Enlarged cavum septum pellucidum and small thymus as markers for 22q11.2 deletion syndrome.

BACKGROUND: Enlarged cavum septum pellucidum (CSP) and hypoplastic thymus are proposed extra-cardiac fetal markers for 22q11.2 deletion syndrome. We sought to determine if they were part of the fetal phenotype of our cohort of fetuses with 22q11.2 deletion syndrome. METHODS: Case-control study of fetuses evaluated from 2016 to 2022. The study group included fetuses with laboratory confirmation of 22q11.2 deletion syndrome. The control group included pregnancies with conotruncal cardiac anomalies with normal microarray as well as structurally normal fetuses with normal microarray. The CSP and thymus were routinely measured during anatomical ultrasound in all patients at their initial visit at 27.1 ± 4.7 weeks. The CSP and thymus measurements were classified as abnormal if they were >95% or <5% for gestational age, respectively. The groups were compared using analysis of variance or Kruskal-Wallis for continuous variables and Fisher's exact test for categorical variables. Logistic regression was performed, and a Receiver Operating Characteristic (ROC) curve was constructed. RESULTS: We identified 47 fetuses with 22q11.2 deletion syndrome and compared them to 47 fetuses with conotruncal anomalies and normal microarray and 47 structurally normal fetuses with normal microarray. 51% (24/47) of fetuses with 22q11.2 deletion syndrome had an enlarged CSP compared to 6% (3/47) of fetuses with a conotruncal anomaly and normal microarray and none of the structurally normal fetuses (p < 0.001). Of the fetuses with 22q11.2 deletion syndrome, 83% (39/47) had a hypoplastic or absent thymus compared to 9% (4/47) of the fetuses with a conotruncal anomaly and normal microarray and none of the structurally normal fetuses (p < 0.001). 87% (41/47) of the fetuses with 22q11.2 deletion syndrome had conotruncal cardiac anomalies. Logistic regression revealed that both enlarged CSP and hypoplastic/absent thymus were associated with 22q11.2 deletion syndrome. The area under the ROC curve for the two markers was 0.94. CONCLUSION: An enlarged CSP and hypoplastic/absent thymus appear to be part of the fetal phenotype of 22q11.2 deletion syndrome. These markers are associated with conotruncal anomalies in the setting of 22q11.2 deletion syndrome but not in normal controls or fetuses with conotruncal defects and normal microarrays.

Magnetic resonance neuroimaging after laser for twin-twin transfusion syndrome with single fetal demise.

BACKGROUND: Neurologic injury in the surviving twin is a risk after single fetal demise in a monochorionic pregnancy. OBJECTIVE: This study aimed to describe fetal magnetic resonance neuroimaging findings in pregnancies complicated by single fetal demise after laser photocoagulation for twin-twin transfusion syndrome. STUDY DESIGN: This was a single-center retrospective analysis of a cohort of prospectively collected patients in a monochorionic twin registry who had fetoscopic laser photocoagulation for twin-twin transfusion syndrome with single fetal demise at follow-up. Magnetic resonance neuroimaging was offered 3 to 4 weeks after the demise to assess for potential neurologic sequelae. Magnetic resonance images were interpreted by 2 board-certified neuroradiologists and classified as normal, mildly abnormal, or severely abnormal. The groups were compared on the basis of recipient vs donor demise using the Fisher exact test and Mann-Whitney U test. Multivariate logistic regression was performed to determine risk factors for abnormal magnetic resonance neuroimaging. RESULTS: In 378 laser photocoagulation procedures, 64 cases (16.9%) of single demise were identified (36 in the donor group and 28 in the recipient group). Of note, 6 patients had rupture of membranes with nonviable delivery (3 from each group). Moreover, 40 patients (69%) underwent magnetic resonance imaging. Of those patients, 12 (30%) had abnormal findings: 10 (83%) were associated with mild changes, and 2 (17%) were associated with severe findings. Abnormal magnetic resonance neuroimaging was seen in 3 of 22 patients (14%) after donor demise and 9 of 18 patients (50%) after recipient demise (P=.02). Logistic regression revealed that recipient vs donor demise was an independent risk factor for abnormal magnetic resonance imaging. In addition, 2 pregnancies with severe magnetic resonance imaging findings had complicated courses. CONCLUSION: Mildly abnormal magnetic resonance neuroimaging findings were common after laser photocoagulation for twin-twin transfusion syndrome complicated by single fetal demise and were more common in cases of recipient demise than donor demise. Severe magnetic resonance neuroimaging findings in this series were limited to patients with complicated peri- or postoperative courses.

Fetal thoracic teratomas: mediastinal or pericardial?

BACKGROUND: Mediastinal and pericardial teratomas have overlapping imaging features that may make accurate prenatal diagnosis challenging. OBJECTIVE: To identify prenatal imaging features that may aid in distinguishing between mediastinal and pericardial teratomas. MATERIALS AND METHODS: Prenatally diagnosed pericardial and mediastinal teratomas evaluated at our fetal center from 1995 to 2020 were included in this Institutional Review Board-approved study. Lesion volume was calculated using prospectively reported ultrasound (US) measurements and the formula of a prolate ellipsoid, which was then normalized to head circumference. Prenatal US and magnetic resonance imaging (MRI) studies were anonymized with two fetal imagers reviewing the US studies and two different fetal imagers reviewing the MRI studies. These experienced reviewers scored location of the mass in the craniocaudal axis and in the transverse axis. MRI reviewers also scored the presence of inferior cardiac compression by the lesion and whether there was identifiable thymic tissue. Reviewer disagreements were resolved by consensus review. RESULTS: Eleven pericardial teratomas and 10 mediastinal teratomas were identified. All cases underwent detailed fetal anatomic US and fetal echocardiogram and 10/11 (91%) pericardial teratomas and 8/10 (80%) mediastinal teratomas underwent fetal MRI. Median volume was higher for mediastinal teratomas compared to pericardial teratomas (42.5 mL [interquartile range (IQR) 15.9 - 67.2 mL] vs. 8.1 mL [IQR 7.7 - 27.7 mL], P=0.01) and median volume/head circumference was also statistically higher in mediastinal teratomas (1.33 [IQR 0.78 - 2.61] vs. 0.43 [IQR 0.38 - 1.10], P=0.01). Logistic regression analysis demonstrated a statistical difference between teratoma types with respect to location in the craniocaudal axis by both modalities with mediastinal teratomas more commonly located in the upper and upper-middle thorax compared to pericardial teratomas, which were more commonly found in the middle thorax (US, P=0.03; MRI, P=0.04). Logistic regression analysis also demonstrated a statistical difference between teratoma types with respect to position along the transverse axis by both modalities with mediastinal teratomas more commonly located midline or left paramedian and pericardial teratomas more often right paramedian in location (US, P<0.01; MRI, P=0.02). Inferior cardiac compression observed by MRI was associated more commonly with mediastinal teratomas compared to pericardial teratomas (87.5% [7/8] vs. 10% [1/10], P<0.01). Identifiable thymus by MRI was more commonly observed in cases of pericardial teratomas, however, this difference was not statistically significant (P=0.32). CONCLUSION: Mediastinal teratomas are associated with larger lesion size and inferior cardiac compression when compared to pericardial teratomas. These features combined with lesion location in the craniocaudal and transverse axes may allow for more accurate prenatal diagnosis and optimal perinatal and surgical management.

O-RADS US Risk Stratification and Management System: A Consensus Guideline from the ACR Ovarian-Adnexal Reporting and Data System Committee.

The Ovarian-Adnexal Reporting and Data System (O-RADS) US risk stratification and management system is designed to provide consistent interpretations, to decrease or eliminate ambiguity in US reports resulting in a higher probability of accuracy in assigning risk of malignancy to ovarian and other adnexal masses, and to provide a management recommendation for each risk category. It was developed by an international multidisciplinary committee sponsored by the American College of Radiology and applies the standardized reporting tool for US based on the 2018 published lexicon of the O-RADS US working group. For risk stratification, the O-RADS US system recommends six categories (O-RADS 0-5), incorporating the range of normal to high risk of malignancy. This unique system represents a collaboration between the pattern-based approach commonly used in North America and the widely used, European-based, algorithmic-style International Ovarian Tumor Analysis (IOTA) Assessment of Different Neoplasias in the Adnexa model system, a risk prediction model that has undergone successful prospective and external validation. The pattern approach relies on a subgroup of the most predictive descriptors in the lexicon based on a retrospective review of evidence prospectively obtained in the IOTA phase 1-3 prospective studies and other supporting studies that assist in differentiating management schemes in a variety of almost certainly benign lesions. With O-RADS US working group consensus, guidelines for management in the different risk categories are proposed. Both systems have been stratified to reach the same risk categories and management strategies regardless of which is initially used. At this time, O-RADS US is the only lexicon and classification system that encompasses all risk categories with their associated management schemes.

Two's Company: Multiple Thoracic Lesions on Prenatal US and MRI.

BACKGROUND: Congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), and CPAM-BPS hybrid lesions are most commonly solitary; however, >1 lung congenital lung lesion may occur. OBJECTIVES: To assess the frequency of multiple congenital thoracic anomalies at a high-volume referral center; determine prenatal ultrasound (US) and magnetic resonance imaging (MRI) features of these multifocal congenital lung lesions that may allow prenatal detection; and determine the most common distribution or site of origin. METHODS: Database searches were performed from August 2008 to May 2019 for prenatally evaluated cases that had a final postnatal surgical diagnosis of >1 congenital lung lesion or a lung lesion associated with foregut duplication cyst (FDC). Lesion location, size, echotexture, and signal characteristics were assessed on prenatal imaging and correlated with postnatal computed tomographic angiography and surgical pathology. -Results: Of 539 neonates that underwent surgery for a thoracic lesion, 35 (6.5%) had >1 thoracic abnormality. Multiple discrete lung lesions were present in 19 cases, and a lung lesion associated with an FDC was present in 16. Multifocal lung lesions were bilateral in 3 cases; unilateral, multilobar in 12; and, unilobar multisegmental in 4. Median total CPAM volume/head circumference ratio for multifocal lung lesions on US was 0.66 (range, 0.16-1.80). Prenatal recognition of multifocal lung lesions occurred in 7/19 cases (36.8%). Lesion combinations were CPAM-CPAM in 10 cases, CPAM-BPS in 5, CPAM-hybrid in 2, hybrid-hybrid in 1, and hybrid-BPS in 1. Of 5 unilateral, multifocal lung lesions, multifocality was prenatally established through identification of a band of normal intervening lung or intrinsic differences in lesion imaging features. CONCLUSIONS: Although less common, multiple thoracic abnormalities can be detected prenatally. Of multifocal lung lesions, the most common combination was CPAM-CPAM, with a unilateral, multilobar distribution. Prenatal recognition is important for pregnancy counseling and postnatal surgical management.

Simple Adnexal Cysts: SRU Consensus Conference Update on Follow-up and Reporting.

This multidisciplinary consensus update aligns prior Society of Radiologists in Ultrasound (SRU) guidelines on simple adnexal cysts with recent large studies showing exceptionally low risk of cancer associated with simple adnexal cysts. Most small simple cysts do not require follow-up. For larger simple cysts or less well-characterized cysts, follow-up or second opinion US help to ensure that solid elements are not missed and are also useful for assessing growth of benign tumors. In postmenopausal women, reporting of simple cysts greater than 1 cm should be done to document their presence in the medical record, but such findings are common and follow-up is recommended only for simple cysts greater than 3-5 cm, with the higher 5-cm threshold reserved for simple cysts with excellent imaging characterization and documentation. For simple cysts in premenopausal women, these thresholds are 3 cm for reporting and greater than 5-7 cm for follow-up imaging. If a cyst is at least 10%-15% smaller at any time, then further follow-up is unnecessary. Stable simple cysts at initial follow-up may benefit from a follow-up at 2 years due to measurement variability that could mask growth. Simple cysts that grow are likely cystadenomas. If a previously suspected simple cyst demonstrates papillary projections or solid areas at follow-up, then the cyst should be described by using standardized terminology. These updated SRU consensus recommendations apply to asymptomatic patients and to those whose symptoms are not clearly attributable to the cyst. These recommendations can reassure physicians and patients regarding the benign nature of simple adnexal cysts after a diagnostic-quality US examination that allows for confident diagnosis of a simple cyst. Patients will benefit from less costly follow-up, less anxiety related to these simple cysts, and less surgery for benign lesions.

Contrast-Enhanced Ultrasound in Extracorporeal Support: In Vitro Studies and Initial Experience and Safety Data in the Extreme Premature Fetal Lamb Maintained by the Extrauterine Environment for Neonatal Development.

OBJECTIVES: To evaluate the effects of ultrasound contrast agent (UCA) administration on hemodynamic parameters and support equipment in in vitro and in vivo models of extracorporeal support. METHODS: In vitro, incrementally increasing bolus doses of a UCA were administered proximal to a membrane oxygenator, and ultrasound cine clips were obtained. The rates of microbubble destruction across the oxygenator and over time were calculated from time-intensity-curves. Measurements across the membrane oxygenator were recorded and compared by a repeated-measures analysis of variance. In vivo, 7 premature fetal lambs were transferred from placental support to the extrauterine environment for neonatal development. Contrast agent boluses were administered for contrast-enhanced ultrasound (CEUS) examinations. Hemodynamic parameters and serum laboratory values were evaluated before and after the examinations by paired t tests. For oxygenator staining, oxygenator membranes from the in vitro circuit, study animals (n = 4), and control animals (n = 4) were stained for the adherent UCA. RESULTS: In vitro, with all doses (0.1-4 mL), there was no difference in measured parameters across the oxygenator (P ≥ .09). Contrast agent destruction (3%-14%) across the oxygenator was observed at the first pass with a progressive decline in contrast intensity over time. In vivo, there was no difference in hemodynamic parameters or serum laboratory values (P ≥ .08) with any CEUS examination (n = 17). For oxygenator staining, all oxygenator membranes were negative for UCA with lipid staining. CONCLUSIONS: The UCA had no detectable effect on the oxygenator or measured parameters in in vitro and in vivo studies, thus providing additional safety data to support the use of CEUS in the setting of extracorporeal support.

Secondary Imaging Findings Aid in Prenatal Diagnosis and Characterization of Congenital Diaphragmatic Hernia: Role of an Abnormal Orientation of Vascular Structures and Gallbladder Position.

OBJECTIVES: To determine whether an abnormal orientation of the abdominal or hepatic vasculature and an abnormal gallbladder position on prenatal ultrasound (US) imaging are associated with intrathoracic liver herniation and postnatal outcomes in cases of congenital diaphragmatic hernia (CDH). METHODS: Children who underwent prenatal US examinations and postnatal CDH repair at our institution were eligible. Prenatal US images were reviewed, and the orientation of the superior mesenteric artery (SMA) and hepatic veins as well as gallbladder position were recorded. Findings were correlated with prenatal US measurements (lung-to-head ratio and calculated observed-to-expected lung-to-head ratio) and postnatal outcomes, including intrathoracic liver herniation, an extracorporeal membrane oxygenation (ECMO) requirement, and mortality. RESULTS: A total of 175 patients met inclusion criteria. The SMA was shown in 168 cases and had a cephalad orientation in 95.4% (161 of 168), which was not associated with outcome measures and represented bowel herniation. A cephalad orientation of the hepatic veins was identified in 52.6% (90 of 171) and was associated with intrathoracic liver herniation, an ECMO requirement, and mortality (P < .01). In right-sided CDH, the gallbladder was intrathoracic in 91.3% (21 of 23). In left-sided CDH, an abnormal gallbladder position was seen in 51.3% (76/152) and was associated with intrathoracic liver herniation, an ECMO requirement, mortality, and lower lung-to-head ratio and observed-to-expected lung-to-head ratio values. When combined, abnormal hepatic vein and gallbladder positions showed good sensitivity and specificity in predicting intrathoracic liver herniation (area under the curve, 0.93). CONCLUSIONS: Abnormal SMA, hepatic vein, and gallbladder positions can be used to improve prenatal characterization of CDH. Accurate depiction of these structures on prenatal US images may aid in patient counseling and postnatal management.

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

OBJECTIVES: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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 diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes.

BACKGROUND: The presence of a hernia sac in congenital diaphragmatic hernia (CDH) has been reported to be associated with higher lung volumes and better postnatal outcomes. OBJECTIVE: To compare prenatal imaging (ultrasound and MRI) prognostic measurements and postnatal outcomes of CDH with and without hernia sac. MATERIALS AND METHODS: We performed database searches from January 2008 to March 2017 for surgically proven cases of CDH with and without hernia sac. All children had a detailed ultrasound (US) examination and most had an MRI examination. We reviewed the medical records of children enrolled in our Pulmonary Hypoplasia Program. RESULTS: Of 200 cases of unilateral CDH, 46 (23%) had hernia sacs. Cases of CDH with hernia sac had a higher mean lung-to-head ratio (LHR; 1.61 vs. 1.17; P<0.01), a higher mean observed/expected LHR (0.49 vs. 0.37; P<0.01), and on MRI a higher mean observed/expected total lung volume (0.53 vs. 0.41; P<0.01). Based on a smooth interface between lung and herniated contents, hernia sac or eventration was prospectively questioned by US and MRI in 45.7% and 38.6% of cases, respectively. Postnatally, hernia sac is associated with shorter median periods of admission to the neonatal intensive care unit (45.0 days vs. 61.5 days, P=0.03); mechanical ventilation (15.5 days vs. 23.5 days, P=0.04); extracorporeal membrane oxygenation (251 h vs. 434 h, P=0.04); decreased rates of patch repair (39.0% vs. 69.2%, P<0.01); and pulmonary hypertension (56.1% vs. 75.4%, P=0.03). CONCLUSION: Hernia sac is associated with statistically higher prenatal prognostic measurements and improved postnatal outcomes. Recognition of a sharp interface between lung and herniated contents may allow for improved prenatal diagnosis; however, delivery and management should still occur at experienced quaternary neonatal centers.

High Tumor Volume to Fetal Weight Ratio Is Associated with Worse Fetal Outcomes and Increased Maternal Risk in Fetuses with Sacrococcygeal Teratoma.

OBJECTIVE: Tumor volume to fetal weight ratio (TFR) > 0.12 before 24 weeks has been associated with poor outcome in fetuses with sacrococcygeal teratoma (SCT). We evaluated TFR in predicting poor fetal outcome and increased maternal operative risk in our cohort of SCT pregnancies. METHODS: This is a retrospective, single-center review of fetuses seen with SCT from 1997 to 2015. Patients who chose termination of pregnancy (TOP), delivered elsewhere, or had initial evaluation at > 24 weeks were excluded. Receiver operating characteristic (ROC) analysis determined the optimal TFR to predict poor fetal outcome and increased maternal operative risk. Poor fetal outcome included fetal demise, neonatal demise, or fetal deterioration warranting open fetal surgery or delivery < 32 weeks. Increased maternal operative risk included cases necessitating open fetal surgery, classical cesarean delivery, or ex utero intrapartum treatment (EXIT). RESULTS: Of 139 pregnancies with SCT, 27 chose TOP, 14 delivered elsewhere, and 40 had initial evaluation at > 24 weeks. Thus, 58 fetuses were reviewed. ROC analysis revealed that at ≤24 weeks, TFR > 0.095 was predictive of poor fetal outcome and TFR > 0.12 was predictive of increased maternal operative risk. CONCLUSION: This study supports the use of TFR at ≤24 weeks for risk stratification of pregnancies with SCT.

Prenatal sonography of multicentric infantile myofibromatosis: Case report and review of the literature.

Scant literature exists on prenatally diagnosed infantile myofibromatosis (IM). We report a case of multicentric IM, which was first recognized as a soft-tissue paraspinal mass on prenatal sonography and subsequently characterized by MRI with pathological confirmation.

Going With the Flow: An Aid in Detecting and Differentiating Bronchopulmonary Sequestrations and Hybrid Lesions.

OBJECTIVES: To assess the ability of prenatal ultrasound (US) in identifying systemic feeding arteries in bronchopulmonary sequestrations and hybrid lesions and report the ability of US in classifying bronchopulmonary sequestrations as intralobar or extralobar. METHODS: Institutional Review Board-approved radiology and clinical database searches from 2008 to 2015 were performed for prenatal lung lesions with final diagnoses of bronchopulmonary sequestrations or hybrid lesions. All patients had detailed US examinations, and most patients had ultrafast magnetic resonance imaging (MRI). Lesion location, size, and identification of systemic feeding arteries and draining veins were assessed with US. RESULTS: The study consisted of 102 bronchopulmonary sequestrations and 86 hybrid lesions. The median maternal age was 30 years. The median gestational age was 22 weeks 5 days. Of bronchopulmonary sequestrations, 66 had surgical pathologic confirmation, and 100 had postnatal imaging. Bronchopulmonary sequestration locations were intrathoracic (n = 77), intra-abdominal (n = 19), and transdiaphragmatic (n = 6). Of hybrid lesions, 84 had surgical pathologic confirmation, and 83 had postnatal imaging. Hybrid lesion locations were intrathoracic (n = 84) and transdiaphragmatic (n = 2). Ultrasound correctly identified systemic feeding arteries in 86 of 102 bronchopulmonary sequestrations and 79 of 86 hybrid lesions. Of patients who underwent MRI, systemic feeding arteries were reported in 62 of 92 bronchopulmonary sequestrations and 56 of 81 hybrid lesions. Ultrasound identified more systemic feeding arteries than MRI in both bronchopulmonary sequestrations and hybrid lesions (P < .01). Magnetic resonance imaging identified systemic feeding arteries that US did not in only 2 cases. In cases in which both systemic feeding arteries and draining veins were identified, US could correctly predict intrathoracic lesions as intralobar or extralobar in 44 of 49 bronchopulmonary sequestrations and 68 of 73 hybrid lesions. CONCLUSIONS: Ultrasound is most accurate for systemic feeding artery detection in bronchopulmonary sequestrations and hybrid lesions and can also type the lesions as intralobar or extralobar when draining veins are evaluated.

Right Congenital Diaphragmatic Hernias: Is There a Correlation between Prenatal Lung Volume and Postnatal Survival, as in Isolated Left Diaphragmatic Hernias?

OBJECTIVES: Whereas left-sided congenital diaphragmatic hernias (L-CDH) have been extensively studied and their prognostic parameters delineated, right-sided hernias (R-CDH) have not. Published results remain inconclusive. The aim of this study is to evaluate if proven prognostic indicators of postnatal survival in the fetus with L-CDH apply to the fetus with R-CDH. METHODS: Retrospective single-center study of R-CDH fetuses with available prenatal studies assessed for fetal lung volume by means of ultrasound-measured observed versus expected (O/E) lung area to head circumference (LHR) and magnetic resonance-calculated O/E total lung volume (TLV) in a 12-year time period. Percentage of herniated liver volume and postnatal use of extracorporeal membrane oxygenation (ECMO) were also evaluated. RESULTS: In a cohort of 24 patients, O/E LHR, O/E TLV, percentage of herniated liver, and postnatal use of ECMO are not prognostic indicators of survival in the fetus with R-CDH. Cut-off values of O/E LHR of ≤45 or O/E TLV ≤25, known to select a population of severe cases for the L-CDH fetus, do not appear to extrapolate to the R-CDH fetus, as survival in both R-CDH groups is 60%. CONCLUSION: The findings in this study suggest that L- and R-CDH appear to behave differently, and that factors that make L-CDH fatal (low O/E TLV and O/E LHR, high-volume herniated liver) may not apply to the fetus with R-CDH.

First International Consensus Report on Adnexal Masses: Management Recommendations.

The First International Consensus Conference on Adnexal Masses was convened to thoroughly examine the state of the science and to formulate recommendations for clinical assessment and management. The panel included representatives of societies in the fields of gynecology, gynecologic oncology, radiology, and pathology and clinicians from Europe, Canada, and the United States. In the United States, there are approximately 9.1 surgeries per malignancy compared to the European International Ovarian Tumor Analysis center trials, with only 2.3 (oncology centers) and 5.9 (other centers) reported surgeries per malignancy, suggesting that there is room to improve our preoperative assessments. The American College of Obstetricians and Gynecologists Practice Bulletin on "Management of Adnexal Masses," reaffirmed in 2015 (Obstet Gynecol 2007; 110:201-214), still states, "With the exception of simple cysts on a transvaginal ultrasound finding, most pelvic masses in postmenopausal women will require surgical intervention." The panel concluded that patients would benefit not only from a more conservative approach to many benign adnexal masses but also from optimization of physician referral patterns to a gynecologic oncologist in cases of suspected ovarian malignancies. A number of next-step options were offered to aid in management of cases with sonographically indeterminate adnexal masses. This process would provide an opportunity to improve risk stratification for indeterminate masses via the provision of alternatives, including but not limited to evidence-based risk-assessment algorithms and referral to an "expert sonologist" or to a gynecologic oncologist. The panel believed that these efforts to improve clinical management and preoperative triage patterns would ultimately improve patient care.

Fetal Lymphatic Malformations: More Variable Than We Think?

Lymphatic malformations are benign lesions that result from abnormal development of the lymphatic and venous systems. These lesions may be detected during routine prenatal ultrasound screening, and typically demonstrate imaging findings of a multiseptate cystic lesion lacking solid components, vascularity, and calcifications. We report 73 cases of prenatally detected lymphatic malformations and describe greater variability in their prenatal sonographic appearance than previously reported, including purely cystic lesions and mixed cystic and solid lesions with calcifications. Appreciation of this increased variability is important in providing accurate prenatal diagnosis, counseling, and management.

The diagnostic features of spina bifida: the role of ultrasound.

Myelomeningocele (MMC) is one of the most devastating, nonlethal congenital anomalies worldwide. The live birth prevalence of MMC changed dramatically in the 1980s with the introduction of maternal serum screening and the widespread use of prenatal ultrasound imaging. The high-resolution ultrasound affordable today with state-of-the-art equipment allows us to make a very accurate diagnosis of MMC, including details related to the entire fetal central nervous system. Ultrasound can accurately localize the site of the osseous and soft tissue defects. Congenital spinal defects can be characterized definitively as open or closed, which are treated very differently with in utero repair, which is done in some cases, compared to only conservative follow-up with postnatal therapy for occult defects. Additional findings of kyphosis, scoliosis and anomalous vertebrate and associated conditions such as cervical syrinx can be identified. The state of the intracranial structures, including the presence or absence of ventriculomegaly and hindbrain herniation, as well as unexpected complications such as intracranial hemorrhage can be diagnosed. The severity of neurological compromise in some fetuses can be estimated by detailed examination of the lower extremities. As well as searching for talipes, we also now routinely characterize flexion and extension motions at the hip, knee and ankle joints. The information provided by ultrasound plays a crucial role, now more than ever, in patient counseling and pregnancy management. This article emphasizes how we utilize ultrasound in the evaluation of patients with suspected MMC at the Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia.

Prenatal MRI evaluation of limb-body wall complex.

BACKGROUND: The sonographic (US) features of limb-body wall complex have been well documented; however the literature regarding the findings on MRI in limb-body wall complex is scant. OBJECTIVE: To characterize the prenatal MRI features of limb-body wall complex. MATERIALS AND METHODS: We performed a retrospective review of all MRI scans of fetuses diagnosed with limb-body wall complex at our institution from 2001 to 2011. Fetuses without correlating US scans or follow-up information were excluded. Three pediatric radiologists blinded to the specific US findings reviewed the prenatal MRIs. Images were evaluated for the organ location and attachment, the body part affected, characterization of the body wall defect, and spinal, limb and umbilical cord abnormalities. RESULTS: Ten subjects met inclusion criteria. MRI was able to detect and characterize the body part affected and associated abnormalities. All fetuses had ventral wall defects, a small thorax and herniated liver and bowel. The kidneys were extracorporeal in three cases. The extruded organs were attached to the placenta or the uterine wall in all cases. Abnormal spinal curvatures of various degrees of severity were present in all cases. Eight cases had a short, uncoiled cord. Limb anomalies were present in 6 of the 10 cases. CONCLUSION: We illustrate the common fetal MRI findings of limb-body wall complex. The prenatal diagnosis of limb-body wall complex and the differentiation of this defect from treatable abdominal wall defects are crucial to providing appropriate guidance for patient counseling and management.

Outcomes of prenatally diagnosed lung lesions in multigestational pregnancies.

BACKGROUND: The outcomes of prenatally diagnosed lung lesions in the context of multigestational pregnancies are unknown. METHODS: Of 960 fetal lung lesion cases evaluated at a single tertiary center over 16 years, 30 occurred in multigestational pregnancies. We reviewed this series to aid in prenatal counseling of affected families and to provide prognostic information for decision making. Pre- and postnatal clinical characteristics were gathered for these pregnancies, and the morbidity and mortality were determined for both affected and normal fetuses, whether twins or triplets. RESULTS: Mortality was found to be 3/30 (10%) for affected fetuses, and morbidity in normal co-twins was consistent with the degree of prematurity. No morbidity was seen in co-twins born at or after 36 weeks of gestation. Median gestational age at delivery was 35 5/7 weeks. CONCLUSIONS: Outcomes for the affected fetus correlate with the size and pathophysiologic consequences of the lesion and are not worse than previously reported outcomes for similar lesions in singleton pregnancies, while morbidity in the normal co-twin is consistent with prematurity related to the fetal age of the multiple gestation at delivery, irrespective of the fetal lung lesion.

Low-dose fetal CT in the prenatal evaluation of skeletal dysplasias and other severe skeletal abnormalities.

OBJECTIVE: Prenatal diagnosis of skeletal dysplasia is often difficult and based on findings with ultrasound, a technique with 40-60% sensitivity. The purpose of this study was to evaluate a preliminary experience in assessing severe prenatal osseous abnormalities with low-dose fetal CT. The hypothesis was that use of CT may improve the prenatal diagnosis of skeletal dysplasia beyond the available capabilities of ultrasound. MATERIALS AND METHODS: Retrospective search of a radiology database (July 2008-February 2011) yielded the records of unenhanced CT examinations of patients referred because of abnormal fetal bones. The original ultrasound and CT reports as interpreted at image acquisition were independently analyzed by two radiologists blinded to the final diagnosis and to the findings of the opposing imaging modality. Blinded review of the images was also performed. Correlation was made with the postmortem and postnatal findings. RESULTS: According to the reports of the studies, 5 of 21 cases were interpreted correctly with CT and incorrectly with ultrasound. In 17 cases, CT revealed additional osseous findings not in the ultrasound report. There were no cases in which ultrasound findings were correct and CT findings were incorrect. Blinded review of the images revealed that CT outperformed ultrasound (p < 0.001). There were a total of four CT errors among 218 total measures recorded and a total of 19 ultrasound errors among 218 total measures. CONCLUSION: Although low-dose fetal CT should never be used as the initial diagnostic modality in cases of suspected skeletal dysplasia, it is a powerful imaging adjunct that depicts the fetal bones in exquisite detail. Use of CT of fetuses at risk of skeletal dysplasia may provide clinicians with more accurate information for counseling of families regarding neonatal morbidity and mortality.