Prospective study of quantitative liver MRI in cystic fibrosis: feasibility and comparison to PUSH cohort ultrasound.

BACKGROUND: Pediatric radiologists can identify a liver ultrasound (US) pattern predictive of progression to advanced liver disease. However, reliably discriminating these US patterns remains difficult. Quantitative magnetic resonance imaging (MRI) may provide an objective measure of liver disease in cystic fibrosis (CF). OBJECTIVE: The purpose of this study was to determine if quantitative MRI, including MR elastography, is feasible in children with CF and to determine how quantitative MRI-derived metrics compared to a research US. MATERIALS AND METHODS: A prospective, multi-institutional trial was performed evaluating CF participants who underwent a standardized MRI. At central review, liver stiffness, fat fraction, liver volume, and spleen volume were obtained. Participants whose MRI was performed within 1 year of US were classified by US pattern as normal, homogeneous hyperechoic, heterogeneous, or nodular. Each MRI measure was compared among US grade groups using the Kruskal-Wallis test. RESULTS: Ninety-three participants (51 females [54.8%]; mean 15.6 years [range 8.1-21.7 years]) underwent MRI. MR elastography was feasible in 87 participants (93.5%). Fifty-eight participants had an US within 1 year of MRI. In these participants, a nodular liver had significantly higher stiffness (P<0.01) than normal or homogeneous hyperechoic livers. Participants with a homogeneous hyperechoic liver had a higher fat fraction (P<0.005) than others. CONCLUSION: MR elastography is feasible in children with CF. Participants with a nodular pattern had higher liver stiffness supporting the US determination of advanced liver disease. Participants with a homogeneous hyperechoic pattern had higher fat fractions supporting the diagnosis of steatosis.

Heterogeneous liver on research ultrasound identifies children with cystic fibrosis at high risk of advanced liver disease.

BACKGROUND: This study examines whether heterogeneous (HTG) pattern on liver ultrasound (US) identifies children at risk for advanced cystic fibrosis liver disease (aCFLD). METHODS: Prospective 6-year multicenter case-controlled cohort study. Children with pancreatic insufficient cystic fibrosis (CF) aged 3-12 years without known cirrhosis underwent screening US. Participants with HTG were matched (by age, Pseudomonas infection status and center) 1:2 with participants with normal (NL) US pattern. Clinical status and laboratory data were obtained annually and US bi-annually for 6 years. Primary endpoint was development of nodular (NOD) US pattern consistent with aCFLD. RESULTS: 722 participants underwent screening US, with 65 HTG and 592 NL. Final cohort included 55 HTG and 116 NL with ≥ 1 follow-up US. ALT, AST, GGTP, FIB-4, GPR and APRI were higher, and platelets were lower in HTG compared to NL. HTG had a 9.5-fold increased incidence (95% confidence interval [CI]:3.4, 26.7, p<0.0001, 32.7% vs 3.4%) of NOD versus NL. HTG had a sensitivity of 82% and specificity of 75% for subsequent NOD. Negative predictive value of a NL US for subsequent NOD was 96%. Multivariate logistic prediction model that included baseline US, age, and log(GPR) improved the C-index to 0.90 compared to only baseline US (C-index 0.78). Based on survival analysis, 50% of HTG develop NOD after 8 years. CONCLUSIONS: Research US finding of HTG identifies children with CF with a 30-50% risk for aCFLD. A score based on US pattern, age and GPR may refine the identification of individuals at high risk for aCFLD. CLINICAL TRIAL REGISTRATION: Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF: NCT 01,144,507 (observational study, no consort checklist).

Urinary excretion of low-osmolar contrast media during small-bowel follow-through studies in infants is not definitive evidence of bowel perforation.

BACKGROUND: Presence of contrast agent in the urinary system in infants after small-bowel follow-through study with low-osmolar contrast media has been described as a sign of bowel perforation. OBJECTIVE: To evaluate how often the presence of contrast agent in the bladder after small-bowel follow-through is a reliable sign of bowel perforation or necrosis. MATERIALS AND METHODS: From the radiology information system, we retrieved imaging reports of infants evaluated with small-bowel follow-through and findings of contrast agent in the bladder. We retrieved demographic and clinical information from the medical records. Presence of bladder contrast medium was considered true-positive evidence of bowel perforation or necrosis if confirmed by pneumoperitoneum, extraluminal contrast agent, surgery or pathology within 3 days of the small-bowel follow-through. False-positives for bowel perforation or necrosis were based on surgical findings or clinical follow-up. RESULTS: Of the 207 infants who had small-bowel follow-through, 18 infants (12 boys; mean age 50 days, range 14 days to 8.5 months) had contrast medium in the bladder after the small-bowel follow-through. Fifteen of the 18 (83.3%) had a history of prematurity and 11 had prior abdominal surgery. Four of the 18 (22.2%) had bowel perforation or necrosis at surgery or pathology performed more than 3 days after the small-bowel follow-through and were considered indeterminate and excluded. Eight of the remaining 14 infants (57.1%) had bowel perforation or necrosis based on surgical evidence of perforation or pathology confirmation of necrosis (n=6), pneumoperitoneum (n=1) or contrast agent leakage from enterocutaneous fistula (n=1). Six of the 14 (42.9%) were false-positives, without evidence of bowel perforation or necrosis based on clinical follow-up (n=4) or surgery (n=2). CONCLUSION: Demonstration of urinary contrast agent post small-bowel follow-through with low-osmolar contrast medium in newborns/infants with complex medical problems is not a definitive indication of bowel perforation or necrosis. More than one-third of our patients with contrast medium in the bladder did not have bowel perforation or necrosis.

Long-term follow-up and liver outcomes in children with cystic fibrosis and nodular liver on ultrasound in a multi-center study.

BACKGROUND: Nodular liver (NOD) in cystic fibrosis (CF) suggests advanced CF liver disease (aCFLD); little is known about progression of liver disease (LD) after detection of sonographic NOD. METHODS: Clinical, laboratory, and ultrasound (US) data from Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in CFLD Study participants with NOD at screening or follow-up were compared with normal (NL). Linear mixed effects models were used for risk factors for LD progression and Kaplan-Meier estimator for time-to-event. RESULTS: 54 children with NOD (22 screening, 32 follow-up) and 112 NL were evaluated. Baseline (BL) and trajectory of forced expiratory volume, forced vital capacity, height/BMI z-scores were similar in NOD vs NL. Platelets were lower in NOD at BL (250 vs 331×103/microL; p < 0.001) and decreased by 8600/year vs 2500 in NL. Mean AST to Platelet Ratio Index (1.1 vs 0.4; p < 0.001), Fibrosis-4 Index (0.4 vs 0.2, p < 0.001), and spleen size z-score (SSZ) [1.5 vs 0.02; p < 0.001] were higher in NOD at BL; SSZ increased by 0.5 unit/year in NOD vs 0.1 unit/year in NL. Median liver stiffness (LSM) by transient elastography was higher in NOD (8.2 kPa, IQR 6-11.8) vs NL (5.3, 4.2-7, p < 0.0001). Over 6.3 years follow-up (1.3-10.3), 6 NOD had esophageal varices (cumulative incidence in 10 years: 20%; 95% CI: 0.0%, 40.0%), 2 had variceal bleeding, and 2 underwent liver transplantation; none had ascites or hepatic encephalopathy. No NL experienced liver-related events. CONCLUSIONS: NOD developed clinically evident portal hypertension faster than NL without worse growth or lung disease.

Health-related Quality of Life in a Prospective Study of Ultrasound to Detect Cystic Fibrosis-related Liver Disease in Children.

OBJECTIVES: Cystic fibrosis liver disease (CFLD) begins early in life. Symptoms may be vague, mild, or nonexistent. Progressive liver injury may be associated with decrements in patient health before liver disease is clinically apparent. We examined Health-Related Quality of Life (HRQOL) in children enrolled in a multi-center study of CFLD to determine the impact of early CFLD on general and disease-specific QOL. METHODS: Ultrasound (US) patterns of normal (NL), heterogeneous (HTG), homogeneous (HMG), or nodular (NOD) were assigned in a prospective manner to predict those at risk for advanced CFLD. Parents were informed of results. We assessed parent/child-reported (age ≥5 years) HRQOL by PedsQL 4.0 Generic Core and CF Questionnaire-revised (CFQ-R) prior to US and annually. HRQOL scores were compared by US pattern at baseline (prior to US), between baseline and 1 year and at 5 years. Multivariate analysis of variance (MANOVA) with Hotelling-Lawley trace tested for differences among US groups. RESULTS: Prior to US, among 515 participants and their parents there was no evidence that HTG or NOD US was associated with reduced PedsQL/CFQ-R at baseline. Parents of NOD reported no change in PedsQL/CFQ-R over the next year. Child-report PedsQL/CFQ-R (95 NL, 20 NOD) showed improvement between baseline and year 5 for many scales, including Physical Function. Parents of HMG children reported improved CFQ-R scores related to weight. CONCLUSIONS: Early undiagnosed or pre-symptomatic liver disease had no impact on generic or disease-specific HRQoL, and HRQoL was remarkably stable in children with CF regardless of liver involvement.

Chest CT in the evaluation of child abuse - When is it useful?

BACKGROUND: Indications for chest CT in evaluation of child abuse are unknown. OBJECTIVE: Determine which groups of children can best benefit from chest CT. PARTICIPANTS AND SETTING: 10-year (1/2010 to 12/2019) retrospective study of children <3 years who had chest CT within 3 days of the initial skeletal survey. METHODS: Demographic and clinical information were obtained from medical records. Two pediatric radiologists reviewed, independently and blinded to clinical information, anonymized rib X-rays (initial and follow up when available) and chest CT. Disagreements were resolved by a third pediatric radiologist. Agreement was evaluated using kappa statistics. Number and percentage of fractures were analyzed by negative binomial models and chi-square tests, respectively. RESULTS: 50 children (21 females) with average age of 9.7 months, 27 of whom had follow-up radiography. Agreement on initial and follow-up X-rays was substantial (k = 0.72) and perfect (k = 1.00), respectively, and almost perfect (k = 0.82) for CT scans. Chest CT demonstrated more fractures than X-ray, both initially (112 vs. 42, p < 0.0001) and at follow-up (93 vs. 49, p < 0.0001). Significantly more additional fractures were found at CT (11/13, 84.6 %) in patients with positive than in those with negative initial surveys (7/37, 18.9 %, p < 0.001). Ten initial surveys had only indeterminate fractures; four of them had fractures and six had no fractures on CT. Chest CT missed one patient (1/27, 3.7 %) with acute nondisplaced anterior rib fractures. CONCLUSION: Chest CT can be considered in children with negative skeletal survey and high clinical suspicion for child abuse, and when the diagnosis of rib fractures is indeterminate.

Radiologists' Diagnostic Performance in Differentiation of Rickets and Classic Metaphyseal Lesions on Radiographs: A Multicenter Study.

BACKGROUND. Despite evidence supporting the specificity of classic metaphyseal lesions (CML) for the diagnosis of child abuse, some medicolegal practitioners claim that CML result from rickets rather than trauma. OBJECTIVE. The purpose of this study was to evaluate radiologists' diagnostic performance in differentiating rickets and CML on radiographs. METHODS. This retrospective seven-center study included children younger than 2 years who underwent knee radiography from January 2007 to December 2018 and who had either rickets (25-hydroxyvitamin D level < 20 ng/mL and abnormal knee radiographs) or knee CML and a diagnosis of child abuse from a child abuse pediatrician. Additional injuries were identified through medical record review. Radiographs were cropped and zoomed to present similar depictions of the knee. Eight radiologists independently interpreted radiographs for diagnoses of rickets or CML, rated confidence levels, and recorded associated radiographic signs. RESULTS. Seventy children (27 girls, 43 boys) had rickets; 77 children (37 girls, 40 boys) had CML. Children with CML were younger than those with rickets (mean, 3.7 vs 14.2 months, p < .001; 89.6% vs 5.7% younger than 6 months; 3.9% vs 65.7% older than 1 year). All children with CML had injuries in addition to the knee CML identified at physical examination or other imaging examinations. Radiologists had almost perfect agreement for moderate- or high-confidence interpretations of rickets (κ = 0.92) and CML (κ = 0.89). Across radiologists, estimated sensitivity, specificity, and accuracy for CML for moderate- or high-confidence interpretations were 95.1%, 97.0%, and 96.0%. Accuracy was not significantly different between pediatric and nonpediatric radiologists (p = .20) or between less experienced and more experienced radiologists (p = .57). Loss of metaphyseal zone of provisional calcification, cupping, fraying, and physeal widening were more common in rickets than CML, being detected in less than 4% of children with CML. Corner fracture, bucket-handle fracture, subphyseal lucency, deformed corner, metaphyseal irregularity, and subperiosteal new bone formation were more common in CML than rickets, being detected in less than 4% of children with rickets. CONCLUSION. Radiologists had high interobserver agreement and high diagnostic performance for differentiating rickets and CML. Recognition that CML mostly occur in children younger than 6 months and are unusual in children older than 1 year may assist interpretation. CLINICAL IMPACT. Rickets and CML have distinct radiographic signs, and radiologists can reliably differentiate these two entities.

Multi-modality imaging characteristics of costochondral fractures, a highly specific rib fracture for child abuse.

BACKGROUND: Rib fractures in young children are strongly associated with nonaccidental trauma (NAT). Costochondral junction (CCJ) fractures are unique with most being identified in the healing phase on radiographs. NAT-associated CCJ fractures, therefore, may be underdiagnosed. Improved diagnoses of CCJ fractures may lead to better identification of NAT. OBJECTIVE: To document the association of CCJ fractures with NAT, and improve CCJ fracture recognition by documenting the imaging features with multiple radiologic modalities. MATERIALS AND METHODS: Children, ages 0-4 years, with CCJ fractures on radiologic reports were identified over a 10-year period. All available radiographic skeletal surveys, chest radiographs, computed tomography (CT), magnetic resonance imaging (MRI) and ultrasound (US) studies were reviewed. We chose CT as the radiologic gold standard. Imaging patterns of the primary fracture and healing changes were documented. The diagnosis of NAT by the child protective team was documented. RESULTS: One hundred and nine CCJ fractures were found in 22 patients, 21 of whom were diagnosed with NAT (95.5%). Radiographic skeletal survey identified 34.6% of CCJ fractures (P < 0.0001) with a sensitivity of 32.5% and specificity of 99.2%. MRI identified 50.0% of CCJ fractures with a sensitivity of 42.9% and specificity of 98.1%. CONCLUSION: CCJ fractures are highly specific for NAT. As sensitivity is low for radiographic skeletal survey in CCJ fracture diagnosis compared with CT, CT may have a role in confirming a clinical suspicion of NAT.

Diagnosis and Follow-up of Incidental Liver Lesions in Children.

ABSTRACT: Incidental liver lesions are identified in children without underlying liver disease or increased risk of hepatic malignancy in childhood. Clinical and imaging evaluation of incidental liver lesions can be complex and may require a multidisciplinary approach. This review aims to summarize the diagnostic process and follow-up of incidental liver lesions based on review of the literature, use of state-of-the-art imaging, and our institutional experience. Age at presentation, gender, alpha fetoprotein levels, tumor size, and imaging characteristics should all be taken into consideration to optimize diagnosis process. Some lesions, such as simple liver cyst, infantile hemangioma, focal nodular hyperplasia (FNH), and focal fatty lesions, have specific imaging characteristics. Recently, contrast-enhanced ultrasound (CEUS) was Food and Drug Administration (FDA)-approved for the evaluation of pediatric liver lesions. CEUS is most specific in lesions smaller than 3 cm and is most useful in the diagnosis of infantile hemangioma, FNH, and focal fatty lesions. The use of hepatobiliary contrast in MRI increases specificity in the diagnosis of FNH. Recently, lesion characteristics in MRI were found to correlate with subtypes of hepatocellular adenomas and associated risk for hemorrhage and malignant transformation. Biopsy should be considered when there are no specific imaging characteristics of a benign lesion. Surveillance with imaging and alpha fetoprotein (AFP) should be performed to confirm the stability of lesions when the diagnosis cannot be determined, and whenever biopsy is not feasible.

Whole-Spine MRI in Children With Suspected Abusive Head Trauma.

BACKGROUND. Abusive head trauma (AHT) in children has recently been associated with findings on cervical spine MRI. OBJECTIVE. The purpose of this study was to evaluate whether whole-spine MRI in children with suspected AHT shows additional abnormalities not identified on cervical spine MRI. METHODS. This retrospective study included 256 children younger than 3 years old (170 boys, 86 girls; mean age, 5.9 months) who underwent skeletal survey and head MRI for suspected child abuse from January 2019 to December 2020. Per institutional protocol, children with suspected AHT also underwent whole-spine MRI. AHT diagnoses were established by a combination of clinical information from medical record review and injuries described in reports from skeletal survey, head MRI, and head CT (if performed). Two pediatric neuroradiologists independently reviewed whole-spine MRI examinations for presence and level of intraspinal hemorrhage (classified as subarachnoid, subdural, or epidural), ligamentous injury, spinal cord edema, and vertebral fractures; subdural hematoma, epidural hematoma, ligamentous injury, and fracture unidentified by skeletal survey were considered major findings. Interobserver agreement was assessed; a third radiologist resolved discrepancies. Findings were summarized with attention to injuries isolated to the thoracolumbar spine. RESULTS. A total of 148 of 256 (57.8%) children underwent whole-spine MRI. AHT was diagnosed in 79 of 148 (53.4%) children who underwent whole-spine MRI versus in 2 of 108 (1.9%) who did not undergo whole-spine MRI (p < .001). Interobserver agreement, expressed as kappa coefficient, was 0.90 for intraspinal hemorrhage, 0.69 for ligamentous injury, 0.66 for spinal cord edema, and 0.95 for fracture. A total of 57 of 148 (38.5%) whole-spine MRI examinations showed injuries, and 34 of 148 (23.0%) showed injuries localized to the thoracolumbar spine. A total of 47 of 148 (31.8%) whole-spine MRI examinations showed major findings, of which 24 (51.1%) were localized to the thoracolumbar spine. Isolated thoracolumbar injuries included 23 of 34 spinal subdural hematomas, 2 of 3 spinal epidural hematomas, and 9 of 11 vertebral fractures, including five fractures not identified by skeletal survey. Diagnosis of AHT was more common in children with positive, versus negative, whole-spine MRI examinations (76.8% vs 39.1%; p < .001). CONCLUSION. In children with suspected AHT, whole-spine MRI commonly shows isolated thoracolumbar injuries. CLINICAL IMPACT. The results support performing whole-spine MRI rather than cervical spine MRI in children with suspected AHT.

ACR Appropriateness Criteria® Methodology.

The ACR Appropriateness Criteria® (AC) are evidence-based guidelines that guide physicians on appropriate image ordering. The AC development and revision process follows a transparent methodology that includes the systematic analysis of current medical literature from peer-reviewed journals and the application of well-established guidelines standards (the Institute of Medicine's Clinical Practice Guidelines We Can Trust) and methodologies (the RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development and Evaluation) to rate the benefits and potential risks, or appropriateness, of imaging and treatment procedures for specific clinical scenarios. In the October 2020 release, the methodology is applied in the development of 198 AC documents covering 1,760 clinical scenarios to make more than 8,815 recommendations, authored by more than 600 members representing multiple expert societies, and using more than 6,200 references. The ACR is recognized as a qualified provider-led entity by CMS for the development of appropriate use criteria. This paper describes the methodology and illustrates adherence to the process in the development of the AC.

Ultrasound of retained gonads in children and young women with androgen insensitivity syndrome.

INTRODUCTION: Gonadal management in androgen insensitivity syndrome (AIS) patients has been controversial due to low risk of testicular cancer. Our study evaluated the role of ultrasound (US) in screening for malignancy in retained gonads in AIS patients. METHODS: This was a retrospective study (2001-2020) of gonadal US in patients with AIS. Demographics and clinical information were retrieved from the medical records. US studies were reviewed for nodule presence, size, echotexture, and change on follow-up studies. When available, pathology correlation was performed. Two-tailed t-test was used to compare age and development of nodules clinically or on US examination. RESULTS: 13 patients were included with a median age was 9.9 years (range 3.8-18.4 years). In 11 patients, gonads were in the inguinal canals on either initial or follow-up US. No nodules were palpable on physical examination, but nodules were detected in ten testicles by US in five of 13 patients (41.7%). Presence of nodules was significantly (p = 0.0038) associated with older age. The largest nodule size varied from 0.4 to 2.2 cm (average 0.9 ± 0.5 cm) and most (7/10, 70%) were hypoechoic. Finding testicular nodules on US led to change in management in three patients; bilateral gonadectomies, unilateral gonadectomy, and gonadal excisional biopsies (Figure). Pathology demonstrated Sertoli hamartoma in these patients, and in an additional two patients who underwent post-puberty gonadectomy. No malignancy was found in any specimen. DISCUSSION: Preservation of the gonads in children with AIS is associated with low risk for malignant transformation. The role of US surveillance of the gonads is unknown. In our series on 13 patients, most of the visualized 24 gonads (22/24, 91.7%) were localized in the groins in either the first or follow-up US studies. Nodules were detected in ten gonads in five of 13 patients (41.7%). Most of these gonads (8/10) had numerous nodules, most (7/10) were hypoechoic with average diameter of the largest nodule of 0.9 ± 0.5 cm. Pathology in 5 patients demonstrated Sertoli hamartomas in all of the gonads. No malignancy was found. In our series, gonadal nodules led to either gonadectomies or excisional biopsies in three patients. Our study has several limitations, related to the retrospective nature of the study and the small size of our series. CONCLUSION: Multiple testicular nodules were commonly detected by US in AIS patients and were not associated with malignancy. Therefore, we are concerned that US screening can lead to unnecessary excisional biopsies and orchiectomies.

Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis.

Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (rs =  - 0.28, P = 0.0071) and positive correlation with aspartate aminotransferase-to-platelet ratio index (rs = 0.38, P = 0.0002), Fibrosis-4 index (rs = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; rs = 0.35, P = 0.0017), GGT-to-platelet ratio (rs = 0.35, P = 0.003), and US spleen size z-score (rs = 0.27, P = 0.0073). Conclusion: VCTE is associated with US patterns and conventional markers in patients with liver disease with CF.

ACR Appropriateness Criteria® Seizures-Child.

In children, seizures represent an extremely heterogeneous group of medical conditions ranging from benign cases, such as a simple febrile seizure, to life-threatening situations, such as status epilepticus. Underlying causes of seizures also represent a wide range of pathologies from idiopathic cases, usually genetic, to a variety of acute and chronic intracranial or systemic abnormalities. This document discusses appropriate utilization of neuroimaging tests in a child with seizures. The clinical scenarios in this document take into consideration different circumstances at the time of a child's presentation including the patient's age, precipitating event (if any), and clinical and electroencephalogram findings and include neonatal seizures, simple and complex febrile seizures, post-traumatic seizures, focal seizures, primary generalized seizures in a neurologically normal child, and generalized seizures in neurologically abnormal child. This practical approach aims to guide clinicians in clinical decision-making and to help identify efficient and appropriate imaging workup. 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.

Initial experience with contrast-enhanced ultrasound in the first week after liver transplantation in children: a useful adjunct to Doppler ultrasound.

BACKGROUND: Doppler US is the primary screening for post-liver transplant vascular complications, but indeterminate findings require further imaging. OBJECTIVE: To evaluate whether contrast-enhanced US improves diagnostic assessment of vascular complications suspected by Doppler US. MATERIALS AND METHODS: We retrospectively reviewed Doppler US and contrast-enhanced US studies obtained in the first week following liver transplant. Doppler US was performed twice daily for the first 5 postoperative days, and CEUS in the first postoperative day and when vascular complications were suspected. We correlated Doppler US and CEUS with surgical findings, and clinical and imaging follow-up. We evaluated Doppler US and CEUS quality in demonstrating the main hepatic artery (HA) at the porta hepatis as follows: Grade 0 = not seen, Grade 1 = only segments, Grade 2 = entire main HA, and Grade 3 = entire main HA to the intrahepatic branching. We used a Wilcoxon signed rank test to test the difference between Doppler US and CEUS methods. RESULTS: Twenty-nine children (15 girls, 14 boys) were identified, with median age 2.2 years (range 0.5-17.6 years). The most common transplant indication was biliary atresia (n=13). There was significantly (P<0.0001) improved main HA visualization with CEUS. In five children, CEUS was performed to evaluate suspected vascular complications; CEUS confirmed normal vascularity in two. CEUS demonstrated portal vein thrombosis (n=2) and main HA thrombosis (n=1), confirmed at surgery. In one child the main HA thrombosis was missed; marked HA narrowing was seen retrospectively on CEUS. CONCLUSION: Immediately following liver transplantation, CEUS improves main HA visualization and diagnostic assessment of vascular complications.

ACR Appropriateness Criteria® Antenatal Hydronephrosis-Infant.

Antenatal hydronephrosis is the most frequent urinary tract anomaly detected on prenatal ultrasonography. It occurs approximately twice as often in males as in females. Most antenatal hydronephrosis is transient with little long-term significance, and few children with antenatal hydronephrosis will have significant obstruction, develop symptoms or complications, and require surgery. Some children will be diagnosed with more serious conditions, such as posterior urethral valves. Early detection of obstructive uropathy is necessary to mitigate the potential morbidity from loss of renal function. Imaging is an integral part of screening, diagnosis, and monitoring of children with antenatal hydronephrosis. Optimal timing and appropriate use of imaging can reduce the incidence of late diagnoses and prevent renal scarring and other complications. In general, follow-up neonatal ultrasound is recommended for all cases of antenatal hydronephrosis, while further imaging, including voiding cystourethrography and nuclear scintigraphy, is recommended for moderate or severe cases, or when renal parenchymal or bladder wall abnormalities are suspected. 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® Vomiting in Infants.

Vomiting in infants under the age of 3 months is one of the most common reasons for parents to seek care from their doctor or present to an emergency room. The imaging workup that ensues is dependent on several factors: age at onset, days versus weeks after birth, quality of emesis, bilious or nonbilious vomiting, and the initial findings on plain radiograph, suspected proximal versus distal bowel obstruction. The purpose of these guidelines is to inform the clinician, based on current evidence, what is the next highest yield and most appropriate imaging study to pursue a diagnosis. The goal is rapid and accurate arrival at a plan for treatment, whether surgical or nonsurgical. The following modalities are discussed for each variant of the symptom: plain radiography, fluoroscopic upper gastrointestinal series, fluoroscopic contrast enema, ultrasound of the abdomen, nuclear medicine gastroesophageal reflux scan. 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.

An apparent new syndrome of extreme short stature, microcephaly, dysmorphic faces, intellectual disability, and a bone dysplasia of unknown etiology.

We report on a 26-year-old male with extreme short stature, microcephaly, macroglossia, other dysmorphic features, severe intellectual disability, and a bone dysplasia. The patient had an extensive genetic and biochemical evaluation that was all normal or noninformative. Recently, the proband died following a period of not eating. He likely had a previously undescribed syndrome of unknown etiology.

Accuracy of ultrasound in the diagnosis of classic metaphyseal lesions using radiographs as the gold standard.

BACKGROUND: Diagnosis of classic metaphyseal lesions (CMLs) in children suspected for child abuse can be challenging. Ultrasound (US) can potentially help diagnose CMLs. However, its accuracy is unknown. OBJECTIVE: To evaluate the accuracy of US in the diagnosis of CMLs using skeletal survey reports as the gold standard. MATERIALS AND METHODS: US of the metaphysis was performed in three patient groups age <1 year. Informed consent was obtained for patients scheduled for renal US (Group 1) and for patients scheduled for skeletal surveys for possible child abuse (Group 2). Targeted US was also performed in selected patients to evaluate for possible CML suspected on radiographs (Group 3). In Groups 1 and 2, US was performed of both distal femurs, and of either the right or left proximal and distal tibia. Two radiologists (Rad1 and Rad2) independently reviewed the US studies, blinded to history and other imaging. US sensitivity and specificity were calculated using the following gold standards: CML definitely seen on skeletal survey (positive), CML definitely not seen on skeletal survey or part of renal US group (negative). Cases where the skeletal survey was indeterminate for CML were excluded. Kappa statistics were used to evaluate interobserver variability. RESULTS: Two hundred forty-one metaphyseal sites were evaluated by US in 63 children (mean age: 5 months; 33 males); 34 had skeletal surveys and 29 had renal US. Kappa for the presence of CML was 0.70 with 95.7% agreement. US sensitivity was 55.0% and 63.2% and the specificity was 97.7% and 96.7% for Rad1 and Rad2, respectively. CONCLUSION: US has low sensitivity and high specificity in CML diagnosis. Thus, negative US does not exclude CML, but when the radiographs are equivocal, positive US can help substantiate the diagnosis.

ACR Appropriateness Criteria® Head Trauma-Child.

Head trauma is a frequent indication for cranial imaging in children. The majority of accidental pediatric head trauma is minor and sustained without intracranial injury. Well-validated pediatric-specific clinical decision guidelines should be used to identify very low-risk children who can safely forgo imaging. In those who require acute imaging, CT is considered the first-line imaging modality for suspected intracranial injury because of the short duration of the examination and its high sensitivity for acute hemorrhage. MRI can accurately detect traumatic complications, but often necessitates sedation in children, owing to the examination length and motion sensitivity, which limits rapid assessment. There is a paucity of literature regarding vascular injuries in pediatric blunt head trauma and imaging is typically guided by clinical suspicion. Advanced imaging techniques have the potential to identify changes that are not seen by standard imaging, but data are currently insufficient to support routine clinical use. 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.