Comparison of current to past outcomes in congenital diaphragmatic hernia using MRI observed-to-expected total fetal lung volume.

BACKGROUND: Fetal Centers use imaging studies to predict congenital diaphragmatic hernia (CDH) prognosis and the need for fetal therapy. Given improving CDH survival, we hypothesized that current fetal imaging severity predictions no longer reflect true outcomes and fail to justify the risks of fetal therapy. METHODS: We analyzed our single-center contemporary data in a left-sided CDH cohort (n = 58) by prognostic criteria determined by MRI observed-to-expected total fetal lung volumes: severe <25%, moderate 25-35%, and mild >35%. We compared contemporary survival to prior studies and the TOTAL trials. RESULTS: Contemporary survival was significantly higher than past studies for all prognostic classifications (mild 100% vs 80-94%, moderate 95% vs 59-75%, severe 79% vs 13-25%; P < 0.01), and to either control or fetal therapy arms of the TOTAL trials. CONCLUSIONS: Current fetal imaging criteria are overly pessimistic and may lead to unwarranted fetal intervention. Fetal therapies remain experimental. Future studies will require updated prognostic criteria.

Troubleshooting Tips for Diagnosing Complex Fetal Genitourinary Malformations.

Fetal genitourinary anomalies can present a diagnostic challenge for the radiologist. The absence of a normally located kidney may represent agenesis or be secondary to a fusion or migration abnormality. A dilated renal pelvis should prompt evaluation for a specific cause, including ureteropelvic junction obstruction, reflux, or an obstructed duplicated system. Cystic parenchymal changes are characteristic of a multicystic dysplastic kidney but may also be seen in obstructive cystic dysplasia. There are numerous causes of megacystis including chromosomal (trisomy 18 syndrome), obstruction (posterior urethral valves, urethral atresia), or muscular dysfunction (prune belly syndrome, megacystis microcolon intestinal hypoperistalsis syndrome). Important mimics of a large bladder include hydrocolpos and urogenital sinus or cloacal malformation. Complications of genitourinary malformations are common and include oligohydramnios, urinary ascites, and urinoma. Making an accurate diagnosis often requires additional US views beyond those obtained in the standard fetal survey and occasionally performing fetal MRI. The appropriate use of orthogonal T2-weighted sequences, in conjunction with diffusion-weighted images for evaluation of the kidneys and gradient-recalled-echo sequences for evaluation of T1-hyperintense meconium in the colon, can play an integral role in diagnosis. Accurate diagnosis of fetal genitourinary malformations is vital to direct patient counseling and pregnancy management as outcomes are highly variable. Some conditions can be surgically corrected quite simply, some require multiple complex procedures, and some are lethal. The authors offer troubleshooting tips to narrow the differential diagnosis for four observations: unilateral absent kidney, dilated renal pelvis, cystic renal parenchyma, and megacystis and its mimics. ©RSNA, 2023 Test Your Knowledge questions are available in the Online Learning Center.

Assessment of Amniotic Fluid Volume in Pregnancy.

Amniotic fluid (AF) is an integral part of the fetal environment and is essential for fetal growth and development. Pathways of AF recirculation include the fetal lungs, swallowing, absorption through the fetal gastrointestinal tract, excretion through fetal urine production, and movement. In addition to being a marker for fetal health, adequate AF is necessary for fetal lung development, growth, and movement. The role of diagnostic imaging is to provide a detailed fetal survey, placental evaluation, and clinical correlation with maternal conditions to help identify causes of AF abnormalities and thereby enable specific therapy. Oligohydramnios prompts evaluation for fetal growth restriction as well as genitourinary issues, including renal agenesis, multicystic dysplastic kidneys, ureteropelvic junction obstruction, and bladder outlet obstruction. Premature preterm rupture of membranes should also be clinically excluded as a cause of oligohydramnios. Clinical trials evaluating amnioinfusion are underway as a potential intervention for renal causes of oligohydramnios. Most cases of polyhydramnios are idiopathic, with maternal diabetes being a common cause. Polyhydramnios prompts evaluation for fetal gastrointestinal obstruction and oropharyngeal or thoracic masses, as well as neurologic or musculoskeletal anomalies. Amnioreduction is performed only for maternal indications such as symptomatic polyhydramnios causing maternal respiratory distress. Polyhydramnios with fetal growth restriction is paradoxical and can occur with maternal diabetes and hypertension. When these maternal conditions are absent, this raises concern for aneuploidy. The authors describe the pathways of AF production and circulation, US and MRI assessment of AF, disease-specific disruption of AF pathways, and an algorithmic approach to AF abnormalities. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Postpartum hemorrhage - what the interventional radiologist should know.

Postpartum hemorrhage is a leading cause of maternal morbidity and mortality around the world and can be caused by multiple etiologies. Distinguishing between the various etiologies that lead to PPH and identifying high risk features are crucial to implementing effective clinical management. In this review, the diagnostic imaging features and management principles of some of the most important causes of postpartum hemorrhage are discussed, with an emphasis on the pearls and pitfalls when minimally invasive treatment via interventional radiologic techniques are employed.

Accuracy and Interobserver Reliability of Magnetic Resonance Imaging for Placenta Accreta Spectrum Disorders.

OBJECTIVE: This study aims to define the accuracy, predictive value, and interobserver reliability of magnetic resonance imaging (MRI) in the diagnosis of placenta accreta spectrum (PAS) disorders. STUDY DESIGN: Two experienced radiologists independently interpreted the MRI studies of patients with possible PAS from two referral centers. Radiologists were blinded to sonographic and clinical information. We calculated diagnostic testing characteristics and kappa statistics of interobserver reliability for MRI findings of PAS. RESULTS: Sixty-eight MRI cases were evaluated. Confirmed PAS and severe PAS were present in 44 (65%) and 20 (29%) cases. For the diagnosis of any PAS, MRI had a sensitivity 66%, specificity 71%, positive predictive value (PPV) 81%, negative predictive value (NPV) 53%, and accuracy 68%. For the diagnosis of severe PAS (percreta), MRI had a sensitivity 85%, specificity 79%, PPV 63%, NPV 93%, and accuracy 81%. The accuracy of individual signs of PAS was lower (44-65%). Interobserver agreement was almost perfect for previa; substantial for myometrial interruptions, PAS, severe PAS, and placental bulging/balling; and moderate to slight for other signs of PAS. CONCLUSION: Although the interobserver reliability of MRI for a diagnosis of PAS is substantial, the accuracy and predictive value are modest and lower than previously reported.

Sonographic Assessment of Fetal Growth Abnormalities.

Fetal growth abnormalities have significant consequences for pregnancy management and maternal and fetal well-being. The accurate diagnosis of fetal growth abnormalities contributes to optimal antenatal management, which may minimize the sequelae of inadequate or excessive fetal growth. An accurate diagnosis of abnormal fetal growth depends on accurate pregnancy dating and serial growth measurements. The fetal size at any given stage of pregnancy is either appropriate or inappropriate for the given gestational age (GA). Pregnancy dating is most accurate in the first trimester, as biologic variability does not come into play until the second and third trimesters. The authors describe the determination of GA with use of standard US measurements and how additional parameters can be used to confirm dating. Once dates are established, serial measurements are used to identity abnormal growth patterns. The sometimes confusing definitions of abnormal growth are clarified, the differentiation of a constitutionally small but healthy fetus from a growth-restricted at-risk fetus is described, and the roles of Doppler US and other adjunctive examinations in the management of growth restriction are discussed. In addition, the definition of selective growth restriction in twin pregnancy is briefly discussed, as is the role of Doppler US in the classification of subtypes of selective growth restriction in monochorionic twinning. The criteria for diagnosing macrosomia and the management of affected pregnancies also are reviewed. The importance of correct pregnancy dating in the detection and surveillance of abnormal fetal growth and for prevention of perinatal maternal and fetal morbidity and mortality cannot be overstated. The online slide presentation from the RSNA Annual Meeting is available for this article. ©RSNA, 2020.

A Radiologist's Guide to the Performance and Interpretation of Obstetric Doppler US.

Doppler US provides a unique window to the fetoplacental circulation, allowing assessment of fetal well-being. Doppler US of the umbilical artery is an integral component of managing the fetus with growth restriction; and Doppler US of the middle cerebral artery, as a noninvasive means of detecting fetal anemia, has revolutionized the management of pregnancies complicated by alloimmunization. Serial use of amniocentesis, with its attendant risks, has been replaced by serial Doppler US examinations. Invasive procedures are now reserved for the treatment of anemia with intrauterine transfusion. Technique is critical to obtain the best waveforms for ease of shape assessment, velocity measurement, and calculation of various ratios. In this article, the safety of Doppler US is reviewed, the fetal circulation is described, and the role of Doppler US is demonstrated in first-trimester screening and in the evaluation of growth restriction, anemia, and other causes of fetal compromise in the second and third trimesters. Sampling technique is explained, and normal and abnormal waveforms are illustrated for the ductus venosus, umbilical artery, umbilical vein, middle cerebral artery, and uterine artery. Some examples of clinical cases are provided to illustrate how the results are used in clinical practice. Clinical examples of velamentous insertion and vasa previa are also provided to aid the practicing radiologist with recognition of these entities. In particular, vasa previa is considered a critical finding; it alters pregnancy management, requiring hospital admission, administration of steroid therapy, and planned early cesarean delivery. ©RSNA, 2019.

Magnetic resonance imaging is often misleading when used as an adjunct to ultrasound in the management of placenta accreta spectrum disorders.

BACKGROUND: Magnetic resonance imaging is reported to have good sensitivity and specificity in the diagnosis of placenta accreta spectrum disorders, and is often used as an adjunct to ultrasound. But the additional utility of obtaining magnetic resonance imaging to assist in the clinical management of patients with placenta accreta spectrum disorders, above and beyond the information provided by ultrasound, is unknown. OBJECTIVE: We aimed to determine whether magnetic resonance imaging provides data that may inform clinical management by changing the sonographic diagnosis of placenta accreta spectrum disorders. STUDY DESIGN: In all, 78 patients with sonographic evidence or clinical suspicion of placenta accreta spectrum underwent magnetic resonance imaging of the abdomen and pelvis in orthogonal planes through the uterus utilizing T1- and T2-weighted imaging sequences at the University of Utah and the University of Colorado from 1997 through 2017. The magnetic resonance imaging was interpreted by radiologists with expertise in diagnosis of placenta accreta spectrum who had knowledge of the sonographic interpretation and clinical risk factors for placenta accreta spectrum disorders. The primary outcome was a change in diagnosis from sonographic interpretation that could alter clinical management, which was defined a priori. Diagnostic accuracy was verified by surgical and histopathologic diagnosis at the time of delivery. RESULTS: A change in diagnosis that could potentially alter clinical management occurred in 28 (36%) cases. Magnetic resonance imaging correctly changed the diagnosis in 15 (19%), and correctly confirmed the diagnosis in 34 (44%), but resulted in an incorrect change in diagnosis in 13 (17%), and an incorrect confirmation of ultrasound diagnosis in 15 (21%). Magnetic resonance imaging was not more likely to change a diagnosis in the 24 cases of posterior and lateral placental location compared to anterior location (33% vs 37%, P = .84). Magnetic resonance imaging resulted in overdiagnosis in 23% and in underdiagnosis in 14% of all cases. When ultrasound suspected severe disease (percreta) in 14 cases, magnetic resonance imaging changed the diagnosis in only 2 cases. Lastly, the proportion of accurate diagnosis with magnetic resonance imaging did not improve over time (61-65%, P = .96 for trend) despite increasing volume and increasing numbers of changed diagnoses. CONCLUSION: Magnetic resonance imaging resulted in a change in diagnosis that could alter clinical management of placenta accreta spectrum disorders in more than one third of cases, but when changed, the diagnosis was often incorrect. Given its high cost and limited clinical value, magnetic resonance imaging should not be used routinely as an adjunct to ultrasound in the diagnosis of placenta accreta spectrum until evidence for utility is clearly demonstrated by more definitive prospective studies.

Intrauterine Linear Echogenicities in the Gravid Uterus: What Radiologists Should Know.

Intrauterine linear echogenicity (ILE) is a common ultrasonographic finding in the gravid uterus and has variable causes and variable maternal and fetal outcomes. Correctly categorizing ILE during pregnancy is crucial for guiding surveillance and advanced imaging strategies. Common causes of ILE include membranes in multiple gestations, uterine synechiae with amniotic sheets, and uterine duplication anomalies. Less common causes include circumvallate placenta, chorioamniotic separation, and hemorrhage between membranes. Amniotic band syndrome is a rare but important diagnosis to consider, as it causes severe fetal defects. Imaging findings enable body stalk anomaly, a lethal defect, to be distinguished from amniotic bands, which although destructive are not necessarily lethal. This review describes the key imaging findings used to differentiate the various types of ILE in pregnancy, thus enabling accurate diagnosis and appropriate patient counseling. Online supplemental material is available for this article. ©RSNA, 2018.

Nongynecologic Findings Seen at Pelvic US.

Ultrasonography (US) is frequently the first imaging modality used to examine women with symptoms involving the pelvis. It is widely available and involves no exposure to ionizing radiation. Images can be acquired with a transabdominal, endovaginal, or translabial approach, and the use of video clips and three-dimensional reconstructions can be helpful. US is excellent for assessment of the uterus, ovaries, and adnexa. Occasionally, nongynecologic pelvic diseases arising from the gastrointestinal, genitourinary, and musculoskeletal systems and the pelvic peritoneal and extraperitoneal spaces may be detected and can be a source of diagnostic dilemma. US can be helpful not only in the detection but also occasionally in the characterization of such entities. Computed tomography and magnetic resonance imaging are useful in complicated cases. In this article, the normal US appearance of the bowel and US signs of bowel disease and specific entities, including appendicitis, diverticular disease, bowel obstruction, appendiceal mucocele, and intestinal tumors, are reviewed. The lower urinary tract is included in the field of view in every pelvic US examination; commonly encountered entities related to the urinary bladder, distal ureter, and urethra are illustrated. In addition to arising in the gastrointestinal and genitourinary tracts, pathologic conditions in the pelvis can arise in the peritoneal or extraperitoneal space. Although conditions of the pelvic peritoneal and extraperitoneal spaces are rare, it is important to recognize these entities and distinguish them from the more common gynecologic diseases. Owing to the implications for diagnosis and management, radiologists and other physicians who perform pelvic US should be aware of the spectrum of nongynecologic pathologic entities that can be detected. ©RSNA, 2017.

Gestational Trophoblastic Disease: Clinical and Imaging Features.

Gestational trophoblastic disease (GTD) is a spectrum of both benign and malignant gestational tumors, including hydatidiform mole (complete and partial), invasive mole, choriocarcinoma, placental site trophoblastic tumor, and epithelioid trophoblastic tumor. The latter four entities are referred to as gestational trophoblastic neoplasia (GTN). These conditions are aggressive with a propensity to widely metastasize. GTN can result in significant morbidity and mortality if left untreated. Early diagnosis of GTD is essential for prompt and successful management while preserving fertility. Initial diagnosis of GTD is based on a multifactorial approach consisting of clinical features, serial quantitative human chorionic gonadotropin (ß-hCG) titers, and imaging findings. Ultrasonography (US) is the modality of choice for initial diagnosis of complete hydatidiform mole and can provide an invaluable means of local surveillance after treatment. The performance of US in diagnosing all molar pregnancies is surprisingly poor, predominantly due to the difficulty in differentiating partial hydatidiform mole from nonmolar abortion and retained products of conception. While GTN after a molar pregnancy is usually diagnosed with serial ß-hCG titers, imaging plays an important role in evaluation of local extent of disease and systemic surveillance. Imaging also plays a crucial role in detection and management of complications, such as uterine and pulmonary arteriovenous fistulas. Familiarity with the pathogenesis, classification, imaging features, and treatment of these tumors can aid in radiologic diagnosis and guide appropriate management. ©RSNA, 2017.

Fetal ovarian cysts: review of imaging spectrum, differential diagnosis, management, and outcome.

Fetal ovarian cysts are the most common abdominal cysts observed in the female fetus but may be mistaken for genitourinary cysts, gastrointestinal cysts, lymphangiomas, or fetus in fetu. Ultrasonography (US) is the imaging modality of choice for fetal assessment, and magnetic resonance imaging is a useful problem-solving tool when uncertainty remains after careful US evaluation. At US, a fetal ovarian cyst manifests as an anechoic thin-walled cyst superior and parasagittal to the bladder. A daughter cyst may occasionally be observed and is pathognomonic for a cyst of ovarian origin. Fetal ovarian cysts may be simple or complicated and unilateral or bilateral, and they may masquerade as a solid mass when hemorrhage or torsion occurs. Complicated cysts may exhibit multiple septations, fluid-fluid levels, or mobile internal echoes. It is important to differentiate a hemorrhagic ovarian cyst from solid abdominal neoplasms that may be seen in a fetus. Recognition of the pertinent imaging findings will help radiologists distinguish fetal ovarian cysts from other fetal intra-abdominal masses in the differential diagnosis. Malignant ovarian neoplasms are rare in the fetus and neonate and thus are not considered in the differential diagnosis. The current literature on the management and outcome of fetal ovarian cysts is reviewed, with imaging studies presented from the authors' practice. Most fetal ovarian cysts resolve spontaneously; if operative intervention is required, the goal should be ovarian preservation.

The role of multimodality imaging after cesarean delivery.

There has been a global increase in the rate of cesarean delivery during the last 30 years. As a result, many women of reproductive age now have uterine scars. Well-recognized obstetric and gynecologic consequences include cesarean scar pregnancy, scar dehiscence or rupture, and scar endometriosis. More recently, abnormal menstrual bleeding, pelvic pain, and secondary infertility have been reported as a consequence of cesarean delivery. This article reviews the range of normal imaging findings after cesarean delivery, as well as the imaging manifestations of complications, both acute and chronic.

Holoprosencephaly: a survey of the entity, with embryology and fetal imaging.

Structural malformations of the brain are an important cause of childhood mortality and morbidity, with the latter having long-term financial and psychosocial implications for the affected child and family. Holoprosencephaly (HPE) is a severe brain malformation characterized by abnormal cleavage of the prosencephalon in the 5th gestational week. Aprosencephaly and atelencephaly occur earlier because of failure in the formation of the prosencephalon and telencephalon, respectively. The HPE holoprosencephaly spectrum classically includes alobar, semilobar, and lobar forms, although there are no clear-cut defining features. The middle interhemispheric variant (MIH), also known as syntelencephaly, is classified as a variant of HPE holoprosencephaly with midline interhemispheric fusion. Other conditions sometimes included in the spectrum of HPE holoprosencephaly include septo-optic dysplasia (SOD); "minimal" HPE holoprosencephaly , which is associated with subtle craniofacial malformations and mild developmental delay; and microform HPE holoprosencephaly , which by definition excludes brain involvement. The focus of this article will be on the spectrum of findings visible in fetal manifestation of the HPE holoprosencephaly spectrum. Brain embryology; the imaging characteristics, epidemiology, and embryology of HPE; and the more common associated anomalies, particularly those of the face ("the face predicts the brain") are reviewed. Recognition of these anomalies is important for accurate parental counseling, since the prognosis is poor but not invariably lethal; children with the milder forms may live well into their teens with severe developmental delays, endocrine dysfunction, and disrupted homeostasis. Available data on outcome in surviving children are summarized. Illustrative fetal ultrasonographic and magnetic resonance images are presented with clinical, autopsy, and postnatal imaging correlation.

Interobserver variability of sonography for prediction of placenta accreta.

OBJECTIVES: The sensitivity of sonography to predict accreta has been reported as higher than 90%. However, most studies are from single expert investigators. Our objective was to analyze interobserver variability of sonography for prediction of placenta accreta. METHODS: Patients with previa with and without accreta were ascertained, and images with placental views were collected, deidentified, and placed in random sequence. Three radiologists and 3 maternal-fetal medicine specialists interpreted each study for the presence of accreta and specific findings reported to be associated with its diagnosis. Investigator-specific sensitivity, specificity, and accuracy were calculated. κ statistics were used to assess variability between individuals and types of investigators. RESULTS: A total of 229 sonographic studies from 55 patients with accreta and 56 control patients were examined. Accuracy ranged from 55.9% to 76.4%. Of imaging studies yielding diagnoses, sensitivity ranged from 53.4% to 74.4%, and specificity ranged from 70.8% to 94.8%. Overall interobserver agreement was moderate (mean κ ± SD = 0.47 ± 0.12). κ values between pairs of investigators ranged from 0.32 (fair agreement) to 0.73 (substantial agreement). Average individual agreement ranged from fair (κ = 0.35) to moderate (κ = 0.53). CONCLUSIONS: Blinded from clinical data, sonography has significant interobserver variability for the diagnosis of placenta accreta.

Determination of fetal lung maturity using magnetic resonance imaging signal intensity measurements.

PURPOSE: The objective of this study was to determine if magnetic resonance signal intensity measurements can be used to predict gestational age and hence fetal lung maturity. METHODS: This institutional review board-approved study was a retrospective review of 394 fetal magnetic resonance imaging cases from a single institution for the years 2001 to 2011. For each case, T1- and T2-weighted sequences were selected for data collection. A single reviewer obtained 10 regions of interest (when possible) from each scan (fetal lung, fetal liver, fetal muscle, fetal spleen, and maternal urine, for both T1- and T2-weighted sequences). The medical record was searched for relevant information including best estimate of gestational age, Apgar scores, karyotype, and fetal diagnosis. A variety of organ-to-organ ratios and direct organ signal intensity measurements were assessed for correlation with gestational age. RESULTS: Three hundred thirty-five cases met inclusion criteria with gestational ages ranging from 17 to 39 weeks (mean, 28.6 weeks). A significant relationship between magnetic resonance signal intensity ratios and gestational age was demonstrated on the T2 lung-to-liver, T2 lung-to-spleen, T2 lung-to-muscle, T1 lung-to-liver, and T1 lung-to-spleen ratios (P < 0.05). T2 lung-to-liver and T2 lung-to-muscle demonstrated the strongest relationship with gestational age (best correlation r = 0.483, P < 0.001). T1 lung-to-liver and T1 lung-to-spleen demonstrated inverse relationships with gestational age (r = -0.174 [P = 0.03] and r = -0.236 [P = 0.02], respectively). CONCLUSIONS: A significant correlation between multiple signal intensity ratios and gestational age is demonstrated. However, the large variances preclude a clinically useful relationship.

Sonographic appearance of the placenta.

The placenta forms the interface between the mother and the fetus and is essential to fetal growth and development. In addition to the exchange of gases and nutrients, it serves immunologic and endocrine functions that protect the fetus and support physiological changes in pregnancy. Although often largely ignored during routine obstetric ultrasound and discarded after delivery, careful sonographic evaluation of the placenta can identify abnormalities that have profound implications for pregnancy outcomes and management. After describing the normal sonographic appearance of the placenta and normal anatomical variations, we review pathologic placental conditions, emphasize ultrasound findings, and highlight implications for pregnancy management and outcomes.

Accuracy of ultrasound for the prediction of placenta accreta.

OBJECTIVE: Ultrasound has been reported to be greater than 90% sensitive for the diagnosis of accreta. Prior studies may be subject to bias because of single expert observers, suspicion for accreta, and knowledge of risk factors. We aimed to assess the accuracy of ultrasound for the prediction of accreta. STUDY DESIGN: Patients with accreta at a single academic center were matched to patients with placenta previa, but no accreta, by year of delivery. Ultrasound studies with views of the placenta were collected, deidentified, blinded to clinical history, and placed in random sequence. Six investigators prospectively interpreted each study for the presence of accreta and findings reported to be associated with its diagnosis. Sensitivity, specificity, positive predictive, negative predictive value, and accuracy were calculated. Characteristics of accurate findings were compared using univariate and multivariate analyses. RESULTS: Six investigators examined 229 ultrasound studies from 55 patients with accreta and 56 controls for 1374 independent observations. 1205/1374 (87.7% overall, 90% controls, 84.9% cases) studies were given a diagnosis. There were 371 (27.0%) true positives; 81 (5.9%) false positives; 533 (38.8%) true negatives, 220 (16.0%) false negatives, and 169 (12.3%) with uncertain diagnosis. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 53.5%, 88.0%, 82.1%, 64.8%, and 64.8%, respectively. In multivariate analysis, true positives were more likely to have placental lacunae (odds ratio [OR], 1.5; 95% confidence interval [CI], 1.4-1.6), loss of retroplacental clear space (OR, 2.4; 95% CI, 1.1-4.9), or abnormalities on color Doppler (OR, 2.1; 95% CI, 1.8-2.4). CONCLUSION: Ultrasound for the prediction of placenta accreta may not be as sensitive as previously described.