A 29 year old with suspected monochorionic diamniotic twins presented for a routine scan at 18+2 weeks gestation. Findings were of bilateral echogenic lungs in both fetuses and CCAM was the provisional diagnosis. On further review in a tertiary centre at 20 weeks, the combination of large, universally echogenic lungs with everted diaphragms raised the possibility of CHAOS (congenital high airway obstruction syndrome). Fetal MRI confirmed atretic segments of trachea at similar levels in each of the twins with lung signals not typical of CCAMs. Serial scans revealed concordant truncus arteriosus/VSD and although the patient had initially refused invasive testing, samples sent at the time of a dual amnio-reduction for symptomatic polyhydramnios at 26 weeks, revealed normal 46XY karyotypes. An EXIT procedure at Caesarean section was planned, but intervention was restricted to a gestation of >34 weeks due to the poor prognosis associated with prematurity in CHAOS. Unfortunately the mother developed obstructive uropathy, with significant renal dysfunction, triggering spontaneous preterm labour at 30+ weeks. Despite senior neonatal staff being present at delivery, neither fetus could be intubated due to tracheal obstruction and they both died shortly afterwards. Post-mortem was declined but postnatal MRI provided clearer images of the tracheal atresia.

P20.11: Fetal teratoma—the role of fetal MRI to plan the management of the patient

A 29 year old with suspected monochorionic diamniotic twins presented for a routine scan at 18+2 weeks gestation. Findings were of bilateral echogenic lungs in both fetuses and CCAM was the provisional diagnosis. On further review in a tertiary centre at 20 weeks, the combination of large, universally echogenic lungs with everted diaphragms raised the possibility of CHAOS (congenital high airway obstruction syndrome). Fetal MRI confirmed atretic segments of trachea at similar levels in each of the twins with lung signals not typical of CCAMs. Serial scans revealed concordant truncus arteriosus/VSD and although the patient had initially refused invasive testing, samples sent at the time of a dual amnio-reduction for symptomatic polyhydramnios at 26 weeks, revealed normal 46XY karyotypes. An EXIT procedure at Caesarean section was planned, but intervention was restricted to a gestation of >34 weeks due to the poor prognosis associated with prematurity in CHAOS. Unfortunately the mother developed obstructive uropathy, with significant renal dysfunction, triggering spontaneous preterm labour at 30+ weeks. Despite senior neonatal staff being present at delivery, neither fetus could be intubated due to tracheal obstruction and they both died shortly afterwards. Post-mortem was declined but postnatal MRI provided clearer images of the tracheal atresia.

P20.08: Monochorionic twin pregnancy complicated by concordant CHAOS, truncus arteriosus and VSD

A 29 year old with suspected monochorionic diamniotic twins presented for a routine scan at 18+2 weeks gestation. Findings were of bilateral echogenic lungs in both fetuses and CCAM was the provisional diagnosis. On further review in a tertiary centre at 20 weeks, the combination of large, universally echogenic lungs with everted diaphragms raised the possibility of CHAOS (congenital high airway obstruction syndrome). Fetal MRI confirmed atretic segments of trachea at similar levels in each of the twins with lung signals not typical of CCAMs. Serial scans revealed concordant truncus arteriosus/VSD and although the patient had initially refused invasive testing, samples sent at the time of a dual amnio-reduction for symptomatic polyhydramnios at 26 weeks, revealed normal 46XY karyotypes. An EXIT procedure at Caesarean section was planned, but intervention was restricted to a gestation of >34 weeks due to the poor prognosis associated with prematurity in CHAOS. Unfortunately the mother developed obstructive uropathy, with significant renal dysfunction, triggering spontaneous preterm labour at 30+ weeks. Despite senior neonatal staff being present at delivery, neither fetus could be intubated due to tracheal obstruction and they both died shortly afterwards. Post-mortem was declined but postnatal MRI provided clearer images of the tracheal atresia.

Successful Percutaneous Embolization of Feeding Vessels of a Lung Tumor in a Hydropic Fetus

Fetuses with large lung tumors associated with hydrops have an almost uniformly fatal outcome if left untreated. Indeed, 2 large referral centers in America have reported a perinatal mortality rate approaching 100% in more than 50 hydropic fetuses with lung tumors managed expectantly. 1-3 Fetal intervention, either with open fetal surgery 1-3 or with percutaneous shunting/ablation techniques, 4 can improve the survival rate, but these approaches have only being carried out in few selected cases. We report the successful percutaneous intrauterine embolization of a large echogenic lung mass in a hydropic fetus at 22 weeks' gestation. The prenatal sonographic findings, antenatal course, and perinatal management are presented.

Prenatal findings and differential diagnosis of scimitar syndrome and pulmonary sequestration

Scimitar syndrome and pulmonary sequestration (PS) have overlapping features. This accounts for postnatal descriptions of a 'sequestrated lung' in scimitar-syndrome patients. We review the similarities and differences in these two conditions, and the antenatal ultrasound findings that allow correct prenatal identification and differential diagnosis. This was a retrospective analysis of prenatally diagnosed cases of scimitar syndrome or PS that underwent fetal echocardiography between January 1995 and November 2004. There were five cases of PS and six of scimitar syndrome. Right-sided mediastinal shift (ipsilateral relative to the affected lung) was the commonest indication for referral in scimitar syndrome whereas in sequestration, referral was because of hyperechogenic lung and contralateral mediastinal shift. Lung echogenicity was normal in scimitar syndrome but focally increased in PS. Abnormal, unobstructed pulmonary venous drainage was identified prenatally in four cases of scimitar syndrome. It was normal in three fetuses with sequestration but abnormal and obstructed in the other two cases complicated by hydrothorax. Abnormal systemic arterial supply to the affected lung was easily demonstrated in all fetuses with PS, but could not be shown prenatally in scimitar-syndrome fetuses. Postnatal embolization was warranted in one case with PS and four with scimitar syndrome, one each in the neonatal period. Prenatally, scimitar syndrome and PS are clearly distinct entities based on lung echogenicity and laterality of mediastinal shift. Hyperechogenic lung and dextrocardia indicate the possibility of PS and scimitar syndrome, respectively. Complete delineation of the vascular pattern should be attempted in all, but is more challenging in scimitar syndrome. Obstructed venous return in PS may identify fetuses at risk of developing hydrothorax.

OC12.06 Perinatal outcomes of fetal echogenic lung lesions: an Australian perspective

OC12.06 Perinatal outcomes of fetal echogenic lung lesions: an Australian perspective

P17.06: Congenital megalourethra-sonographic features & outcome

Materials and Methods: A collaborative study initiated by the College Francais d’Echographie Foetale (CFEF) assemble 7 laryngeal atresias. We drawn up a questioning to reach our objectives. Results: Median maternal age was 26 years, sex ratio = 1, gestational age at discovery was 22 W.+/−3 W. All ultrasound scan findings were related in a table. We found a high frequency (6/7) of large echogenic lungs, tracheal dilatation, inverted or flattened diaphragm, and compressed median heart. Ascites, polyhydramnios (5/7), subcutaneous edema (4/7) and moderate pleural bleeding (2/7) were noted. All fetuses have normal karyotype. Associated malformations were found: 1 Fraser syndrome, 1 IUGR wilt oesophagus stenosis and cryptorchidia, 1 supernumerary vessel connecting the pulmonary artery to the left atrium, with single pulmonary vein. Also a mono-chorionic, bi-amniotic twin pregnancy was described: T2 deceased to D21(Leucomalacia). Four fetuses were terminated. One died intra-partum. Two deceased during the post-natal period. Autopsy and/or bronchoscopy were performed in six cases. The obstruction level was performed; almost always type 2 of the classification by Smith and Bain. The atypical case was in relationship with a probable eso-tracheal fistula. Conclusion: Prenatal diagnosis of laryngeal atresia can be performed in prenatal. The ultrasound semiology is almost constant. The prognosis in our series is poor. In case of no other malformation an EXIT (EX utero Intrapartum Treatment) procedure could be proposed to parents after correctly information.

Prenatal Diagnosis of Congenital Lobar Emphysema

Congenital lobar emphysema (CLE) is a rare developmental anomaly of the lower respiratory tract, which is characterized by hyperinflation of 1 or more of the pulmonary lobes. The routine use of prenatal sonography has resulted in early identification and serial evaluation of congenital lung lesions. 1 Prenatal diagnosis of CLE is rarely reported in the literature. 2 Here we report a case of a fetus with an echogenic lung presenting in the second trimester suspected of CLE. A discussion of the causes and natural course of CLE is presented.

Prenatal diagnosis and outcome of echogenic fetal lung lesions

To describe the antenatal findings and outcome of fetuses with echogenic lung lesions. 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. 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. 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.

Case report: Antenatal diagnosis of congenital high airway obstruction syndrome - laryngeal atresia

Congenital high airway obstruction syndrome (CHAOS) is a near fatal condition of multifactorial inheritence, in which the fetus has a dilated trachea, enlarged echogenic lungs, an inverted or flattened diaphagram, and ascites. A case of CHAOS, diagnosed antenatally on USG at 28 weeks of gestation, is being reported here.

OC156: A longitudinal study of the intrauterine growth of echogenic fetal lung masses

OC156: A longitudinal study of the intrauterine growth of echogenic fetal lung masses

Prenatal diagnosis of congenital cystic adenomatoid malformation using three‐dimensional inversion rendering: A case report

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.

OP02.04: Prenatal intervention in fetuses with severe congenital diaphragmatic hernia and congenital cystic adenomatoid malformation

Objectives: The natural history of echogenic lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high incidence of adverse features (hydrops) and a poor outcome and did not accord with recent experience. Our purpose was to review the evolution and outcome of these lesions in our series. Methods: A retrospective review was conducted on fetuses diagnosed antenatally in the Fetal Medicine Unit of Hospital Vall d Hebron in Barcelona, Spain, between 2001 and 2007. A computer search identified all referred cases, and the records of these patients were examined to determine the pregnancy outcome. Results: In a 6-year period, 34 fetuses with echogenic lung lesions were referred for further management. The median age at diagnosis was 23 (range, 20–41) weeks. The lesion was on the right side in 19 (55%) and 15 (45%). All cases were unilateral. One case was diagnosed as CCAM type I (3%), 14 CCAM type II (41%) 11 CCAM type III (32%), 3 PS (8.8%) and five were mixed PS/CCAM (15%). All the fetuses had no associated anomalies and normal karyotype except one (47 XX+18). Termination of pregnancy was performed in seven cases of CCAM. In all cases histologic examination showed definitive diagnostic features of CCAM. In two cases diagnostic type of CCAM was changed from CCAM type II to type I and from CCAM type III to type II. Mediastinal shift was present in 16 fetuses (47%). Severe signs of fetal distress (hydrops) were present in three (8.8%). Because of hydrops, antenatal intervention was performed in two fetuses (thoracoamniotic shunting) and it was resolved. During the course of the pregnancy 70.5% (24/34) cases reduced in size or resolved spontaneously. All cases had good perinatal outcome. Conclusions: Most cases of prenatally diagnosed echogenic lung lesions had a good outcome. This review has positively influenced the counseling of women with this diagnosis.