Publications by authors named "Edward Y Lee"

272 Publications

Thoracic Multidetector Computed Tomography Findings of Dedicator of Cytokinesis 8 Deficiency in Children.

J Thorac Imaging 2021 Mar 22. Epub 2021 Mar 22.

*Department of Radiology †Department of Pathology ‡Department of Pediatrics, Division of Pulmonary Medicine §Department of Pediatrics, Division of Immunology, Boston Children's Hospital and Harvard Medical School, Boston, MA.

Purpose: To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of dedicator of cytokinesis 8 (DOCK8) deficiency, a rare autosomal recessive form of hyperimmunoglobulin E syndrome, in children.

Materials And Methods: All pediatric patients (age 18 y and below) with a known diagnosis of DOCK8 deficiency based on genetic testing who underwent thoracic MDCT studies from November 2004 to November 2020 were included. Two pediatric radiologists independently evaluated MDCT studies for the presence of thoracic abnormalities in the lung [ground-glass opacity (GGO), consolidation, pulmonary nodule, mass, cyst, and bronchiectasis], pleura (pleural effusion and pneumothorax), and mediastinum (lymphadenopathy). When a lung abnormality was present, laterality, distribution (upper, middle, and lower lung zone), and extent were also evaluated. When a pleural abnormality was identified, laterality and size of the abnormality were also assessed. When mediastinal lymphadenopathy was present, its location and size were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics.

Results: In all, 17 thoracic MDCT studies from 17 individual pediatric patients [5 males (29%) and 12 females (71%); mean age: 7.4 y; SD: 3.7; range: 1 to 13 y] comprised the final study population. Among 17 thoracic MDCT studies, 11 studies (65%) were performed with intravenous contrast (IV) and the remaining 6 MDCT studies (35%) were obtained without IV contrast. Bilateral bronchiectasis (11/17; 65%) with a middle lung zone predominance (8/11; 73%) was the most frequently detected lung abnormality, followed by GGO in 9/17 patients (53%). Among 11 contrast-enhanced MDCT studies, the majority (9 patients, 82%) had mediastinal lymphadenopathy. There was excellent interobserver κ agreement between 2 independent reviewers for detecting abnormalities on thoracic MDCT studies (κ>0.90).

Conclusion: Children with DOCK8 deficiency have characteristic thoracic MDCT findings, including bilateral bronchiectasis with a middle lung zone predominance, GGO, and mediastinal lymphadenopathy. When these characteristic thoracic MDCT findings are detected, although rare, DOCK8 deficiency should be considered as a possible underlying diagnosis in the pediatric population.
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http://dx.doi.org/10.1097/RTI.0000000000000587DOI Listing
March 2021

Thoracic MDCT findings of a combined congenital lung lesion: Bronchial atresia associated with congenital pulmonary airway malformation.

Pediatr Pulmonol 2021 Jul 1. Epub 2021 Jul 1.

Department of Radiology, Harvard Medical School, Boston Children's Hospital, Boston, Massachusetts, USA.

Purpose: To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children.

Materials And Methods: All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. ill-defined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics.

Results: Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98).

Conclusion: The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass, representing the CPAM component. Accurate recognition of these characteristic MDCT findings of combined BA-CPAM congenital lung lesion has great potential to help differentiate this combined congenital lung lesion from other thoracic pathology in children.
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http://dx.doi.org/10.1002/ppul.25556DOI Listing
July 2021

Invited Commentary: New Paradigm for Imaging Evaluation of Pediatric Mediastinal Masses-ITMIG Classification System.

Radiographics 2021 Jul-Aug;41(4):E123-E125. Epub 2021 Jun 4.

From the Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115.

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http://dx.doi.org/10.1148/rg.2021210015DOI Listing
June 2021

Reply to "Radiologic Manifestations of Multisystem Inflammatory Syndrome in Children".

AJR Am J Roentgenol 2021 Aug 2;217(2):W9. Epub 2021 Jun 2.

Montefiore Medical Center, Bronx, NY.

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http://dx.doi.org/10.2214/AJR.21.25854DOI Listing
August 2021

Updates and Advances: Pediatric Musculoskeletal Infection Imaging Made Easier for Radiologists and Clinicians.

Semin Musculoskelet Radiol 2021 Feb 21;25(1):167-175. Epub 2021 May 21.

Division of Thoracic Imaging, Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.

Infants and children often present with a wide range of musculoskeletal (MSK) infections in daily clinical practice. This can vary from relatively benign superficial infections such as cellulitis to destructive osseous and articular infections and life-threatening deep soft tissue processes such as necrotizing fasciitis. Imaging evaluation plays an essential role for initial detection and follow-up evaluation of pediatric MSK infections. Therefore, a clear and up-to-date knowledge of imaging manifestations in MSK infections in infants and children is imperative for timely and accurate diagnosis that, in turn, can result in optimal patient management. This article reviews an up-to-date practical imaging techniques, the differences between pediatric and adult MSK infections, the spectrum of pediatric MSK infections, and mimics of pediatric MSK infections encountered in daily clinical practice by radiologists and clinicians.
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http://dx.doi.org/10.1055/s-0041-1723004DOI Listing
February 2021

Pediatric Musculoskeletal Trauma: Upper Limb.

Semin Musculoskelet Radiol 2021 Feb 21;25(1):105-122. Epub 2021 May 21.

Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.

Pediatric upper extremity injuries, acute and chronic due to overuse, are commonly encountered in the clinical setting. However, interpretation of imaging studies is often challenging in pediatric patients because of changing postnatal skeletal development and the broad spectrum of pathology that can potentially occur. This article discusses normal skeletal development and the pathomechanics of skeletal injuries in pediatric patients. It also outlines a framework for imaging as it pertains to the pediatric upper extremity, with specific emphasis on acute and chronic injuries to the shoulder, elbow, forearm, and wrist.
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http://dx.doi.org/10.1055/s-0041-1723963DOI Listing
February 2021

Thoracic Multidetector Computed Tomography Angiography of Primary Pulmonary Vein Stenosis in Children: Evaluation of Characteristic Extravascular Findings.

J Thorac Imaging 2021 May 12. Epub 2021 May 12.

Departments of Radiology Cardiology Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA.

Purpose: The purpose of this study was to investigate the extravascular thoracic multidetector computed tomography (MDCT) angiography findings of pediatric primary pulmonary vein stenosis (PVS) by comparing extravascular thoracic MDCT angiography findings in children with and without PVS.

Materials And Methods: All pediatric patients (age 18 y and below) with a known diagnosis of primary PVS, confirmed by echocardiogram and/or conventional angiography, who underwent thoracic MDCT angiography studies from July 2006 to December 2020 were included. A comparison group, comprised of age-matched and sex-matched pediatric patients without PVS who underwent thoracic MDCT angiography studies during the same study period, was also generated. Two pediatric radiologists independently evaluated thoracic MDCT angiography studies for the presence of extravascular thoracic abnormalities in the lung (ground-glass opacity [GGO], consolidation, pulmonary nodule, mass, cyst, septal thickening, fibrosis, and bronchiectasis), pleura (pleural thickening, pleural effusion and pneumothorax), and mediastinum (lymphadenopathy and mass). When a thoracic abnormality was identified, the location and distribution of the abnormality (in relation to the location of PVS) were also evaluated. Extravascular thoracic MDCT angiography findings of pediatric patients with and without primary PVS were compared. Interobserver agreement between the 2 independent reviewers was evaluated with κ statistics.

Results: The study group consisted of 15 thoracic MDCT angiography studies from 15 individual pediatric patients with primary PVS (8 males [53%] and 7 females [47%]; mean age: 10.9 mo; SD: 11.7 mo; range: 1 to 48 mo). The comparison group consisted of 15 thoracic MDCT angiography studies from 15 individual pediatric patients without PVS (8 males [53%] and 7 females [47%]; mean age: 10.2 mo; SD: 11.5 mo; range: 1 to 48 mo). In children with primary PVS, the characteristic extravascular thoracic MDCT angiography findings were GGO (14/15; 93%), septal thickening (5/15; 33%), pleural thickening (14/15; 93%), and ill-defined, mildly heterogeneously enhancing, noncalcified soft tissue mass (14/15; 93%) following the contours of PVS in the mediastinum. There was excellent interobserver κ agreement between 2 independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (κ=0.99 for the study group and κ=0.98 for the comparison group).

Conclusions: Children with primary PVS have characteristic extravascular thoracic MDCT angiography findings. In the lungs and pleura, GGO, septal thickening, and pleural thickening are common findings. Importantly, in the mediastinum, the presence of a mildly heterogeneously enhancing, noncalcified soft tissue mass in the distribution of PVS is a novel characteristic thoracic MDCT angiography finding unique to pediatric primary PVS. When this constellation of extravascular thoracic MDCT angiography findings is detected, although rare, primary PVS should be considered as a possible underlying diagnosis, especially in symptomatic children.
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http://dx.doi.org/10.1097/RTI.0000000000000590DOI Listing
May 2021

Thoracic CTA in infants and young children: Image quality of dual-source CT (DSCT) with high-pitch spiral scan mode (turbo flash spiral mode) with or without general anesthesia with free-breathing technique.

Pediatr Pulmonol 2021 Aug 20;56(8):2660-2667. Epub 2021 May 20.

Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Purpose: To determine whether diagnostic quality thoracic computed tomography angiography (CTA) studies can be obtained without general anesthesia (GA) in infants and young children using dual-source computed tomography (DSCT) with turbo flash spiral mode (TFSM) and free-breathing technique.

Materials And Methods: All consecutive infants and young children (≤ 6 years old) who underwent thoracic CTA studies from January 2018 to October 2020 for suspected congenital thoracic disorders were categorized into two groups: with GA (Group 1) and without GA (Group 2). All thoracic CTA studies were performed on a DSCT scanner using TFSM and free-breathing technique. Two pediatric thoracic radiologists independently evaluated motion artifact in three lung zones (upper, mid, and lower). Degree of motion artifact was graded 0-3 (0, none; 1, mild; 2, moderate; and 3, severe). Logistic models adjusted for age and gender were used to compare the degree of motion artifact between lung zones. Interobserver agreement between reviewers was evaluated with kappa statistics.

Results: There were a total of 73 pediatric patients (43 males (59%) and 30 females (41%); mean age, 1.4 years; range, 0-5.9 years). Among these 73 patients, 42 patients (58%) underwent thoracic CTA studies with GA (Group 1) and the remaining 31 patients (42%) underwent thoracic CTA studies without GA (Group 2). Overall, the degree of motion artifact was higher for Group 2 (without GA). However, only a very small minority (1/31, 3%) of Group 2 (without GA) thoracic CTA studies had severe motion artifact. There was no significant difference between the two groups with respect to the presence of severe motion artifact (odds ratio [OR] = 6, p = .222). When two groups were compared with respect to the presence of motion artifact for individual lung zones, motion artifact was significantly higher in the upper lung zone for Group 2 (without GA) (OR = 20, p = .043). Interobserver agreement for motion artifact was high, the average Kappa being 0.81 for Group 1 and 0.95 for Group 2.

Conclusion: Although the degree of motion artifact was higher in the group without GA, only a small minority (3%) of thoracic CTA studies performed without GA had severe motion artifact, rendering the study nondiagnostic. Therefore, the results of this study support the use of thoracic CTA without GA using DSCT with TFSM and free-breathing in infants and young children. In addition, given that motion artifact was significantly higher in the upper lung zone without GA, increased stabilization in the upper chest and extremities should be considered.
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http://dx.doi.org/10.1002/ppul.25446DOI Listing
August 2021

Thoracic Multidetector Computed Tomography Evaluation of Inflammatory Myofibroblastic Tumor of the Lung in Pediatric Patients in the Era of Modern Diagnosis.

J Thorac Imaging 2021 Apr 2. Epub 2021 Apr 2.

Departments of Radiology Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA Department of Pediatric Radiology, Clinical Radiology Institute, University Medical Centre Ljubljana, Ljubljana, Slovenia Department of Radiology, College of Medicine and Health Sciences, Al Ain, United Arab Emirates.

Purpose: The purpose of this study was to investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven inflammatory myofibroblastic tumor (IMT) of the lung in children in the era of modern understanding based on refined pathologic diagnosis.

Materials And Methods: All pediatric patients (age 18 y and above) with a known pathologic diagnosis of IMT of the lung who underwent thoracic MDCT studies from May 2008 to December 2020 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung (nodule, mass, cyst, ground-glass opacity, consolidation), pleura (pleural effusion, pneumothorax), and mediastinum and hilum (lymphadenopathy). When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), location (laterality, lobar distribution, and intraparenchymal vs. pleural-based), borders (well-circumscribed vs. ill-defined), the presence and type of associated calcification (punctate, dense, curvilinear, or flocculent), the presence of associated cavitation, contrast enhancement pattern (homogeneous, heterogenous, central, or peripheral), and other associated findings (neural foramen involvement, anomalous vessels, mass effect, and invasion of adjacent thoracic structures) were also evaluated. Interobserver agreement between 2 independent reviewers was evaluated with κ statistics.

Results: In all, 12 thoracic MDCT studies from 12 individual pediatric patients (5 males [42%] and 7 females [58%]; mean age: 9.9 y; SD: 4.4 y; range: 2 to 16 y) comprised the final study population. All 12 thoracic MDCT studies (100%) were performed with intravenous contrast. The most frequent MDCT finding of IMT of the lung in children is a solitary (92%), pleural-based (83%), well-circumscribed (100%), solid (92%) mass with heterogenous contrast enhancement (100%), often with dense calcification (50%), which occurred in both lungs and all lobes with similar frequency. No pleural abnormality (pleural effusion, pneumothorax) or mediastinal abnormality (lymphadenopathy) was detected. In addition, although mass effect on adjacent thoracic structures was frequently seen (42%), no invasion, neural foramen involvement, or associated anomalous vessels was identified. There was excellent interobserver κ agreement between 2 independent reviewers for detecting abnormalities on thoracic MDCT studies (κ>0.95).

Conclusions: IMT of the lung in children typically presents as a solitary, pleural-based, well-circumscribed, solid mass with heterogenous contrast enhancement, often with dense calcification, without significant laterality or lobar preference. In addition, pleural or mediastinal abnormalities are characteristically absent. These notable MDCT attributes of IMT of the lung are an important and novel finding, with great potential to help differentiate pediatric IMT of the lung from other thoracic masses in children.
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http://dx.doi.org/10.1097/RTI.0000000000000589DOI Listing
April 2021

Perceived Impact of COVID-19 on Pediatric Radiology Departments Around the World: WFPI COVID-19 Task Force Survey Results from 6 Continents.

Radiol Cardiothorac Imaging 2020 Oct 17;2(5):e200422. Epub 2020 Sep 17.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, United States of America (A.F., P.R.J., A.J.W., E.Y.L.); Department of Radiology, University of Bristol, Bristol, United Kingdom (J.K.); Department of Imaging and Interventional Radiology, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong, China (W.C.C.); Department of Pediatric Radiology, University Hospital Materno-Infantil Vall d'Heborn, Barcelona, Spain (P.G.); Alta Exelencia Diagnostica and Department of Radiology, Clinica Diagnostico por Imagem (CDPI), Rio De Janeiro, Brazil (P.D.); Department of Radiology, College of Medicine and Health Sciences, Al Ain, United Arab Emirates (K.M.D.); Department of Pediatric Radiology, Clinical Radiology Institute, University Medical Center Ljubljana, Ljubljana, Slovenia (D.P.); Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa (T.K.).

Purpose: To investigate how COVID-19 has impacted pediatric radiology practice around the world at the present time.

Materials And Methods: This study was based on a survey conducted by the World Federation of Pediatric Imaging (WFPI) COVID-19 task force. Pediatric radiology representatives from countries around the world were identified and asked to complete an electronic survey consisting of 17 questions related to the impact of, concerns surrounding, and education related to COVID-19 on pediatric radiology. For each outcome variable, a pair of 2-way frequency tables was constructed: one against COVID-19 prevalence, and another against region. Tests of independence of the tables' row and column factors were performed based on Fisher's exact test and odds ratios (OR) were estimated via logistic regression models corresponding to these 2-way tables.

Results: The survey (response rate 87%, 72/83) comprised representatives from 71 countries and Hong Kong across 6 continents. 66/72 respondents (92%) indicated that COVID-19 has resulted in moderate (21/72, 29%), significant (36/72, 50%), or complete (9/72, 13%) change in radiology departments/practices in their countries. The two most frequent concerns over the next four months were personal/family health (54/72, 75%) and exposure (48/72, 67%). 79% (57/72) of respondents indicated some level of discomfort in identifying pediatric COVID-19 imaging manifestations. Changes in resident education were reported by 68/72 (94%) respondents, and 22/72 (31%) were concerned that the likelihood of current trainees pursuing a career in pediatric radiology will be impacted.

Conclusion: COVID-19 has had a substantial negative impact on pediatric radiology practice around the world.
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http://dx.doi.org/10.1148/ryct.2020200422DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7504684PMC
October 2020

Thoracic Imaging Findings of Multisystem Inflammatory Syndrome in Children Associated with COVID-19: What Radiologists Need to Know Now.

Radiol Cardiothorac Imaging 2020 Aug 30;2(4):e200346. Epub 2020 Jul 30.

Department of Radiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave, Boston, MA 02115 (A.J.W., A.M.F., E.Y.L.); and Department of Radiology and Pediatrics, The Children's Hospital at Montefiore and Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, 10467 (E.B., M.C.L., J.K.).

The coronavirus disease 2019 (COVID-19) global pandemic is an ongoing public health emergency, with over 4 million confirmed cases worldwide. Due to the novel nature of this coronavirus and our evolving understanding of its pathophysiology, there is continued uncertainty surrounding diagnosis and management of COVID-19, especially in pediatric patients. In addition, a new febrile hyperinflammatory Kawasaki-like syndrome (also known as multisystem inflammatory syndrome in children, or MIS-C) has emerged in pediatric patients with temporal association to COVID-19 infection. This review article aims to provide an up-to-date review of the clinical and imaging findings of pediatric MIS-C associated with COVID-19, compared with typical acute pediatric COVID-19 infection, with an emphasis on thoracic imaging findings. Supplemental material is available for this article. © RSNA, 2020.
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http://dx.doi.org/10.1148/ryct.2020200346DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7397565PMC
August 2020

International Expert Consensus Statement on Chest Imaging in Pediatric COVID-19 Patient Management: Imaging Findings, Imaging Study Reporting, and Imaging Study Recommendations.

Radiol Cardiothorac Imaging 2020 Apr 23;2(2):e200214. Epub 2020 Apr 23.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115 (A.M.F., A.J.W., E.Y.L.); Department of Radiology, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Wash (G.S.P.); Department of Imaging and Interventional Radiology, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong, China (W.C.C.); Department of Radiology, ALTA-Excelência Diagnóstica/Dasa, Rio de Janeiro, Brazil (P.D.); Department of Radiology, College of Medicine and Health Sciences, Al Ain, United Arab Emirates (K.M.D.); Department of Pediatric Radiology, University Hospital Materno-Infantil Vall d'Hebron, Barcelona, Spain (P.G.P.); and Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa (T.K.).

Coronavirus disease 2019 (COVID-19) has quickly spread since it was first detected in December 2019 and has evolved into a global pandemic with over 1.7 million confirmed cases in over 200 countries around the world at the time this document is being prepared. Owing to the novel nature of the virus and the rapidly evolving understanding of the disease, there is a great deal of uncertainty surrounding the diagnosis and management of COVID-19 pneumonia in pediatric patients. Chest imaging plays an important role in the evaluation of pediatric patients with COVID-19; however, there is currently little information available describing imaging manifestations of COVID-19 in pediatric patients and even less information discussing the utilization of imaging studies in pediatric patients. To specifically address these concerns, a group of international experts in pediatric thoracic imaging from five continents convened to create a consensus statement describing the imaging manifestations of COVID-19 in the pediatric population, discussing the potential utility of structured reporting during the COVID-19 pandemic, and generating consensus recommendations for utilization of chest radiographs and CT in the evaluation of pediatric patients with COVID-19. The results were compiled into two structured reporting algorithms (one for chest radiographs and one for chest CT) and eight consensus recommendations for the utilization of chest imaging in pediatric COVID-19 infection. © RSNA, 2020.
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http://dx.doi.org/10.1148/ryct.2020200214DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7233446PMC
April 2020

Diagnostic utility of MDCT in evaluation of persistent stridor in children: Large airway causes and benefit of additional findings.

Pediatr Pulmonol 2021 Jul 31;56(7):2169-2176. Epub 2021 Mar 31.

Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Objectives: To assess the diagnostic utility of MDCT in the evaluation of persistent stridor in children for the underlying large airway causes and benefit of additional findings.

Methods: All consecutive pediatric patients who underwent MDCT for the evaluation of persistent stridor from December 2018 to February 2020 were included. Two pediatric radiologists independently reviewed MDCT studies for the presence of abnormalities at six large airway levels: (1) nasopharynx, (2) oropharynx, (3) glottis, (4) subglottis, (5) trachea, and (6) mainstem bronchi. In addition, studies were evaluated for the presence of non-airway abnormalities. Interobserver agreement between two reviewers was evaluated with kappa statistics.

Results: There were a total of 40 pediatric patients (age range: 1 day-4 years. MDCT detected large airway abnormalities in 20 (50%) out of 40 patients, including 4 (20%) in nasopharynx, 4 (20%) in glottis, 4 (20%) in trachea, 3 (15%) in subglottis, 3 (15%) in mainstem bronchi, and 2 (10%) in oropharynx. Non-airway abnormalities were seen in 13 (32.5%) children, including 9 (69%) in the lungs, 3 (23%) in the soft tissue, and 1 (8%) in the bone. The remaining 7 (17.5%) studies were normal. There was excellent interobserver agreement seen for detecting large airway and non-airway abnormalities (k > 0.90).

Conclusion: MDCT has high diagnostic utility in diagnosing large airway causes of persistent stridor in children. It can also provide additional information regarding non-airway abnormalities. Therefore, MDCT has the potential to be utilized as a noninvasive problem-solving imaging modality in pediatric patients with persistent stridor.
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http://dx.doi.org/10.1002/ppul.25382DOI Listing
July 2021

CT Features of Benign Intrapulmonary Lymph Nodes in Pediatric Patients With Known Extrapulmonary Solid Malignancy.

AJR Am J Roentgenol 2021 05 17;216(5):1357-1362. Epub 2021 Mar 17.

Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, MA.

The purpose of our study was to determine the CT features of benign intrapulmonary lymph nodes in pediatric patients with known extrapulmonary solid malignancy. A retrospective review of surgical pathology archives was performed to identify consecutive chest CT studies of pediatric patients (≤ 18 years) with extrapulmonary solid malignancy and histologically confirmed benign intrapulmonary lymph nodes between January 1, 2004, and March 15, 2020. CT features of intrapulmonary lymph nodes-including size, shape, margin, type, associated calcification or fat, and location-were independently evaluated by two pediatric radiologist reviewers. The CT features of benign intrapulmonary lymph nodes in pediatric patients were analyzed using summary statistics. Interobserver agreement was measured with the kappa coefficient. There were 36 pathology-confirmed benign intrapulmonary lymph nodes in 27 pediatric patients (18 boys and nine girls; mean age, 12 years; age range, 1-18.2 years). Twenty-three (63.9%) of the benign intrapulmonary lymph nodes were biopsied from the right lung and 13 (36.1%) from the left lung ( = .03). The mean size, determined from CT studies, of benign intrapulmonary lymph nodes was 3.6 mm (SD, 1.4 mm; range, 1.3-7.8 mm). Triangular shape (25/36, 69.4%) was the most common shape of the benign intrapulmonary lymph nodes. Less commonly seen shapes of benign intrapulmonary lymph nodes were oval (6/36, 16.7%), round (3/36, 8.3%), and trapezoidal (2/36, 5.6%). All benign intrapulmonary lymph nodes were smoothly marginated and solid without associated calcification or fat. Of the 36 benign intrapulmonary lymph nodes, 15 (41.7%) were pleura-based; 11 (30.6%), perifissural; and 10 (27.8%), parenchymal. The kappa value for interobserver agreement between the two reviewers was 0.917 (95% CI, 0.825-1.000; standard error, 0.047), which corresponds to near-perfect agreement. In pediatric patients with known extrapulmonary solid malignancy, benign intrapulmonary lymph nodes are subcentimeter (mean size, 3.6 mm), smoothly marginated, and solid without containing calcification or fat on CT. In particular, triangular shape was the most commonly encountered shape of a benign intrapulmonary lymph node.
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http://dx.doi.org/10.2214/AJR.20.23363DOI Listing
May 2021

Pulmonary manifestations of immune dysregulation in CTLA-4 haploinsufficiency and LRBA deficiency.

Pediatr Pulmonol 2021 Jul 23;56(7):2232-2241. Epub 2021 Mar 23.

Division of Pulmonary Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Objective: The primary immunodeficiency syndromes of cytotoxic T lymphocyte-associated protein 4 (CTLA-4) haploinsufficiency and lipopolysaccharide-responsive and beige-like anchor protein (LRBA) deficiency present with multisystem immune dysregulation. The aim of this study was to characterize and compare the pulmonary manifestations of these two diseases.

Methods: We retrospectively analyzed the pulmonary clinical, radiologic, and histopathologic characteristics of six patients with CTLA-4 haploinsufficiency and four patients with LRBA deficiency with pulmonary involvement followed at a large tertiary care center.

Results: Chronic respiratory symptoms were more frequent in patients with LRBA deficiency versus CTLA-4 haploinsufficiency (3/4 vs. 1/6). Cough was the most common respiratory symptom. Abnormalities in pulmonary exam and pulmonary function testing were more frequent in LRBA deficiency (4/4, 2/4) compared to CTLA-4 haploinsufficiency (1/6, 2/6). Chest computed tomography (CT) findings included mediastinal lymphadenopathy (4/4 in LRBA deficiency vs. 1/4 in CTLA-4 haploinsufficiency), pulmonary nodules (4/4, 3/4), ground-glass opacification (4/4, 3/4), and bronchiectasis (3/4, 1/4). Lymphocytic inflammation, concentrated bronchovasculocentrically and paraseptally, was the predominant pathologic finding and was observed in all patients who had lung biopsies (N = 3 with LRBA deficiency; N = 3 with CTLA-4 haploinsufficiency).

Conclusion: Despite phenotypic overlap amongst these diseases, LRBA deficiency demonstrated greater severity of pulmonary disease, indicated by respiratory symptoms, pulmonary exam, and intrathoracic radiologic findings. Chest CT was the most sensitive indicator of pulmonary involvement in both disorders. Lymphocytic inflammation is the key histologic feature of both disorders. Pediatric pulmonologists should consider these disorders of immune dysregulation in the relevant clinical context to provide earlier diagnosis, comprehensive pulmonary evaluation and treatment.
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http://dx.doi.org/10.1002/ppul.25373DOI Listing
July 2021

Comparison of chest radiography and chest CT for evaluation of pediatric COVID-19 pneumonia: Does CT add diagnostic value?

Pediatr Pulmonol 2021 06 25;56(6):1409-1418. Epub 2021 Feb 25.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Purpose: First, to investigate the added diagnostic value of chest computed tomography (CT) for evaluating COVID-19 in symptomatic children by comparing chest CT findings with chest radiographic findings, and second, to identify the imaging signs and patterns on CT associated with COVID-19 pneumonia in children.

Materials And Methods: From March 2020 to December 2020, 56 consecutive children (33 males and 23 girls; mean age ± SD, 14.8 ± 5.0 years; range, 9 months-18 years) with mild to moderate symptom and laboratory confirmed COVID-19 (based on Centers for Disease Control criteria) underwent both chest radiography and chest CT on the same day within the first 2 days of initial presentation to the hospital. Two experienced radiologists independently evaluated chest radiographs and chest CT studies for thoracic abnormalities. The findings from chest radiography and chest CT were compared to evaluate the added diagnostic value of chest CT for affecting patient management. Interobserver agreement was measured with Cohen's κ statistics.

Results: Eleven (19.6%) of 56 patients had abnormal chest radiographic findings, including ground-glass opacity (GGO) in 5/11 (45.4%) and combined GGO and consolidation in 6/11 (54.5%). On chest CT, 26 (46.4%) of 56 patients had abnormal CT findings, including combined GGO and consolidation in 19/26 (73.1%), GGO in 6/26 (23.1%), and consolidation in 1/26 (3.8%). Chest CT detected all thoracic abnormalities seen on chest radiography in 11/26 (42.3%) cases. In 15/26 (57.7%), chest CT detected lung abnormalities that were not observed on chest radiography, which included GGO and consolidation in 9/15 (60%), GGO in 5/15 (33.3%), and consolidation in 1/15 (6.6%) cases. These additional CT findings did not affect patient management. In addition, chest CT detected radiological signs and patterns, including the halo sign, reversed halo sign, crazy paving pattern, and tree-in-bud pattern. There was almost perfect interobserver agreement between the two reviewers for detecting findings on both chest radiographs (κ, 0.89, p = .001) and chest CT (κ, 0.96, p = .001) studies.

Conclusion: Chest CT detected lung abnormalities, including GGO and/or consolidation, that were not observed on chest radiography in more than half of symptomatic pediatric patients with COVID-19 pneumonia. However, these additional CT findings did not affect patient management. Therefore, CT is not clinically indicated for the initial evaluation of mild to moderately symptomatic pediatric patients with COVID-19 pneumonia.
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http://dx.doi.org/10.1002/ppul.25313DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8014659PMC
June 2021

Prospective Evaluation of Free-Breathing Fast T2-Weighted MultiVane XD Sequence at 3-T MRI for Large Airway Assessment in Pediatric Patients.

AJR Am J Roentgenol 2021 04 3;216(4):1074-1080. Epub 2021 Feb 3.

Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, MA.

The purpose of our study was to prospectively evaluate the technical feasibility of the free-breathing fast T2-weighted MultiVane XD sequence (sequence with non-Cartesian k-space filling using radial rectangular blades) at 3-T MRI for large airway assessment in pediatric patients. Forty consecutive pediatric patients (23 boys and 17 girls; age range, 5-15 years) referred for MRI examination for indications not related to neck, chest, or large airway disorders were enrolled in this prospective research study. All children underwent MRI in three planes using a free-breathing fast T2-weighted MultiVane XD sequence at 3-T MRI. The MR images were assessed by two pediatric radiologists independently for visualization of the large airways at six levels. The quality of the MR images was assessed and graded. Interobserver agreement between two radiologists was assessed using the kappa test, McNemar test, and intraclass correlation coefficients. High-quality MR images of the large airways were obtained in at least one plane in 38 MRI examinations (95.0%) by reviewer 1 and 37 MRI examinations (92.5%) by reviewer 2. Best-quality MR images with the least artifacts were seen in the sagittal plane followed by the coronal plane and the axial plane. The kappa test of agreement showed almost-perfect agreement between the two radiologists for MR image quality in the sagittal (κ = 1), coronal (κ = 0.96), and axial (κ = 0.81) planes. The McNemar test and intraclass correlation coefficients revealed similar results. The free-breathing fast T2-weighted MultiVane XD sequence at 3-T MRI is a technically feasible and promising new MRI technique for evaluating the large airways of pediatric patients in daily clinical practice.
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http://dx.doi.org/10.2214/AJR.20.23225DOI Listing
April 2021

Lung ultrasound for evaluation of pediatric COVID-19 infection: What we already know, what we need to investigate now, and what we can expect in the future.

Pediatr Pulmonol 2021 06 1;56(6):1295-1296. Epub 2021 Feb 1.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

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http://dx.doi.org/10.1002/ppul.25281DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8014223PMC
June 2021

Pediatric Lung MRI: Currently Available and Emerging Techniques.

AJR Am J Roentgenol 2021 03 21;216(3):781-790. Epub 2021 Jan 21.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA.

The purpose of this article is to review currently available and emerging techniques for pediatric lung MRI for general radiologists. MRI is a radiation-free alternative to CT, and clearly understanding the strengths and limitations of established and emerging techniques of pediatric lung MRI can allow practitioners to select and combine the optimal techniques, apply them in clinical practice, and potentially improve early diagnostic accuracy and patient management.
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http://dx.doi.org/10.2214/AJR.20.23104DOI Listing
March 2021

Practical Imaging Strategies for Intussusception in Children.

AJR Am J Roentgenol 2020 12 21;215(6):1449-1463. Epub 2020 Oct 21.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA.

Intussusception is the most common cause of intestinal obstruction in young children. Radiology has a key role in its diagnosis and treatment. This systematic review summarizes the currently available evidence for best practices in radiologic management of pediatric intussusception. High diagnostic accuracy and lack of ionizing radiation make ultrasound (US) the preferred imaging modality for diagnosing intussusception. For intussusception reduction, fluoroscopy-guided pneumatic enema and US-guided hydrostatic enema are equally dependable and safe techniques. The areas that warrant further research in this field include the efficacy and safety of the US-guided pneumatic enema, potential benefits of sedation and general anesthesia for the reduction procedure, and the optimal management of intussusceptions potentially involving pathologic lead points.
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http://dx.doi.org/10.2214/AJR.19.22445DOI Listing
December 2020

Private Tour Guide to Pediatric Coronavirus Disease of 2019 and Multisystem Inflammatory Syndrome in Children in 10 Minutes: What Thoracic Radiologists Need to Know.

J Thorac Imaging 2021 Jan;36(1):24-30

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA.

Filtering through the plethora of radiologic studies generated in response to the coronavirus disease of 2019 (COVID-19) pandemic can be time consuming and impractical for practicing thoracic radiologists with busy clinical schedules. To further complicate matters, several of the imaging findings in the pediatric patients differ from the adult population. This article is designed to highlight clinically useful information regarding the imaging manifestations of pediatric COVID-19 pneumonia, including findings more unique to pediatric patients, and multisystem inflammatory syndrome in children.
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http://dx.doi.org/10.1097/RTI.0000000000000565DOI Listing
January 2021

Imaging Findings in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated With Coronavirus Disease (COVID-19).

AJR Am J Roentgenol 2021 Feb 29;216(2):507-517. Epub 2020 Jul 29.

Department of Radiology, Montefiore Medical Center, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 111 E 210th St, Bronx, NY 10467.

A multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease (COVID-19) has recently been described. The purpose of our study was to evaluate the imaging findings of MIS-C associated with COVID-19. Imaging studies and medical records of patients (age range, 0-20 years) admitted with MIS-C between April 22 and May 21, 2020, were retrospectively reviewed. Thoracic imaging studies were evaluated for parenchymal, mediastinal and hilar, and cardiovascular abnormalities. Abdominal imaging studies were evaluated for abnormalities of solid viscera, hollow viscera, and the peritoneum as well as the mesentery and retroperitoneum. Studies were reviewed independently by two radiologists, and disagreements were resolved by a third senior radiologist. Sixteen patients (10 male and six female patients; age range, 20 months-20 years) were included in this study. All 16 patients presented with fever. Other presenting signs and symptoms included the following: vomiting (12/16, 75%), abdominal pain (11/16, 69%), rash (10/16, 63%), conjunctivitis (8/16, 50%), diarrhea (7/16, 44%), headache (6/16, 38%), and sore throat (5/16, 31%). Shortness of breath and cough were each present in one patient. Chest radiography showed cardiomegaly (10/16, 63%), congestive heart failure or cardiogenic pulmonary edema (9/16, 56%), atelectasis (9/16, 56%), pleural effusions (7/16, 44%), acute respiratory distress syndrome (2/16, 13%), and pneumonia (1/16, 6%). Eight patients (50%) were evaluated for pulmonary embolism (PE) (six [75%] by CT angiography [CTA] and two [25%] by ventilation-perfusion scintigraphy). In two of the eight patients (25%), CTA showed a segmental PE. Abdominal imaging findings (ultrasound, CT, and radiography) included small-volume ascites (6/16, 38%), hepatomegaly (6/16, 38%), echogenic kidneys (5/16, 31%), bowel wall thickening (3/16, 19%), gallbladder wall thickening (3/16, 19%), mesenteric lymphadenopathy (2/16, 13%), splenomegaly (1/16, 6%), and bladder wall thickening (1/16, 6%). The frequencies of findings based on all the reviewed modalities were as follows: cardiomegaly (12/16, 75%), pleural effusion (10/16, 63%), and atelectasis (10/16, 63%). Absolute interobserver agreement was 0.69-1 for thoracic findings and 0.17-1 for abdominal findings. Fifteen patients (94%) were discharged from the hospital (length of hospital stay: range, 3-20 days), and one patient remained in the hospital at the end of the study period. There were no mortalities. MIS-C associated with COVID-19 is characterized predominantly by cardiovascular abnormalities, although solid visceral organ, gallbladder, and bowel abnormalities as well as ascites are also seen, reflecting a multisystemic inflammatory process. The constellation of imaging findings in the setting of COVID-19 may alert radiologists to the diagnosis of MIS-C before rapid deterioration of patients.
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http://dx.doi.org/10.2214/AJR.20.24032DOI Listing
February 2021

Practical guide for pediatric pulmonologists on imaging management of pediatric patients with COVID-19.

Pediatr Pulmonol 2020 09 1;55(9):2213-2224. Epub 2020 Jul 1.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

Understanding of coronavirus disease 2019 is rapidly evolving with new articles on the subject daily. This flood of articles can be overwhelming for busy practicing clinicians looking for key pieces of information that can be applied in daily practice. This review article synthesizes the reported imaging findings in pediatric Coronavirus disease 2019 (COVID-19) across the literature, offers imaging differential diagnostic considerations and useful radiographic features to help differentiate these entities from COVID-19, and provides recommendations for requesting imaging studies to evaluate suspected cases of pediatric COVID-19.
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http://dx.doi.org/10.1002/ppul.24870DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7283678PMC
September 2020

Pediatric SARS, H1N1, MERS, EVALI, and Now Coronavirus Disease (COVID-19) Pneumonia: What Radiologists Need to Know.

AJR Am J Roentgenol 2020 Sep 30;215(3):736-744. Epub 2020 Apr 30.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115.

The purpose of this article is to review new pediatric lung disorders-including disorders that have occurred in recent years years such as severe acute respiratory syndrome (SARS), swine-origin influenza A (H1N1), Middle East respiratory syndrome (MERS), e-cigarette or vaping product use-associated lung injury (EVALI), and coronavirus disease (COVID-19) pneumonia-to enhance understanding of the characteristic imaging findings. Although the clinical symptoms of SARS, H1N1, MERS, EVALI, and COVID-19 pneumonia in pediatric patients may be nonspecific, some characteristic imaging findings have emerged or are currently emerging. It is essential for radiologists to have a clear understanding of the characteristic imaging appearances of these lung disorders in pediatric patients to ensure optimal patient care.
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http://dx.doi.org/10.2214/AJR.20.23267DOI Listing
September 2020

Supernumerary intrathoracic rib, a rare congenital anomaly: Case report and review of the literature.

Pediatr Pulmonol 2020 06 20;55(6):1487-1489. Epub 2020 Apr 20.

Division of Pulmonary Medicine, Boston Children's Hospital, Boston, Massachusetts.

A 17-year-old female who presented with cough, chest pain, dyspnea, and hemoptysis was found to have an intrathoracic rib. Patients who are diagnosed with intrathoracic ribs are most often asymptomatic and should undergo limited diagnostic workup. Intrathoracic ribs are rare congenital anomalies incidentally identified after chest radiography performed for another indication, as is the case with this patient. In this case, further evaluation was necessary due to persistent symptoms. Here, we used contrast-enhanced ultrasound after chest radiography and computed tomography to further evaluate the fatty intrathoracic mass and exclude vascular features suggestive of a tumor.
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http://dx.doi.org/10.1002/ppul.24783DOI Listing
June 2020

Imaging of Disorders Spanning the Spectrum from Childhood to Adulthood.

Authors:
Edward Y Lee

Radiol Clin North Am 2020 05 2;58(3):xv. Epub 2020 Mar 2.

Division of Thoracic Imaging, Department of Radiology Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

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http://dx.doi.org/10.1016/j.rcl.2020.02.004DOI Listing
May 2020

Congenital Incidental Findings in Children that Can Be Mistaken as True Pathologies in Adults: Pearls and Pittfalls of Imaging Diagnosis.

Radiol Clin North Am 2020 May;58(3):639-652

Division of Thoracic Imaging, Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 20115, USA. Electronic address:

Congenital entities sharing imaging characteristics with true pathologies occasionally are discovered incidentally in adults. These may occur in the neck, chest, abdomen/pelvis, or musculoskeletal systems. Although these incidental findings share imaging features with true pathologic processes, up-to-date knowledge and assessment with the most appropriate imaging modalities generally allow a distinction between congenital entities that may be safely dismissed and pathologic processes requiring further assessment and treatment. This article reviews several of the most common congenital processes that may present incidentally in adult patients mimicking disease. Emphasis is on findings that can be used to distinguish congenital process from true disease processes.
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http://dx.doi.org/10.1016/j.rcl.2020.01.006DOI Listing
May 2020

Genetic Syndromes Affecting Both Children and Adults: A Practical Guide to Imaging-based Diagnosis, Management, and Screening Recommendations for General Radiologists.

Radiol Clin North Am 2020 May;58(3):619-638

Division of Thoracic Imaging, Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

Genetic syndromes are an infrequently encountered but challenging group of conditions for both pediatric and adult radiologists given the multitude of possible findings and important complications associated with these syndromes. This article reviews characteristic syndromic imaging features, as well as discussing important complications and screening recommendations for a selected group of clinically relevant genetic syndromes affecting both pediatric and adult populations.
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http://dx.doi.org/10.1016/j.rcl.2020.01.003DOI Listing
May 2020

Three Distinct Vascular Anomalies Involving Skeletal Muscle: Simplifying the Approach for the General Radiologist.

Radiol Clin North Am 2020 May;58(3):603-618

Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

Venous malformations and hemangiomas of the skeletal muscle are separate entities with different clinical presentation, histology, and imaging findings. Recent advances in the field of vascular anomalies and current efforts in the unification of terminology by the International Society for the Study of Vascular Anomalies are pivotal in understanding and differentiating intramuscular venous malformations and intramuscular capillary-type hemangioma. Fibroadipose vascular anomaly is another recently defined vascular anomaly affecting the skeletal muscle, with a distinct clinical presentation, histology, and imaging appearance. These 3 distinct vascular anomalies are reviewed and their histologic features, clinical presentation, imaging appearance, and treatment are discussed.
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http://dx.doi.org/10.1016/j.rcl.2020.01.005DOI Listing
May 2020

Spectrum of Imaging Manifestations of Vascular Malformations and Tumors Beyond Childhood: What General Radiologists Need to Know.

Radiol Clin North Am 2020 May;58(3):583-601

Division of Thoracic Imaging, Department of Radiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

Vascular anomalies encompass a collection of diagnoses that differ greatly in terms of clinical presentation, natural history, imaging findings, and management. The purpose of this article is to review diagnostic imaging findings of vascular malformations and vascular tumors, excluding the central nervous system, that occur beyond childhood. A widely accepted classification system created by the International Society for the Study of Vascular Anomalies provides a framework for this review, focusing on the entities most likely to be encountered by general radiologists, although several rare but clinically important entities are also reviewed.
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http://dx.doi.org/10.1016/j.rcl.2020.01.004DOI Listing
May 2020