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.

US-guided Interventions to Diagnose and Treat Gynecologic and First-Trimester Disease.

US-guided procedures have an essential role in the diagnosis and treatment of multiple obstetric and gynecologic conditions, can be performed with either transvaginal or transabdominal approaches, and include biopsy of masses, aspiration of fluid collections, injection of therapeutic materials, and saline-infused sonohysterography and hysterosalpingo contrast-enhanced sonography. The full digital presentation is available online. ©RSNA, 2021.

Role of Imaging in Obstetric Interventions: Criteria, Considerations, and Complications.

US has an established role in the prenatal detection of congenital and developmental disorders. Many pregnant women undergo US at 18-20 weeks of gestation for assessment of fetal anatomy and detection of structural anomalies. With advances in fetoscopy and minimally invasive procedures, in utero fetal interventions can be offered to address some of the detected structural and physiologic fetal abnormalities. Most interventions are reserved for conditions that, if left untreated, often cause in utero death or a substantially compromised neonatal outcome. US is crucial for preprocedural evaluation and planning, real-time procedural guidance, and monitoring and assessment of postprocedural complications. Percutaneous needle-based interventions include in utero transfusion, thoracentesis and placement of a thoracoamniotic shunt, vesicocentesis and placement of a vesicoamniotic shunt, and aortic valvuloplasty. Fetoscopic interventions include myelomeningocele repair and tracheal balloon occlusion for congenital diaphragmatic hernia. In rare cases, open hysterotomy may be required for repair of a myelomeningocele or resection of a sacrococcygeal teratoma. Monochorionic twin pregnancies involve specific complications such as twin-twin transfusion syndrome, which is treated with fetoscopic laser ablation of vascular connections, and twin reversed arterial perfusion sequence, which is treated with radiofrequency ablation. Finally, when extended placental support is necessary at delivery for repair of congenital high airway obstruction or resection of lung masses, ex utero intrapartum treatment can be planned. Radiologists should be aware of the congenital anomalies that are amenable to in utero interventions and, when necessary, consider referral to centers where such treatments are offered. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. ©RSNA, 2021.

Outcomes of Monochorionic, Diamniotic Twin Pregnancies with Prenatally Diagnosed Intertwin Weight Discordance.

OBJECTIVE: Monochorionic, diamniotic (MCDA) twin pairs are predisposed to various pregnancy complications due to the unique placental angioarchitecture of monochorionicity. Few studies have evaluated the outcomes of weight-discordant MCDA pairs without selective fetal growth restriction (SFGR) or the risk factors for development of SFGR. This study aims to describe the natural history of expectant, noninvasive management of weight-discordant MCDA twins and to evaluate risk factors associated with progression to SFGR. STUDY DESIGN: This was a retrospective cohort study at a single, tertiary care center in the United States. All MCDA twins with isolated intertwin weight discordance (ITWD) ≥ 20% diagnosed before 26 weeks' gestational age (GA) were included. The primary outcome of descriptive analyses was overall pregnancy outcome, incorporating both survival to delivery and GA at delivery, as defined by the North American Fetal Therapy Network. The secondary outcome was SFGR in one twin (defined as estimated fetal weight < 10% for GA) and factors associated with this progression. Only those with fetal ultrasound (US) within 4 weeks of delivery were included in this secondary analysis. RESULTS: Among 73 MCDA pairs with ITWD, 73% had a good pregnancy outcome, with dual live delivery at a median GA of 33 weeks. Among the 34 pairs with adequate US follow-up, 56% developed SFGR. There were no differences in GA at delivery or discordance at birth between those who did and those who did not develop SFGR. There was a nonsignificant association between increasing ITWD at diagnosis and subsequent development of SFGR. CONCLUSION: Expectant, noninvasive management can be considered in MCDA twin pregnancies with ITWD ≥ 20% diagnosed before 26 weeks. This approach is associated with a good pregnancy outcome in the majority of cases, even after the development of SFGR in the smaller twin. KEY POINTS: · Nearly 75% of weight-discordant mo/di twins have a good pregnancy outcome.. · Weight-discordant mo/di twins deliver at a mean gestational age of 33 weeks without invasive therapy.. · Noninvasive management should be considered for weight-discordant mo/di twins..

Subclinical Atherosclerosis in Pediatric Liver Transplant Recipients: Carotid and Aorta Intima-Media Thickness and Their Predictors.

OBJECTIVE: To investigate prevalence and predictors of cardiovascular risk in pediatric liver transplant recipients using noninvasive markers of subclinical atherosclerosis: carotid intima-media thickness (cIMT) and aorta intima-media thickness (aIMT). STUDY DESIGN: Cross-sectional study of 88 pediatric liver transplant recipients. The cIMT and aIMT were measured by ultrasound imaging using standardized protocol. RESULTS: Participants were 15.4 ± 4.8 years of age, and 11.2 ± 5.6 years post-transplantation. The cIMT and aIMT were both higher in males than females. In analyses adjusted for sex, age, and height, the cIMT was higher in subjects transplanted for chronic/cirrhotic liver disease and lower in subjects on cyclosporine (n = 9) than tacrolimus (n = 71). The cIMT was not associated with rejection history or current corticosteroid use. The cIMT increased with increasing diastolic blood pressure and triglycerides. The aIMT (n = 83) also increased with age, and its rate of increase post-transplant varied by age at transplantation. In adjusted analyses, aIMT was higher in subjects with glucose intolerance. In analysis of patients ≤20 years of age for whom blood pressure percentiles could be calculated (n = 66), aIMT increased with increasing diastolic blood pressure percentile (0.010 mm per 5-percentile; 95% CI, 0.000-0.021; P = 0.05). Neither the cIMT nor the aIMT was associated with obesity, systolic hypertension, or other dyslipidemia at study visit. CONCLUSION: Measures of long-term cardiovascular risk were associated with conditions that are more common in pediatric liver transplant recipients than nontransplanted peers, namely, diastolic hypertension and glucose intolerance. Larger, longitudinal studies are warranted to investigate whether cIMT could be useful for stratifying these patients' cardiovascular risk-and potential need for proactive intervention-during long-term follow-up.

Stage I Twin-Twin Transfusion Syndrome: Outcomes of Expectant Management and Prognostic Features.

OBJECTIVE: This article describes the natural history of stage I twin-twin transfusion syndrome (TTTS) including risk of progression to higher stage TTTS and pregnancy outcomes, and to identify risk factors for progression. STUDY DESIGN: Retrospective cohort study of monochorionic diamniotic (MCDA) twin pregnancies from 2006 to 2016 with expectantly managed Quintero stage I TTTS. RESULTS: A total of 30 MCDA twin pregnancies with expectantly managed stage I TTTS were identified. Of these, eight (26.7%) progressed to higher stage TTTS. Median gestational age (GA) at diagnosis for those that progressed was 18.9 ± 2.9 weeks versus 21.4 ± 3.4 weeks in those that remained stable (p = 0.06). Presence of an arterioarterial (A-A) anastomosis was assessed for 20/30 patients, and eight A-A were identified. Of those, 7/8 (87.5%) remained stable. In the complete cohort, a tiny or transiently visible bladder was noted in seven donor twins. Of these, 4/7 (57.1%) progressed. Excluding one termination, 47/58 (81.0%) fetuses survived. CONCLUSION: With expectant management of stage I TTTS, nearly three-fourths of pregnancies remain stable and most have survival of ≥ 1 twin. A-A anastomoses were not associated with progression to higher stage TTTS, whereas earlier GA at diagnosis or a small or intermittently visible donor bladder may herald greater risk of progression.

Polyhydramnios Affecting a Recipient-like Twin: Risk of Progression to Twin-Twin Transfusion Syndrome and Outcomes.

OBJECTIVE: The significance of polyhydramnios of one twin in the absence of oligohydramnios of the cotwin in monochorionic diamniotic (MCDA) twin pregnancies (polyhydramnios affecting a recipient-like twin [PART]) is unknown. Our aim is to assess the risk of progression to twin-twin transfusion syndrome (TTTS) with PART, progression to ≥ stage II TTTS, and neonatal survival. STUDY DESIGN: This study was a retrospective cohort study of MCDA twin pregnancies with PART evaluated at a referral center from 2008 to 2015. RESULTS: Sixty-four MCDA twin pregnancies with PART were identified. Fifteen (23.4%) progressed to TTTS, including 10 (15.6%) who progressed to ≥ stage II TTTS. Three pregnancies were terminated and one underwent selective reduction by radiofrequency ablation. Overall survival was 113 out of 128 (88.3%). Of those who remained stable, 91.8% (N = 45) had survival of both neonates. In multivariate analysis, the presence of arterioarterial (A-A) anastomosis by in utero Doppler ultrasound was associated with decreased risk of progression to TTTS (odds ratio: 0.12, p = 0.03, 95% confidence interval: 0.02-0.78). CONCLUSION: Most MCDA twin pregnancies with PART do not progress to TTTS and have a favorable prognosis. Progression rates are higher than observed in uncomplicated MCDA twins; however, so close surveillance is warranted. The presence of an A-A anastomosis appears to confer decreased risk of progression to TTTS.

Hepatic steatosis after pediatric liver transplant.

Hepatic steatosis develops after liver transplantation (LT) in 30% of adults, and nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in nontransplanted children. However, posttransplant steatosis has been minimally studied in pediatric LT recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. In this single-center study of pediatric patients transplanted 1988-2015 (n = 318), 31% of those with any posttransplant biopsy (n = 271) had ≥ 1 biopsy with steatosis. Median time from transplant to first biopsy with steatosis was 0.8 months (interquartile range [IQR], 0.3-6.5 months) and to last biopsy with steatosis was 5.5 months (IQR, 1.0-24.5 months); 85% of patients with steatosis also had for-cause biopsies without steatosis. All available for-cause biopsies were re-evaluated (n = 104). Of 9 biopsies that could be interpreted as nonalcoholic steatohepatitis (NASH)/borderline NASH, with steatosis plus inflammation or ballooning, 8 also had features of cholestasis or rejection. Among 70 patients with surveillance biopsies 3.6-20.0 years after transplant, only 1 overweight adolescent had a biopsy with NAFLD (grade 1 steatosis, mild inflammation, no ballooning or fibrosis)-despite a 30% prevalence of overweight/obesity in the cohort and 27% with steatosis on previous for-cause biopsy. Steatosis on preceding for-cause biopsy was not associated with portal (P = 0.49) or perivenular fibrosis (P = 0.85) on surveillance biopsy. Hepatic steatosis commonly develops early after transplant in children and adolescents, but it rarely persists. Biopsies that did have steatosis with NASH characteristics were all for-cause, mostly in patients with NAFLD risk factors and/or confounding causes of liver damage. Prospective studies that follow children into adulthood will be needed to evaluate if and when hepatic steatosis presents a longterm risk for pediatric LT recipients. Liver Transplantation 23 957-967 2017 AASLD.

Sonography Predicts Liver Steatosis in Patients With Chronic Hepatitis B.

OBJECTIVES: Liver inflammation and fibrosis may impair the ability of sonography to identify steatosis. We determined the accuracy of sonography in grading steatosis in patients with chronic hepatitis B compared to liver biopsy. METHODS: We conducted was a single-center retrospective study of all nontransplanted patients with chronic hepatitis B undergoing sonography and liver biopsy between 2004 and 2014 (n = 109). Steatosis was graded by sonography as none, mild, moderate, or severe. Liver histologic analysis graded steatosis (0, <5%; 1, <33%; 2, <66%; or 3, ≥66%) and staged fibrosis (F0-F4). Severe steatosis was defined as grade 2 or 3. Clinical variables within 6 months of liver biopsy were collected, and the association with steatosis was analyzed by univariate logistic regression. RESULTS: Patients were predominantly Asian (83%), male (62%), and hepatitis B e antigen negative (62%). Twenty-nine percent of patients were obese; 9% had diabetes mellitus; 23% had hypertension; and 31% had dyslipidemia. Forty-four percent of patients had steatosis on liver biopsy; 8% had severe steatosis. The presence of any steatosis on sonography correctly identified any steatosis on liver biopsy in 29 of 48 patients (60%). The absence of steatosis on sonography ruled out severe steatosis on biopsy (specificity, 100%). Severe steatosis on sonography correctly predicted the presence of severe steatosis on liver biopsy (89%; P < .001); however, it was not accurate at distinguishing between steatosis grades. Predictors of biopsy-proven steatosis on univariate analysis included diabetes (P < .001), hypertension (P = .03), hypercholesterolemia (P = .02), and body mass index (P < .001). CONCLUSIONS: Sonography had excellent accuracy in identifying patients with steatosis on biopsy. Abdominal sonography can be used to predict clinically important steatosis in patients with chronic hepatitis B.

US Findings in Patients at Risk for Pancreas Transplant Failure.

Purpose To determine if ultrasonographic (US) findings, including Doppler US findings, are associated with subsequent pancreas transplant failure. Materials and Methods A cohort of adult patients who underwent pancreas transplantation at a tertiary institution over the course of 10 years (from 2003 to 2012) was retrospectively evaluated for failure, which was defined as return to insulin therapy or surgical graft removal. The institutional review board provided a waiver of informed consent. All US images obtained within the 1st postoperative year were reviewed for three findings: arterial flow (presence or absence of intraparenchymal forward diastole flow), splenic vein thrombus, and edema. These findings were correlated with pancreas graft failure within 1-year after surgery by using Cox proportional hazards models and hazard ratios. Results A total of 228 transplants were included (mean patient age, 41.6 years; range, 19-57 years; 122 men, 106 women). Absent or reversed arterial diastolic flow was identified in nine of 20 failed transplants (sensitivity, 45%; 95% confidence interval [CI]: 23, 68) and in 15 of 208 transplants that survived (specificity, 93% [193 of 208]; 95% CI: 89, 96). The Cox proportional hazard ratio was 6.2 (95% CI: 3.1, 12.4). Splenic vein thrombus was identified in 10 of 20 failed transplants (sensitivity, 50%; 95% CI: 27, 73) and in 25 of 208 transplants that survived (specificity, 88% [183 of 208]; 95% CI: 83, 92). The Cox proportional hazard ratio was 4.2 (95% CI: 2.4, 7.4). Edema had the lowest specificity (Cox proportional hazard ratio, 2.0; 95% CI: 1.3, 2.9). In the multivariate analysis, only absent or reversed arterial diastolic flow remained significantly associated with transplant failure (adjusted hazard ratio, 3.6; 95% CI: 1.0, 12.8; P = .045). Conclusion Absent or reversed diastolic arterial Doppler flow has a stronger association with transplant failure than does splenic vein thrombus or edema. (©) RSNA, 2016.

In Utero Diagnosis of Unilateral Bronchial Atresia: One Pathologically Proven and 2 Presumed Cases With Similar Sonographic Findings at Presentation.

Unilateral bronchial atresia is a rare prenatal diagnosis that can be confused with other congenital lung abnormalities, particularly congenital pulmonary airway malformation. Accurate distinction between these entities is important for appropriate clinical care and prognosis. Familiarity of the key imaging and clinical features of unilateral bronchial atresia in comparison to other fetal lung abnormalities should increase the likelihood of achieving a timely and accurate diagnosis.

Targeted hepatic sonography during clinic visits for detection of fatty liver in overweight children: a pilot study.

OBJECTIVES: The purpose of this study was to assess the feasibility and utility of targeted hepatic sonography to evaluate for hepatic steatosis during a subspecialty clinic visit. METHODS: In this pilot study, we performed targeted hepatic sonography on 25 overweight children aged 7 to 17 years consecutively seen in a pediatric obesity clinic. Long-axis images of the right lobe of the liver and a split-screen image of liver and spleen were taken. Images were interpreted in real time by the radiologist and shown to the family. Demographics, clinical measurements, and laboratory parameters were also collected from the specialty clinic visit on the same day. RESULTS: Sonography required a median of 4 minutes during the visit (interquartile range, 3-5 minutes). All consented patients completed the study. The median alanine aminotransferase (ALT) level was 23 U/L in those with no steatosis (n = 14), 26 U/L with mild steatosis (n = 6), and 41 U/L with moderate/marked steatosis (n = 5). Children with ALT levels of 25 to 50 U/L had very variable sonographic measures of hepatic steatosis. When the participants were categorized by the overall degree of fatty liver, hepatic steatosis was significantly associated with the aspartate aminotransferase level (P = .028), ALT level (P = .003), and diastolic blood pressure (P = .05) but did not correlate with age, sex, Latino race, or insulin resistance. CONCLUSIONS: Targeted hepatic sonography added information not apparent from routine ALT screening and provided immediate feedback to clinicians and families about the effect of obesity on end organs. This examination could be a feasible, informative addition to screening for children at high risk for nonalcoholic fatty liver disease who are seen in clinics that specialize in obesity.

The natural history of fetal gallstones: a case series and updated literature review.

INTRODUCTION: The incidence of fetal gallstones is estimated at 0.45% and its clinical relevance after birth remains unknown. This study aimed to describe the natural history of fetal gallstones and their clinical sequelae after birth. METHODS: We queried a database of fetuses referred for second and third trimester sonograms performed for high-risk pregnancies, and identified cases with fetal gallstones (1996-2019). Demographics, prenatal/postnatal imaging findings, and clinical sequelae were collected. A literature review was performed according to PRISMA guidelines. RESULTS: We screened approximately 200,000 obstetric sonograms; 34 fetuses were found to have cholelithiasis. The median gestational age at the time of sonogram was 35 weeks (range 22-38). Fifty-six percent were female and 11.8% were twin pregnancies with one affected fetus. Median maternal age was 28 years (range 17-42). Eight fetuses underwent postnatal imaging and 4 had persistent cholelithiasis. There was one case of in utero demise. Two patients had structural anomalies (renal and cardiac) by sonogram. A subset of 17 patients was followed long-term (range 3-20 years), and none developed clinical sequelae from cholelithiasis. DISCUSSION/CONCLUSIONS: No child developed postnatal clinical sequelae related to cholelithiasis identified in utero. Fetal cholelithiasis can be managed expectantly without follow-up imaging in asymptomatic patients.

Diastolic Cardiac Pathology and Clinical Twin-Twin Transfusion Syndrome in Monochorionic/Diamniotic Twins.

OBJECTIVE: to identify differences in echocardiographic profiles of monochorionic/diamniotic pregnancies with early or mild twin-twin transfusion syndrome (TTTS), compared to monochorionic/diamniotic twins affected only by discordant growth or discordant fluid. STUDY DESIGN: retrospective evaluation of sonograms and echocardiograms of twin pregnancies referred for suspected TTTS. RESULTS: 112 monochorionic/diamniotic pairs were studied. 41 did not have/develop TTTS, 61 had Stage I/II TTTS. Ten developed TTTS after initially not meeting criteria. TTTS recipients had a higher rate of venous Doppler or tricuspid inflow abnormalities than purported "recipients" in non-TTTS pregnancies (86% vs. 37%, P<0.001). TTTS recipients had shorter tricuspid inflow duration/RR intervals than non-TTTS fetuses (32+/-6% versus 37+/-4%, P<0.001). Logistic regression and recursive partitioning identified shorter tricuspid inflow duration, longer isovolumic relaxation, and ductus venosus abnormality associated with TTTS. CONCLUSION: Diastolic pathology, specifically shorter tricuspid inflow duration, may be considered a hallmark of TTTS distinguishing these pregnancies from other monochorionic/diamniotic twin complications.

Bilateral Choroid Plexus Papillomas Diagnosed by Prenatal Ultrasound and MRI.

We present a rare prenatal diagnosis of bilateral choroid plexus papillomas by obstetrical ultrasound and fetal MRI at 20 weeks 6 days gestation. The fetus demonstrated bilateral enlarged, echogenic choroid plexus with increased Doppler flow suggestive of vascularized choroid tissue. Same-day fetal MRI demonstrated that the choroid plexus appeared enlarged bilaterally without definite hemorrhage. The combined features on ultrasound and MRI suggested bilateral choroid plexus papillomas with increased cerebrospinal fluid production, leading to ventriculomegaly and enlarged extra-axial spaces. The diagnosis was confirmed by postnatal pathology, which demonstrated WHO grade II atypical choroid plexus papillomas.

ACR Appropriateness Criteria® Second and Third Trimester Screening for Fetal Anomaly.

The Appropriateness Criteria for the imaging screening of second and third trimester fetuses for anomalies are presented for fetuses that are low risk, high risk, have had soft markers detected on ultrasound, and have had major anomalies detected on ultrasound. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Perinatal outcome of conservative management versus fetal intervention for twin reversed arterial perfusion sequence with a small acardiac twin.

OBJECTIVE: To examine the outcomes of patients with twin reversed arterial perfusion (TRAP) sequence in which the acardiac twin was

ACR Appropriateness Criteria® Assessment of Gravid Cervix.

Preterm birth remains the leading cause of perinatal morbidity and mortality. Although the greatest risk factor for preterm birth is a history of prior preterm birth, a short cervical length (≤25 mm) before 24 weeks' gestational age is also associated with increased risk of spontaneous preterm delivery. As such, cervical length assessment has become of particular interest in predicting those patients at risk for preterm birth. Other clinical scenarios (eg, preterm labor, induction of labor, and active labor) may arise, in which assessment of the cervix may be of interest. Ultrasound is the mainstay imaging modality for assessing the gravid cervix, with transvaginal ultrasound recommended in patients at high risk for preterm birth or suspected preterm labor. Transperineal ultrasound is an alternate approach in those cases where transvaginal ultrasound in contraindicated. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Nuchal Translucency Evaluation at 11 to 14 Weeks of Gestation.

A fetus with an increased nuchal translucency at 11 to 14 weeks gestation is at risk for aneuploidy, genetic syndromes, structural anomalies, and intrauterine fetal demise in both single and twin gestations. In addition to referral to genetics for counseling and consideration of diagnostic genetic testing, a detailed anatomic survey and fetal echocardiogram are indicated in the second trimester to screen for congenital malformations and major heart defects. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.