Publications by authors named "Judy H Squires"

25 Publications

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Contrast-enhanced ultrasound of benign and malignant liver lesions in children.

Pediatr Radiol 2021 May 12. Epub 2021 May 12.

Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA.

Contrast-enhanced ultrasound (CEUS) is increasingly being used in children. One of the most common referrals for CEUS performance is characterization of indeterminate focal liver lesions and follow-up of known liver lesions. In this setting, CEUS is performed with intravenous administration of ultrasound contrast agents (UCAs). When injected into a vein, UCA microbubbles remain confined within the vascular network until they dissipate. Therefore, visualization of UCA within the tissues and lesions corresponds to true blood flow. CEUS enables continuous, real-time observation of the enhancement pattern of a focal liver lesion, allowing in most cases for a definite diagnosis and obviating the need for further cross-sectional imaging or other interventional procedures. The recent approval of Lumason (Bracco Diagnostics, Monroe Township, NJ) for pediatric liver CEUS applications has spurred the widespread use of CEUS. In this review article we describe the role of CEUS in pediatric liver applications, focusing on the examination technique and interpretation of main imaging findings of the most commonly encountered benign and malignant focal liver lesions. We also compare the diagnostic performance of CEUS with other imaging modalities for accurate characterization of focal liver lesions.
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http://dx.doi.org/10.1007/s00247-021-04976-2DOI Listing
May 2021

Contrast-enhanced ultrasound of pediatric lungs.

Pediatr Radiol 2021 May 12. Epub 2021 May 12.

Department of Radiology, Boston Children's Hospital, Harvard University, Boston, MA, USA.

In addition to radiography, ultrasound (US) has long proved to be a valuable imaging modality to evaluate the pediatric lung and pleural cavity. Its many inherent advantages, including real-time performance, high spatial resolution, lack of ionizing radiation and lack of need for sedation make it preferable over other imaging modalities such as CT. Since the introduction of ultrasound contrast agents (UCAs), contrast-enhanced ultrasound (CEUS) has become a valuable complementary US technique, with many well-established uses in adults and evolving uses in children. Lung CEUS applications are still not licensed and are performed off-label, although the added value of CEUS in certain clinical scenarios is increasingly reported. The limited evidence of CEUS in the evaluation of pediatric lungs focuses primarily on community-acquired pneumonia and its complications. In this clinical setting, CEUS is used to confidently and accurately diagnose necrotizing pneumonia and to delineate pleural effusions and empyema. In addition to intravenous use, UCAs can be administered directly into the pleural cavity through chest catheters to improve visualization of loculations within a complex pleural effusion, which might necessitate fibrinolytic therapy. The purpose of this paper is to present the current experience on pediatric lung CEUS and to suggest potential additional uses that can be derived from adult studies.
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http://dx.doi.org/10.1007/s00247-020-04914-8DOI Listing
May 2021

Contrast-Enhanced Ultrasound in Children: Implementation and Key Diagnostic Applications.

AJR Am J Roentgenol 2021 Apr 28. Epub 2021 Apr 28.

Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN.

Contrast-enhanced ultrasound (CEUS) utilization is expanding rapidly, particularly in children, in whom the modality offers important advantages of dynamic evaluation of the vasculature, portability, lack of ionizing radiation, and lack of need for sedation. Accumulating data establish an excellent safety profile of ultrasound contrast agents in children. Although only FDA-approved for IV use in children for characterizing focal liver lesions and for use during echocardiography, growing off-label applications are expanding the diagnostic potential of ultrasound. Focal liver lesion evaluation is the most common use of CEUS, and the American College of Radiology Pediatric LI-RADS Working Group recommends including CEUS for evaluation of a newly discovered focal liver lesion in many circumstances. Data also support the role of CEUS in hemodynamically stable children with blunt abdominal trauma, and CEUS is becoming a potential alternative to CT in this setting. Additional potential applications that require further study include evaluation of pathology in the lung, spleen, brain, pancreas, bowel, kidney, female pelvis, and scrotum. This review explores the implementation of CEUS in children, describing basic principles of ultrasound contrast agents and CEUS technique and summarizing current and potential IV diagnostic applications based on pediatric-specific supporting evidence.
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http://dx.doi.org/10.2214/AJR.21.25713DOI Listing
April 2021

Ultrasound Evaluation of Gastric Emptying Time in Healthy Term Neonates after Formula Feeding.

Anesthesiology 2021 Jun;134(6):845-851

Background: The current American Society of Anesthesiologists fasting guideline for formula-fed infants in the periprocedural setting is 6 h. Prolonged fasting in very young infants is associated with an increased risk for hypoglycemia and dehydration as well as patient discomfort and patient/parental dissatisfaction. This study aimed to determine the time to gastric emptying in healthy neonates after formula feeding by serially evaluating the gastric antrum with ultrasound. The authors hypothesized that gastric emptying times in formula-fed neonates are significantly shorter than the current 6 h fasting recommendation.

Methods: After institutional review board approval and written informed parental consent, ultrasound examination was performed in healthy full-term neonates before and after formula feeding at 15-min intervals until return to baseline. Ultrasound images of the gastric antrum were measured to obtain cross-sectional areas, which were then used to estimate gastric antral volumes.

Results: Forty-six of 48 recruited neonates were included in the final analysis. Gastric emptying times ranged from 45 to 150 min and averaged 92.9 min (95% CI, 80.2 to 105.7 min; 99% CI, 76.0 to 109.8 min) in the overall study group. No significant differences were found in times to gastric emptying between male and female neonates (male: mean, 93.3 [95% CI, 82.4 to 104.2 min]; female: mean, 92.6 [95% CI, 82.0 to 103.2 min]; P = 0.930) or those delivered by vaginal versus cesarean routes (vaginal: mean, 93.9 [95% CI, 81.7 to 106.1 min]; cesarean: mean, 92.2 [95% CI, 82.5 to 101.9 min]; P = 0.819).

Conclusions: These results demonstrate that gastric emptying times are substantially less than the current fasting guideline of 6 h for formula-fed, healthy term neonates.

Editor’s Perspective:
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http://dx.doi.org/10.1097/ALN.0000000000003773DOI Listing
June 2021

Magnetic resonance imaging of pediatric liver tumors.

Pediatr Radiol 2021 Apr 14. Epub 2021 Apr 14.

Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Liver tumors in children can be benign or malignant. Although several clinical factors are important in the evaluation of these lesions, MRI is particularly important for lesion characterization and tumor staging. In children, use of a hepatobiliary contrast agent is recommended to evaluate a known or suspected liver lesion. In this review, we discuss the most common benign and malignant pediatric liver tumors, including vascular tumors, mesenchymal hamartoma, focal nodular hyperplasia, hepatocellular adenoma, hepatoblastoma, hepatocellular carcinoma, fibrolamellar hepatocellular carcinoma, undifferentiated embryonal sarcoma and metastases, with emphasis on relevant clinical information and imaging appearance at MRI using hepatobiliary agents.
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http://dx.doi.org/10.1007/s00247-021-05058-zDOI Listing
April 2021

Contrast-enhanced ultrasound of the small organs in children.

Pediatr Radiol 2021 Apr 8. Epub 2021 Apr 8.

Department of Radiology, King's College Hospital, Denmark Hill, London, UK.

In pediatric and adult populations, intravenous contrast-enhanced ultrasound (CEUS) remains off-label for imaging of organs other than the liver and heart. This limited scope inhibits potential benefits of the new modality from a more widespread utilization. Yet, CEUS is potentially useful for imaging small organs such as the thyroid gland, lymph nodes, testes, ovaries and uterus, with all having locations and vasculature favorable for this type of examination. In the adult population, the utility of CEUS has been demonstrated in a growing number of studies for the evaluation of these small organs. The aim of this article is to present a review of pediatric CEUS of the thyroid gland, lymph nodes, testes, ovaries and uterus as well as to draw from the adult literature indications for possible applications in children.
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http://dx.doi.org/10.1007/s00247-021-05006-xDOI Listing
April 2021

Current State of Imaging of Pediatric Pancreatitis: Expert Panel Narrative Review.

AJR Am J Roentgenol 2021 Mar 17. Epub 2021 Mar 17.

Department of Radiology, The Children's Hospital of Philadelphia.

Pancreatitis is as common in children as it is in adults, though causes and accepted imaging strategies differ in children. In this narrative review we discuss the epidemiology of childhood pancreatitis and key imaging features for pediatric acute, acute recurrent, and chronic pancreatitis. We rely heavily on our collective experience in discussing advantages and disadvantages of different imaging modalities; practical tips for optimization of ultrasound, CT, and MRI with MRCP in children; and image interpretation pearls. Challenges and considerations unique to imaging of pediatric pancreatitis are discussed, including timing of imaging, role of secretin-enhanced MRCP, utility of emergent MRI, severity prediction, autoimmune pancreatitis, and best methods for serial imaging. We suggest a methodical approach to pancreatic MRI interpretation in children, including a sample structured report, and we provide consensus statements based on our experience imaging children with pancreatitis.
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http://dx.doi.org/10.2214/AJR.21.25508DOI Listing
March 2021

Diagnostic Performance of LI-RADS Version 2018 for Evaluation of Pediatric Hepatocellular Carcinoma.

Radiology 2021 Apr 23;299(1):190-199. Epub 2021 Feb 23.

From the Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S Kingshighway Blvd, Campus Box 8131-MIR, St Louis, MO 63110 (G.K., T.J.F.); Department of Diagnostic Imaging, The Hospital for Sick Children and Department of Medical Imaging, University of Toronto, Toronto, Canada (G.B.C.); Department of Radiology, University of Texas Southwestern Medical Center, Dallas, Tex (G.R.S.); Department of Radiology, Emory University and Children's Healthcare of Atlanta, Atlanta, Ga (A.L.A.); Department of Radiology, University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, Pa (J.H.S.); Division of Biostatistics, Washington University School of Medicine, St Louis, Mo (A.S.); Nemours Children's Hospital, Nemours Children's Health System, University of Central Florida College of Medicine, Orlando, Fla (D.J.P.); and Department of Radiology, Cincinnati Children's Hospital, and Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, Ohio (A.J.T.).

Background The Liver Imaging Reporting and Data System (LI-RADS) has standardized the evaluation of adult but not pediatric hepatocellular carcinoma (HCC). Purpose To evaluate the performance of LI-RADS version 2018 for diagnosis of pediatric HCC. Materials and Methods This multi-institution retrospective study evaluated all available dynamic CT and/or MRI scans of pediatric (≤18 years) HCC from five institutions between July 2009 and April 2019. The control group included an equal number of other enhancing hepatic lesions. Blinded to final diagnosis, three radiologists independently applied LI-RADS version 2018 criteria. The reference standard was pathologic examination or more than 1 year follow-up. Sensitivity and specificity of LI-RADS were computed using a dichotomous classification of LR-1, LR-2, or LR-3 versus LR-4, LR-5, LR-TIV (tumor in vein), or LR-M (probably or definitely malignant but not HCC-specific) for predicting hepatic malignancy in the entire cohort and in patients at risk for HCC. Results The cohort consisted of 116 children: 58 with HCC (mean age, 12 years ± 5; 31 girls) and 58 with other enhancing hepatic masses (mean age, 12 years ± 5; 42 girls). Frequencies of major criteria in classic HCC for the three readers were as follows: nonrim arterial phase hyperenhancement, 49%-62% (19-24 of 39 patients); nonperipheral "washout," 36%-59% (14-23 of 39 patients); and enhancing "capsule," 28%-38% (11-15 of 39 patients). For the full cohort, the sensitivity of LR-4, LR-5, LR-TIV, or LR-M for malignancy among the three readers ranged from 85% (95% CI: 76, 94) to 88% (95% CI: 80, 96); specificity of LR-1, LR-2, or LR-3 for benignity ranged from 54% (95% CI: 40, 68) to 70% (95% CI: 57, 83). In the at-risk subgroup, sensitivity ranged from 58% (95% CI: 36, 80) to 68% (95% CI: 48, 89); specificity ranged from 56% (95% CI: 37, 74) to 63% (95% CI: 45, 81). All lesions categorized as LR-TIV ( = 10-13) were HCCs. Conclusion Liver Imaging Reporting and Data System version 2018 had moderate sensitivity but low specificity for the diagnosis of pediatric hepatocellular carcinoma (HCC), which had low frequencies of the major criteria used for adult HCC diagnosis. © RSNA, 2021 See also the editorial by Paltiel in this issue.
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http://dx.doi.org/10.1148/radiol.2021203559DOI Listing
April 2021

50 Years Ago in TheJournalofPediatrics: The Unraveling of Hepatic Radioresistance and the Rise of Radiation-Induced Liver Disease.

J Pediatr 2021 01;228:43

Division of Pediatric Radiology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.

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http://dx.doi.org/10.1016/j.jpeds.2020.07.073DOI Listing
January 2021

ACR Appropriateness Criteria® Vomiting in Infants.

J Am Coll Radiol 2020 Nov;17(11S):S505-S515

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

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.
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http://dx.doi.org/10.1016/j.jacr.2020.09.002DOI Listing
November 2020

North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and the Society for Pediatric Radiology Joint Position Paper on Noninvasive Imaging of Pediatric Pancreatitis: Literature Summary and Recommendations.

J Pediatr Gastroenterol Nutr 2021 01;72(1):151-167

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH.

Abstract: The reported incidence of pediatric pancreatitis is increasing. Noninvasive imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), play important roles in the diagnosis, staging, follow-up, and management of pancreatitis in children. In this position paper, generated by members of the Pancreas Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the Abdominal Imaging Committee of The Society for Pediatric Radiology (SPR), we review the roles of noninvasive imaging in pediatric acute, acute recurrent, and chronic pancreatitis. We discuss available evidence related to noninvasive imaging, highlighting evidence specific to pediatric populations, and we make joint recommendations for use of noninvasive imaging. Further, we highlight the need for research to define the performance and role of noninvasive imaging in pediatric pancreatitis.
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http://dx.doi.org/10.1097/MPG.0000000000002964DOI Listing
January 2021

Pediatric Hepatoblastoma, Hepatocellular Carcinoma, and Other Hepatic Neoplasms: Consensus Imaging Recommendations from American College of Radiology Pediatric Liver Reporting and Data System (LI-RADS) Working Group.

Radiology 2020 09 30;296(3):493-497. Epub 2020 Jun 30.

From the Department of Radiology, University of Texas Southwestern Medical Center, Dallas, Tex (G.R.S.); Department of Radiology, University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, Pa (J.H.S.); Department of Radiology, Emory University and Children's Healthcare of Atlanta, Atlanta, Ga (A.A.); Department of Diagnostic Imaging, The Hospital for Sick Children and Department of Medical Imaging, University of Toronto, Toronto, Canada (G.B.C.); Department of Radiology, Montefiore Medical Center, Bronx, NY (V.C.); Department of Radiology, Duke University Medical Center, Durham, NC (J.T.D.); Department of Radiology, Mallinckrodt Institute of Radiology, St. Louis Children's Hospital, Washington University School of Medicine, St Louis, Mo (G.K.); Department of Radiology, Nationwide Children's Hospital, Columbus, Ohio (R.K.); Stanford University School of Medicine, Lucile Packard Children's Hospital, Stanford, Calif (M.P.L.); Department of Radiology, Texas Children's Hospital, Houston, Tex (P.M.M.); Nemours Children's Hospital, Nemours Children's Health System, University of Central Florida College of Medicine, Orlando, Fla (D.J.P.); Liver Imaging Group, Department of Radiology, University of California San Diego, San Diego, Calif (C.B.S.); Department of Radiology, Cincinnati Children's Hospital, Cincinnati, Ohio (A.J.T.); and Department of Radiology, University of Cincinnati College of Medicine, 3333 Burnet Ave, MLC 5031, Cincinnati, OH 45229 (A.J.T.).

Appropriate imaging is imperative in evaluating children with a primary hepatic malignancy such as hepatoblastoma or hepatocellular carcinoma. For use in the adult patient population, the American College of Radiology created the Liver Imaging Reporting and Data System (LI-RADS) to provide consistent terminology and to improve imaging interpretation. At present, no similar consensus exists to guide imaging and interpretation of pediatric patients at risk for developing a liver neoplasm or how best to evaluate a pediatric patient with a known liver neoplasm. Therefore, a new Pediatric Working Group within American College of Radiology LI-RADS was created to provide consensus for imaging recommendations and interpretation of pediatric liver neoplasms. The article was drafted based on the most up-to-date existing information as interpreted by imaging experts comprising the Pediatric LI-RADS Working Group. Guidance is provided regarding appropriate imaging modalities and protocols, as well as imaging interpretation and reporting, with the goals to improve imaging quality, to decrease image interpretation errors, to enhance communication with referrers, and to advance patient care. An expanded version of this document that includes broader background information on pediatric hepatocellular carcinoma and rationale for recommendations can be found in Appendix E1 (online).
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http://dx.doi.org/10.1148/radiol.2020200751DOI Listing
September 2020

Contrast-Enhanced Ultrasound of Congenital and Infantile Hemangiomas: Preliminary Results From a Case Series.

AJR Am J Roentgenol 2020 03 22;214(3):658-664. Epub 2020 Jan 22.

Department of Pediatric Radiology, UPMC Children's Hospital of Pittsburgh, 4401 Penn Ave, 2nd Fl Radiology, Pittsburgh, PA 15224.

The contrast-enhanced ultrasound (CEUS) imaging features of hepatic vascular tumors in infants, including infantile hemangioma (IH) and congenital hemangioma (CH), are not well reported. Frequent inaccurate use of lesion terminology in the literature has created diagnostic confusion. The purpose of this study is to describe the CEUS features of IH and CH. Ten patients, ranging in age from 8 days to 16 months, with hepatic vascular tumors were included for retrospective analysis. Gray-scale ultrasound, color Doppler ultrasound, and CEUS features were reviewed, and interobserver kappa coefficients were calculated. Final diagnoses were clinically determined by a pediatrician with expertise in vascular anomalies except in one patient who underwent surgical excision. Of the 10 patients, five had CHs and five had IHs. All 10 lesions were hyperenhancing in the early arterial phase. In the portal venous phase, four of five (80%) CHs showed hyperenhancement relative to normal liver parenchyma, whereas four of five (80%) IHs showed isoenhancement. In the late phase, washout of contrast material was seen in three of five (60%) IHs, whereas one IH remained isoenhancing and one IH was hyperenhancing. None of the CHs showed late washout. Interobserver kappa coefficients for CEUS features ranged from 0.60 to 1.00. Except for the CEUS feature portal venous phase enhancement (κ = 0.60), good to excellent (κ = 0.74-1.00) agreement about CEUS features of IHs and CHs was observed. A significant proportion of IHs (60%) showed washout at delayed phase imaging, which has also been reported with malignancies. Recognition of the overlap in imaging appearance of these two entities is vital to preventing misdiagnosis of malignancy.
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http://dx.doi.org/10.2214/AJR.19.22174DOI Listing
March 2020

Feasibility and reproducibility of shear wave elastography in pediatric cranial ultrasound.

Pediatr Radiol 2020 06 20;50(7):990-996. Epub 2019 Dec 20.

Department of Radiology, Children's Hospital of Pittsburgh, 4401 Penn Ave., 2nd Floor Radiology, Pittsburgh, PA, 15224, USA.

Background: Head ultrasound (US) is commonly used to evaluate the neonatal brain but may be limited by its lack of sensitivity and specificity. Ultrasound shear wave elastography (SWE) might provide additional information to conventional gray-scale imaging.

Objective: To assess whether SWE of brain parenchyma can be (1) successfully performed at a large academic medical center where US technologists perform the majority of examinations and (2) used to detect intracranial pathology.

Materials And Methods: Pediatric patients undergoing head ultrasound underwent simultaneous SWE examination. We included normal examinations (n=70) and those with intracranial pathology (n=8) for analysis. We analyzed inter-reader variability and examination success rates and assessed the stiffness of white matter and deep gray nuclei in normal and pathological states across multiple gestational age groups.

Results: Average gestational age of the term, pre-term and extreme pre-term groups were 38.4±1.2 weeks, 29.0±3.7 weeks and 28.3±3.1 weeks, respectively. Overall examination success rate was 79.5%. We observed a decrease in the SWE examination time from the first month (5.9±3.7 min) to the second month (4.1±1.7 min; P=0.01). Forty-one repeat examinations were performed on 14 children by different technologists, with an intraclass correlation coefficient (ICC) of 0.91. Mean stiffness in the periventricular white matter was lower than in the deep gray nuclei in all gestational age groups: term group (1.3 m/s vs. 1.5 m/s, P<0.001), pre-term (1.3 m/s vs. 1.4 m/s P=0.12), and extremely preterm group (1.2 m/s vs. 1.4 m/s, P=0.001). Mean stiffness for the deep gray nuclei differed between the term (1.5±0.3 m/s) and pre-term (1.4±0.2 m/s) groups (P<0.01). No significant differences in white matter stiffness were seen in relation to gestational age. Infants with large intraparenchymal hemorrhage had increased white matter stiffness (1.3±0.1 m/s) and deep gray nuclei stiffness (1.6±0.2 m/s) compared to full-term infants with normal head ultrasounds. These differences approached statistical significance with P=0.09 and P=0.06, respectively.

Conclusion: We demonstrated that SWE performed by pediatric sonography technologists is reproducible. We found differences in stiffness between deep gray nuclei and periventricular white matter across multiple age groups.
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http://dx.doi.org/10.1007/s00247-019-04592-1DOI Listing
June 2020

Contrast-enhanced ultrasound of liver lesions in children.

Pediatr Radiol 2019 10 16;49(11):1422-1432. Epub 2019 Oct 16.

Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Initial pediatric imaging of the liver heavily relies on ultrasonography (US) because it is free of ionizing radiation, easily portable and readily available. Although conventional US (gray-scale and color Doppler) is often an excellent screening tool, its relative low specificity compared to CT/MRI limits liver lesion characterization. The United States Food and Drug Administration's recent approval of an intravenous US contrast agent for pediatric liver lesion characterization (sulfur hexafluoride lipid-type A microspheres) and its excellent safety profile have spurred increased interest in contrast-enhanced US for definitive diagnosis of pediatric liver lesions. This review focuses on the safety of contrast-enhanced US, role of contrast-enhanced US in the evaluation of focal liver lesions, basic contrast-enhanced US technique for liver imaging, and interpretation principles. The authors review common focal liver lesions, with special attention to the role of contrast-enhanced US in the pediatric oncology population.
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http://dx.doi.org/10.1007/s00247-019-04361-0DOI Listing
October 2019

Expanding etiology of progressive familial intrahepatic cholestasis.

World J Hepatol 2019 May;11(5):450-463

Division of Gastroenterology, Hepatology, and Nutrition, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, United States.

Background: Progressive familial intrahepatic cholestasis (PFIC) refers to a disparate group of autosomal recessive disorders that are linked by the inability to appropriately form and excrete bile from hepatocytes, resulting in a hepatocellular form of cholestasis. While the diagnosis of such disorders had historically been based on pattern recognition of unremitting cholestasis without other identified molecular or anatomic cause, recent scientific advancements have uncovered multiple specific responsible proteins. The variety of identified defects has resulted in an ever-broadening phenotypic spectrum, ranging from traditional benign recurrent jaundice to progressive cholestasis and end-stage liver disease.

Aim: To review current data on defects in bile acid homeostasis, explore the expanding knowledge base of genetic based diseases in this field, and report disease characteristics and management.

Methods: We conducted a systemic review according to PRISMA guidelines. We performed a Medline/PubMed search in February-March 2019 for relevant articles relating to the understanding, diagnosis, and management of bile acid homeostasis with a focus on the family of diseases collectively known as PFIC. English only articles were accessed in full. The manual search included references of retrieved articles. We extracted data on disease characteristics, associations with other diseases, and treatment. Data was summarized and presented in text, figure, and table format.

Results: Genetic-based liver disease resulting in the inability to properly form and secrete bile constitute an important cause of morbidity and mortality in children and increasingly in adults. A growing number of PFIC have been described based on an expanded understanding of biliary transport mechanism defects and the development of a common phenotype.

Conclusion: We present a summary of current advances made in a number of areas relevant to both the classically described FIC1 (), BSEP (), and MDR3 () transporter deficiencies, as well as more recently described gene mutations -- TJP2 (), FXR (), MYO5B (), and others which expand the etiology and understanding of PFIC-related cholestatic diseases and bile transport.
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http://dx.doi.org/10.4254/wjh.v11.i5.450DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6547292PMC
May 2019

Pediatric ovarian volumes measured at ultrasound after contralateral unilateral oophorectomy.

Pediatr Radiol 2019 05 17;49(5):632-637. Epub 2019 Jan 17.

Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Background: Changes that occur in the remaining ovary after contralateral oophorectomy are not well described.

Objective: To determine average ovarian volume in pediatric patients after contralateral oophorectomy compared to age-matched controls with two normal ovaries.

Materials And Methods: We performed a retrospective review of ultrasound examinations and electronic medical records of patients ages 0-18 years who had unilateral oophorectomy from 2000 to 2017 (n=64). We used 384 consecutive normal age-matched ovaries for comparison, analyzing mean ovarian volumes.

Results: Higher mean ovarian volume (mL) was observed in patients who had oophorectomy compared to controls in the first decade of life (P<0.003) and second decade of life (P<0.0003). Higher mean ovarian volume was seen in both premenarchal and menstruating patients with prior oophorectomy when compared to controls (P<0.05 and P<0.0001, respectively). When comparing volume during menstrual cycle, we saw higher mean ovarian volumes in the oophorectomy group compared to the control group for the follicular (P<0.0001), pre-ovulatory (P=0.0005) and luteal phases (P<0.0003). We provide an updated reference of normal ovarian volumes for pediatric patients, with values similar to those already reported in the literature.

Conclusion: Ovarian volume is higher in pediatric patients with one normal ovary following contralateral oophorectomy. The provided normative volumes can be used in evaluating these patients.
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http://dx.doi.org/10.1007/s00247-018-04336-7DOI Listing
May 2019

Comparison of Traditional and Web-Based Medical Student Teaching by Radiology Residents.

J Am Coll Radiol 2019 Apr 16;16(4 Pt A):492-495. Epub 2018 Nov 16.

Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; Department of Radiology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.

Purpose: Web-based learning tools are increasingly available for use and have been described in the pedagogical literature. However, rigorous comparisons between traditional learning methods and newer collaborative online tools have not been performed. Herein, we describe a web-based curriculum hosted on the collaborative Radiopaedia.org website. This curriculum was compared with traditional learning tools in a randomized, controlled fashion.

Materials And Methods: Medical students rotating through inpatient pediatrics were offered a 1-hour case-based learning session led by radiology residents. Students were randomized to receive online (Radiopaedia.org) versus traditional supplemental educational materials (reading material covering the same content) for review before the in-class session. A 15-point questionnaire was administered at two different points during the rotation: at the beginning of the clinical rotation and at the end of the classroom session.

Results: Fifty-eight students were approached for enrollment and a total of 47 (81%) consented to participation and completed the study requirements. Students who completed the web-based module had higher mean knowledge scores (74%) compared with those who were provided the traditional learning material (68%) (P = .06). Specifically, they demonstrated increased knowledge of the ACR Appropriateness Criteria and the "silhouette sign."

Conclusions: A randomized, controlled, nonblinded evaluation of a novel radiology curriculum intervention hosted on Radiopaedia.org demonstrates improved test scores compared with traditional teaching methods.
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http://dx.doi.org/10.1016/j.jacr.2018.09.048DOI Listing
April 2019

Ultrasound Elastography Applications in Pediatrics.

Ultrasound Q 2018 Dec;34(4):199-205

Children's Mercy Hospital, Kansas City, MO.

Ultrasound is one of the most important imaging modalities in pediatric imaging because of its accessibility, portability, lack of ionizing radiation, and ability to generally perform examinations without need for sedation. Ultrasound elastography can measure the stiffness of various tissues. This review article aims to discuss how ultrasound elastography has performed in evaluating multiple pathologies in the pediatric population. The best studied pediatric applications are in liver diseases such as fibrosis, biliary atresia, and hepatic vascular congestion. Measuring muscle stiffness in cerebral palsy is the most promising pediatric musculoskeletal application, but many other applications are in the early stages of research. Other applications in pediatric small organ imaging have been explored and still need more study before gaining clinical relevance.
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http://dx.doi.org/10.1097/RUQ.0000000000000379DOI Listing
December 2018

Contrast-Enhanced Ultrasonography During Extracorporeal Membrane Oxygenation.

J Ultrasound Med 2019 Feb 29;38(2):545-548. Epub 2018 Jul 29.

Department of Cardiothoracic Surgery, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA.

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http://dx.doi.org/10.1002/jum.14710DOI Listing
February 2019

Hepatic Vascular Tumors in the Neonate: Angiosarcoma.

J Pediatr 2018 02 1;193:245-248.e1. Epub 2017 Dec 1.

Division of Newborn Medicine, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA.

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http://dx.doi.org/10.1016/j.jpeds.2017.10.015DOI Listing
February 2018

Canal of Nuck hernia: a multimodality imaging review.

Pediatr Radiol 2017 Jul 25;47(8):893-898. Epub 2017 Apr 25.

Department of Radiology, University of Pittsburgh Medical Center, 200 Lothrop St., Pittsburgh, PA, 15213, USA.

Canal of Nuck abnormalities are a rare but important cause of morbidity in girls, most often those younger than 5 years of age. The canal of Nuck, which is the female equivalent of the male processus vaginalis, is a protrusion of parietal peritoneum that extends through the inguinal canal and terminates in the labia majora. The canal typically obliterates early in life, but in some cases the canal can partially or completely fail to close, potentially resulting in a hydrocele or hernia of pelvic contents. Recognition of this entity is especially important in cases of ovarian hernia due to the risk of incarceration and torsion. We aim to increase awareness of this condition by reviewing the embryology, anatomy and diagnosis of canal of Nuck disorders with imaging findings on US, CT and MRI using several cases from a single institution.
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http://dx.doi.org/10.1007/s00247-017-3853-6DOI Listing
July 2017

Identification of Suspected Hepatocellular Carcinoma with Contrast-Enhanced Ultrasound.

J Pediatr 2017 03 4;182:398-398.e1. Epub 2017 Jan 4.

Department of Hepatology Children's Hospital of Pittsburgh Pittsburgh, Pennsylvania.

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http://dx.doi.org/10.1016/j.jpeds.2016.12.029DOI Listing
March 2017

The role of imaging in diagnosing diseases of the distal radioulnar joint, triangular fibrocartilage complex, and distal ulna.

AJR Am J Roentgenol 2014 Jul;203(1):146-53

1 All authors: Department of Radiology, University of Cincinnati College of Medicine, 234 Goodman St (Mail code 0761), Cincinnati, OH 45267-0761.

Objective: The purpose of this article is to review the anatomy, biomechanics, and multimodality imaging findings of common and uncommon distal radioulnar joint (DRUJ), triangular fibrocartilage complex, and distal ulna abnormalities.

Conclusion: The DRUJ is a common site for acute and chronic injuries and is frequently imaged to evaluate chronic wrist pain, forearm dysfunction, and traumatic forearm injury. Given the complex anatomy of the wrist, the radiologist plays a vital role in the diagnosis of wrist pain and dysfunction.
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http://dx.doi.org/10.2214/AJR.13.11573DOI Listing
July 2014

Tibial nerve intraneural ganglion cyst in a 10-year-old boy.

Pediatr Radiol 2014 Apr 8;44(4):488-90. Epub 2013 Nov 8.

Department of Radiology, University of Cincinnati College of Medicine, 234 Goodman Street (ML 0761), Cincinnati, OH, 45267-0761, USA,

Intraneural ganglion cysts are uncommon cystic lesions of peripheral nerves that are typically encountered in adults. In the lower extremity, the peroneal nerve is most frequently affected with involvement of the tibial nerve much less common. This article describes a tibial intraneural ganglion cyst in a 10-year-old boy. Although extremely rare, intraneural ganglion cysts of the tibial nerve should be considered when a nonenhancing cystic structure with intra-articular extension is identified along the course of the nerve. This report also details the unsuccessful attempt at percutaneous treatment with US-guided cyst aspiration and steroid injection, an option recently reported as a viable alternative to open surgical resection.
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http://dx.doi.org/10.1007/s00247-013-2814-yDOI Listing
April 2014