ACR Appropriateness Criteria® Urinary Tract Infection-Child: 2023 Update.

Urinary tract infection (UTI) is a frequent infection in childhood. The diagnosis is usually made by history and physical examination and confirmed by urine analysis. Cystitis is infection or inflammation confined to the bladder, whereas pyelonephritis is infection or inflammation of kidneys. Pyelonephritis can cause renal scarring, which is the most severe long-term sequela of UTI and can lead to accelerated nephrosclerosis, leading to hypertension and chronic renal failure. The role of imaging is to guide treatment by identifying patients who are at high risk to develop recurrent UTIs or renal scarring. This document provides initial imaging guidelines for children presenting with first febrile UTI with appropriate response to medical management, atypical or recurrent febrile UTI, and follow-up imaging for children with established vesicoureteral reflux. 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 process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

ACR Appropriateness Criteria® Soft Tissue Vascular Anomalies: Vascular Malformations and Infantile Vascular Tumors (Non-CNS)-Child.

Soft tissue vascular anomalies may be composed of arterial, venous, and/or lymphatic elements, and diagnosed prenatally or later in childhood or adulthood. They are divided into categories of vascular malformations and vascular tumors. Vascular malformations are further divided into low-flow and fast-flow lesions. A low-flow lesion is most common, with a prevalence of 70%. Vascular tumors may behave in a benign, locally aggressive, borderline, or malignant manner. Infantile hemangioma is a vascular tumor that presents in the neonatal period and then regresses. The presence or multiple skin lesions in an infant can signal underlying visceral vascular anomalies, and complex anomalies may be associated with overgrowth syndromes. 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 process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

ACR Appropriateness Criteria® Crohn Disease-Child.

Crohn disease is an inflammatory condition of the gastrointestinal tract with episodes of exacerbation and remission occurring in children, adolescents, and adults. Crohn disease diagnosis and treatment depend upon a combination of clinical, laboratory, endoscopic, histological, and imaging findings. Appropriate use of imaging provides critical information in the settings of diagnosis, assessment of acute symptoms, disease surveillance, and therapy monitoring. Four variants are discussed. The first variant discusses the initial imaging for suspected Crohn disease before established diagnosis. The second variant pertains to appropriateness of imaging modalities during suspected acute exacerbation. The third variant is a substantial discussion of recommendations related to disease surveillance and monitoring of Crohn disease. Finally, panel recommendations and discussion of perianal fistulizing disease imaging completes the document. 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.

Bladder prolapse through a patent urachus presenting as an umbilical mass in the newborn: characteristic prenatal sonographic findings and the diagnostic benefit of postnatal cystography.

Bladder prolapse through a patent urachus is rare. We present a newborn with an unusual exophytic, erythematous umbilical mass. Voiding cystography readily demonstrated continuity of the bladder dome with the umbilical mass, confirming bladder prolapse through a patent urachus. The diagnosis of bladder prolapse was rapidly made in a second newborn with similar imaging and clinical findings and confirmed by cystography. We discuss the embryology of this condition including the association with a vesico-allantoic cyst in utero. Pre- and postnatal images are presented. The use of cystography in diagnosis is emphasized.

Feasibility study of a novel portable digital radiography system modified for fluoroscopy in the neonatal intensive care unit.

BACKGROUND: A portable radiographic system capable of fluoroscopic imaging in the neonatal intensive care unit (NICU) potentially benefits critically ill neonates by eliminating the need to transport them to a fluoroscopy suite. OBJECTIVE: To evaluate whether a portable bedside fluoroscopy system in the NICU can deliver comparable image quality at a similar dose rate to a standard system in a fluoroscopy suite. MATERIALS AND METHODS: In phase A, 20 patients <3 years of age and scheduled to undergo upper gastrointestinal series (upper GI) or voiding cystourethrograms (VCUG) in the radiology fluoroscopy suite were recruited to evaluate a portable fluoroscopic unit. A modified portable radiographic system with a cassette-sized detector and an in-room fluoroscopy system were sequentially used in the same examination. Four radiologists compared the image quality of 20 images from each system using the Radlex score (1-4) for five image quality attributes. The radiation dose rates for the portable and in-suite systems were collected. In phase B, fluoroscopy studies were performed in 5 neonates in the NICU and compared to the 20 previous neonatal studies performed in the department. Clinical workflow, examination time, fluoroscopy time, scattered radiation dose and patient radiation dose were evaluated. RESULTS: In phase A, average dose rates for in-room and portable systems were equivalent, (0.322 mGy/min and 0.320 mGy/min, respectively). Reader-averaged Radlex scores for in-room and portable systems were statistically significantly greater (P<0.05) for all attributes on the portable system except for image contrast. In phase B, scattered radiation from the average fluoroscopy time (26 s) was equivalent to the scattered radiation of 2.6 portable neonatal chest radiographs. Procedure time and diagnostic quality were deemed equivalent. The average dose rate in the NICU with the portable system was 0.21 mGy/min compared to 0.29 mGy/min for the in-room system. CONCLUSION: The portable fluoroscopy unit is capable of providing comparable image quality at equivalent dose levels to an in-room system for neonates with minimal risks to the staff and other patients in the NICU.

Is necrotizing enterocolitis the same disease in term and preterm infants?

INTRODUCTION: Necrotizing enterocolitis predominantly affects preterm (PT) infants. The paucity of data regarding the clinical course in term infants makes it difficult to predict outcomes and counsel families. To identify predisposing factors and gain a better understanding of the clinical course of NEC in term infants, we reviewed our experience with term infants and compared it to outcomes in PT infants. METHODS: We performed a 10 year retrospective review of all infants admitted to our NICU with Bell stage 2 NEC or greater. Infants < 37 weeks gestation were considered PT. Term and PT infant comorbidities, outcomes and intraoperative findings were compared. RESULTS: Fifteen (12%) of 125 infants were term. Compared to PT infants, term infants were more likely to have congenital heart disease (33% term vs. 10% PT, p = 0.02) and develop NEC sooner (4 days in term vs. 17 days in PT, p < 0.001) but were less likely to require operative intervention (20% term vs. 38% PT; p = 0.17). There was no significant difference in Bell stage, survival and development of intestinal failure. NEC totalis occurred exclusively in PT infants. CONCLUSIONS: NEC in term infants has unique clinical features that distinguishes it from NEC in PT infants.

Cytotoxic lesion of the splenium of the corpus callosum in a patient with EVALI.

Since first recognized in July 2019, numerous cases of a lung illness associated with electronic-cigarette use or vaping particularly tetrahydrocannabinol in adolescents and adults have been reported. As of January 2020, the Center for Disease Control has reported over 2500 cases of electronic-cigarette or vaping product use-associated lung injury (EVALI), including 60 deaths. Affected patients most commonly present with respiratory or gastrointestinal complaints although neurological symptoms including headache, confusion and lethargy have been reported. We present a new as yet unpublished finding in the brain of a previously healthy teenage boy with EVALI. Brain imaging may be warranted in this patient population.

Vaping associated lung injury: A potentially life-threatening epidemic in US youth.

BACKGROUND: The Center for Disease Control (CDC) has identified a national outbreak in the United States of over 2600 cases of e-cigarette or vaping product use-associated lung injury (EVALI), including 60 deaths as of January 2020. We describe our experience in six adolescents. MATERIAL AND METHODS: We identified all pediatric patients diagnosed with EVALI by CDC guidelines over a 6-month period at our health system. Clinical presentation, hospital course, and imaging were reviewed. RESULTS: Six patients were identified (three males, three females; median age 18.5 years). Presenting symptoms varied, including constitutional, gastrointestinal, neurologic, and respiratory complaints with pulmonary symptomatology becoming the dominant feature of the illness. Three patients required intensive care unit-level care, one of whom expired 36 days after presentation. Three had bronchoalveolar lavage, two with evidence of lipid-laden macrophages. Four had pulmonary function testing with various results. Admission chest radiographs in all revealed bibasilar interstitial infiltrate which rapidly progressed. Five patients had computed tomography chest imaging demonstrating: confluent pulmonary infiltrates with subpleural sparing (n = 2), generalized ground-glass opacities (n = 1), patchy ground-glass opacities (n = 1) and a reticulonodular pattern (n = 1). Brain magnetic resonance imaging (MRI) obtained in two patients was normal in one and showed a focal signal abnormality in the corpus callosum in one. CONCLUSION: We describe the clinical course and radiologic findings of EVALI in our adolescent patients and present a new finding in the brain not yet described in the literature. Given the diversity of presenting symptoms, a high level of suspicion for EVALI is necessary for patients reporting vaping product use regardless of the presence of pulmonary complaints. Brain MRI should be strongly considered in patients with neurologic symptoms.

Radiation dose monitoring in pediatric fluoroscopy: comparison of fluoroscopy time and dose-area product thresholds for identifying high-exposure cases.

BACKGROUND: Fluoroscopy time has been used as a surrogate for radiation dose monitoring in pediatric fluoroscopy; however it does not account for factors such as magnification or collimation. Dose-area product (DAP) is a more accurate measure of radiation exposure but its dependence on patient weight and body-part thickness is a challenge in children of varying ages. OBJECTIVE: To determine whether fluoroscopy time and DAP produce concurrent results when they are used to identify high-exposure cases, and to establish radiation dose thresholds for our institution. MATERIALS AND METHODS: During a 2-year period we prospectively monitored pediatric fluoroscopy studies performed at the Children's Hospital at Montefiore. We recorded study type, fluoroscopy time, DAP, patient age, weight and height. We then calculated 90th percentile fluoroscopy time and DAP thresholds for weight and age. RESULTS: We evaluated 1,011 cases (453 upper gastrointestinal [UGI] series, 266 voiding cystourethrograms [VCUGs], 120 contrast enemas, 108 speech studies, and 64 esophagrams). Fluoroscopy time demonstrated moderate correlation with DAP (rs=0.45, P<0.001, Spearman rank). DAP strongly correlated with patient weight (rs=0.71, P<0.001) and age (rs=0.70, P<0.001). Concordance of cases exceeding 90th percentile thresholds for fluoroscopy time and DAP were κ=0.27 for UGI series and κ=0.49 for VCUG for weight-based cutoffs, and κ=0.36 for UGI series and κ=0.40 for VCUG for age-based cutoffs. CONCLUSION: The limited correlation of fluoroscopy time with DAP suggests these methods are not equivalent for dose monitoring. However, the strong correlation of DAP with patient weight and age presents a challenge for establishing DAP thresholds in children, who range widely in size. Despite controlling for weight or age, there was limited overlap of cases exceeding the 90th percentile threshold for fluoroscopy time and DAP. This further reinforces the non-overlapping outcome of these two methods and indicates that fluoroscopy time might be inadequate for dose monitoring.

Identification of clinical parameters to increase the diagnostic yield of the non-emergent upper gastrointestinal series in pediatric outpatients.

BACKGROUND: Outpatient, non-emergent upper gastrointestinal (GI) series are frequently requested in children with no surgical history who have nonspecific symptoms such as abdominal pain, failure to thrive and vomiting. The positive yield of an upper GI series in these patients, and, thus, its utility, has not been studied. OBJECTIVES: We evaluated the incidence of positive upper GI findings in children without a history of GI pathology or abdominal surgery in order to identify clinical indications associated with a greater diagnostic yield. MATERIALS AND METHODS: Findings of upper GI series performed between October 2015 and October 2017 in three institutions in children younger than 18 years of age were retrospectively reviewed. The upper GI series protocol for each institution was also reviewed. Children with a medical or surgical GI history, children with insufficient history in the chart and those with an incomplete upper GI series were excluded from the study. Exam indications, patient demographics and clinical history were obtained from the electronic medical records. RESULTS: Of 1,267 children who underwent outpatient upper GI series, 720 (median age: 2 years) had no GI history and were included in the study. The most common indications were non-bilious vomiting (62%), reflux symptoms (28%) and abdominal pain (20%). Upper GI series were normal in 605/720 cases (84%), including 25/26 children with reported bilious emesis. Of the 115 positive studies, 78 (68%) showed only gastroesophageal reflux (GER) (median age: 11 months). Of the remaining 37 studies, 19 demonstrated esophageal findings. One case of malrotation without midgut volvulus was identified in a patient who presented with dysphagia and reflux symptoms. Using a multinomial logistic regression model and adjusting for other variables, reflux symptoms and younger patient age were independent predictors of GER on upper GI series (relative risk ratios of 2.2 and 0.9, respectively). Dysphagia and/or foreign body sensation and older patient age were independent predictors of the presence of esophageal findings (relative risk ratios of 3.3 and 1.1, respectively). CONCLUSION: The yield of routine upper GI series in children with nonspecific symptoms, such as abdominal pain and vomiting, and no surgical history is low. Diagnostic yield was improved in older children and in those complaining of dysphagia and/or foreign body sensation. Routine upper GI series should be avoided in clinically well children with symptoms only of uncomplicated GER and no significant GI history. In children with a history of dysphagia and/or foreign body sensation, an esophagram/barium swallow can suffice.

Methods to improve the noninvasive diagnosis and assessment of disease severity in children with suspected nonalcoholic fatty liver disease (NAFLD): Study design.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is strongly associated with obesity and is the most common liver disease in the developed world. In children with suspected NAFLD, present guidelines suggest consideration of alternative diagnoses via extensive blood testing, though the yield of this work up is unknown. Furthermore, the gold standard diagnostic test for NAFLD remains liver biopsy, making the development of non-invasive tests critically important. OBJECTIVES: Our objectives are: 1) to determine the accuracy of elastography and multiple serum biomarkers - each assessed individually and as algorithms (including those previously tested in adults) - for the diagnosis of nonalcoholic steatohepatitis (NASH) and early fibrosis in children and (2) to examine the utility of extensive testing for rare alternative diagnoses in overweight or obese children with elevated alanine aminotransferase (ALT) suspected to have NAFLD. DESIGN: This is an ongoing, cross-sectional study in children 2-18 years of age with up to 2 years of prospective follow up. Eligible patients are asymptomatic, overweight or obese, and have an ALT ≥35 U/L upon enrollment. Two forms of elastography are obtained serially along with anthropometric data and routine laboratory tests. Elastography and serum biomarkers are also performed immediately prior to any clinically-indicated biopsy. METHODS: Between April 2015 and April 2018, 193 children have been enrolled in this ongoing study and 71 have undergone liver biopsy. Here we carefully report the rationale, methodology, and preliminary data for this study.

Radiation Exposure of Premature Infants Beyond the Perinatal Period.

OBJECTIVES: To determine the odds of premature compared with term infants exceeding the recommended radiation exposure threshold in the first year after discharge from birth hospitalization. METHODS: In this observational retrospective cohort study, we compared the radiation exposure of premature and term infants between 2008 and 2015 in an urban hospital system. The primary outcome was crossing the radiation exposure threshold of 1 millisievert. We assessed prematurity's effect on this outcome with multivariable logistic regression. RESULTS: In our study, 20 049 term and 2047 preterm infants met inclusion criteria. The population was approximately one-half female, predominantly multiracial or people of color (40% African American and 44% multiracial), and of low socioeconomic status. Premature infants had 2.25 times greater odds of crossing the threshold compared with term infants after adjustment for demographics (95% confidence interval [CI]: 1.66-3.05). Adjustment for complex chronic conditions, which are validated metrics of pediatric chronic illness, attenuated this association; however, premature infants still had 1.58 times greater odds of crossing the threshold (95% CI: 1.16-2.15). When the final model was analyzed by degree of prematurity, very preterm and extremely preterm infants were significantly more likely to cross the threshold (1.85 [95% CI: 1.03-3.32] and 2.53 [95% CI: 1.53-4.21], respectively), whereas late preterm infants were not (1.14 [95% CI: 0.73-1.78]). CONCLUSIONS: Premature infants crossed the recommended radiation threshold more often than term infants in the year after discharge from birth hospitalization.

Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.

PURPOSE: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. METHODS: We review the imaging findings in childhood diseases associated with dermatologic manifestations. FINDINGS: Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura). CONCLUSION: Familiarity with pertinent findings in childhood diseases presenting with dermatologic manifestations in childhood diseases aids the radiologist in confirming the diagnosis and guiding imaging workup.

Evaluating an Image Gently and Image Wisely Campaign in a Multihospital Health Care System.

PURPOSE: The efficacy of an Image Gently(®)/Image Wisely(®) radiology departmental campaign consisting of the optimization of CT protocols to reduce dose while maintaining quality, and an educational effort to alter the ordering patterns of referring physicians at a multihospital academic center, was evaluated. METHODS: The numbers of CT, MR, and ultrasound studies performed at inpatient, outpatient, and emergency facilities in the hospital system before and after the initiation of the departmental campaign (2010) were obtained for a 10-year period (2004-2014) using a radiology information system. For the same time period, dose per scan (volumetric CT dose index) was obtained through the Dose Index Registry(®)/National Radiology Data Registry for frequently performed studies. Descriptive statistics were used to analyze temporal trends in radiation dose and utilization across differing age groups: <20, 20 to 39, and 40 to 59 years. RESULTS: The radiology information system yielded 865,879 imaging examinations and 4,508,030 patients. Although patient and imaging volume grew annually over the study period (by 6.8% and 4.9%, respectively), CT utilization as a percentage of total imaging decreased, compensated for by an increase in ultrasound use. This was most marked in the youngest age group. MR use as a percentage of total imaging was unchanged. The median volumetric CT dose index for each study protocol was reduced or stabilized. CONCLUSIONS: The campaign resulted in a reduction in CT utilization, a reduction in radiation dose per study, and a compensatory rise in ultrasound use. An interactive aggressive educational campaign directed toward referring providers combined with protocol dose reduction efforts can be successful in reducing patient exposure from medical radiation.

Yield of diagnostic tests in obese children with an elevated alanine aminotransferase.

AIM: Nonalcoholic fatty liver disease (NAFLD) is associated with obesity and affects roughly 10% of children. However, NAFLD is often diagnosed by exclusion - that is, obese children with an elevated alanine aminotransferase (ALT) are screened for other liver diseases in the absence of a biopsy. This testing is nonstandardized, and professional society recommendations differ. This study examines the yield of testing for disorders other than NAFLD in this patient population. METHODS: A retrospective study was performed in 120 obese, asymptomatic, noncholestatic children with an ALT ≥40 U/L and additional diagnostic testing. RESULTS: No patients were found to have Wilson's, hepatitis A, hepatitis B, hepatitis C, cytomegalovirus, alpha-1 antitrypsin deficiency, autoimmune hepatitis, celiac disease or Epstein-Barr virus. Only one patient (1/120) was identified with definite disease other than NAFLD, which was muscular dystrophy. The positive predictive value of a screening test was 5%, and the specificity was 97%. Of 70 children with an abdominal ultrasound, no significant abnormalities were identified. CONCLUSION: Extensive testing in asymptomatic, noncholestatic, obese children with an elevated ALT may be of limited diagnostic value and false-positive tests are likely. Large, prospective studies are needed to help focus the work up in this patient population.

Ovary preservation in the treatment of childhood Meigs syndrome.

Meigs syndrome, the combination of benign ovarian tumor, ascites, and pleural effusion, is present in a small percentage of ovarian fibromas and is infrequently reported in children. When associated with elevated CA-125 suspicion is raised for malignancy, often prompting aggressive surgical intervention. We present a case of childhood Meigs syndrome and review the relevant literature with emphasis on ovary preservation. Out of nine identified pediatric cases, one involved ovary sparing treatment and none recurred or progressed to malignancy. Our report highlights the importance of presurgical identification of Meigs syndrome in order to curtail salpingo-oophorectomy when feasible.

Adrenocortical carcinoma with extension to the inferior vena cava and right atrium: 20-month-old girl with TP53 mutation.

A 20-month-old female presented with respiratory distress and a right adrenal mass extending into the inferior vena cava and right atrium. The mass was initially thought to be neuroblastoma. Pathology later revealed adrenocortical carcinoma. Inferior vena cava extension is far more common in adrenocortical carcinoma than neuroblastoma, and its presence should prompt clinical and laboratory evaluation for an adrenocortical tumor. The genetic findings in TP53 associated with this disease are discussed.

Shwachman-Bodian-Diamond syndrome: metaphyseal chondrodysplasia in children with pancreatic insufficiency and neutropenia.

Shwachman-Bodian-Diamond syndrome (OMIM 260400) was identified in 1964 by pediatricians Harry Shwachman, a leader in cystic fibrosis, and Louis K. Diamond, a hematologist, along with pediatrician and morbid anatomist Martin Bodian. Initially the syndrome's clinical presentation included exocrine pancreatic insufficiency (lipomatous replacement of the pancreas) and neutropenia. In 1967 skeletal changes of metaphyseal chondrodysplasia were also described, completing the triad of findings; these abnormalities are present in all affected children and should be viewed as an integral feature of the syndrome, also called Shwachman-Diamond syndrome.

Bedside upper gastrointestinal series in critically ill low birth weight infants.

BACKGROUND: The upper gastrointestinal (UGI) series is the preferred method for the diagnosis of malrotation. A bedside UGI technique was developed at our institution for use in low birth weight, critically ill neonates to minimize the risks of transportation from the neonatal intensive care unit (NICU) such as hypothermia and dislodgement of support lines and tubes. OBJECTIVE: To determine the ability of a bedside UGI technique to identify the position of the duodenojejunal junction (DJJ) in low birth weight, critically ill infants in the NICU. MATERIALS AND METHODS: We retrospectively reviewed bedside UGI examinations performed in premature infants weighing less than 1,500 g from 2008 to 2013 and correlated the findings with clinical data, imaging studies and surgical findings. RESULTS: Of 27 patients identified (weight range: 633-1,495 g), 21 (78%) bedside UGI series were diagnostic. Twenty of 27 cases (74%) demonstrated normal intestinal rotation. One case demonstrated malrotation with midgut volvulus, which was confirmed at surgery. In six cases (22%), the position of the DJJ could not be accurately determined. No cases of malrotation with midgut volvulus were missed. None of the patients with normal bedside UGI studies was found to have malrotation based on clinical follow-up (mean: 20 months), surgical findings or further imaging. CONCLUSION: The bedside UGI is a useful technique to exclude malrotation in critically ill neonates and minimizes potential risks of transportation to the radiology suite. Pitfalls that may preclude a diagnostic examination include incorrect timing of radiographs, patient rotation, suboptimal enteric tube position and bowel distention. In cases of diagnostic uncertainty, a follow-up study should be performed.