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1.
Pediatr Radiol ; 54(2): 239-249, 2024 02.
Artículo en Inglés | MEDLINE | ID: mdl-38112762

RESUMEN

BACKGROUND: Improving access to magnetic resonance imaging (MRI) in childhood can be facilitated by making it faster and cheaper and reducing need for sedation or general anesthesia (GA) to mitigate motion. Some children achieve diagnostic quality MRI without GA through the use of non- practices fostering their cooperation and/or alleviating anxiety. Employed before and during MRI, these variably educate, distract, and/or desensitize patients to this environment. OBJECTIVE: To assess current utilization of non-sedate practices in pediatric MRI, including variations in practice and outcomes. MATERIALS AND METHODS: A survey-based study was conducted with 1372 surveys emailed to the Society for Pediatric Radiology members in February 2021, inviting one response per institution. RESULTS: Responses from 50 unique institutions in nine countries revealed 49/50 (98%) sites used ≥ 1 non-sedate practice, 48/50 (96%) sites in infants < 6 months, and 11/50 (22%) for children aged 6 months to 3 years. Non-sedate practices per site averaged 4.5 (range 0-10), feed and swaddle used at 47/49 (96%) sites, and child life specialists at 35/49 (71%). Average success rates were moderate (> 50-75%) across all sites and high (> 75-100%) for 20% of sites, varying with specific techniques. Commonest barriers to use were scheduling conflicts and limited knowledge. CONCLUSION: Non-sedate practice utilization in pediatric MRI was near-universal but widely variable across sites, ages, and locales, with room for broader adoption. Although on average non-sedate practice success rates were similar, the range in use and outcomes suggest a need for standardized implementation guidelines, including patient selection and outcome metrics, to optimize utilization and inform educational initiatives.


Asunto(s)
Anestesia General , Imagen por Resonancia Magnética , Lactante , Niño , Humanos , Movimiento (Física) , Imagen por Resonancia Magnética/métodos , Encuestas y Cuestionarios , Examen Físico
2.
Gastroenterology ; 163(5): 1306-1320, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35872072

RESUMEN

BACKGROUND & AIMS: Cross-sectional imaging is important in the assessment of transmural inflammation in Crohn's disease (CD). Small bowel involvement is often more extensive in pediatric CD, requiring a panentering measuring tool. We undertook to develop a magnetic resonance enterography (MRE)-based index that would measure inflammation in all segments of the intestine, without rectal contrast. METHODS: Children with CD underwent ileocolonoscopy and MRE and half were prospectively followed for 18 months when MRE was repeated. Item generation and reduction were performed by a Delphi panel of pediatric radiologists, a systematic literature review, a cross-sectional study of 48 MREs, and a steering committee. Formatting and weighting were performed using multivariate modeling adjusted by a steering committee. MREs were read locally and centrally. Reliability, validity, and responsiveness were determined using several clinimetric and psychometric approaches. RESULTS: Thirty items were initially generated and reduced to 5 using regression analysis on 159 MREs: wall thickness, wall diffusion weighted imaging, ulcerations, mesenteric edema, and comb sign. In the validation cohort of 81 MREs, the weighted global PICMI correlated well with the radiologist global assessment (r = 0.85; P < .001) and with the simple endoscopic score in a subsample with ileocolonic disease (r = 0.63; P < .001). Interobserver and test-retest reliability were high (interclass correlation coefficients, 0.84; 95% confidence interval [CI], 0.79-0.87; and 0.81, 95% CI, 0.65-0.90, respectively; both P < .001). Excellent responsiveness was found at repeated visits (n = 116 MREs; area under the receiver operating characteristic curve 0.96; 95% CI, 0.93-0.99). Transmural healing was defined as PICMI ≤10 and response as a change of >20 points with excellent discriminative validity (area under the receiver operating characteristic curve = 0.96; 95% CI, 0.93-0.99). CONCLUSIONS: The PICMI is a valid, reliable, and responsive index for assessing transmural inflammation in pediatric CD. It scores the entire bowel length and does not require intravenous contrast or rectal enema and, therefore, is suitable for use in children. (ClinicalTrials.gov, Number: NCT01881490.).


Asunto(s)
Enfermedad de Crohn , Humanos , Niño , Enfermedad de Crohn/diagnóstico , Íleon/patología , Reproducibilidad de los Resultados , Estudios Transversales , Imagen por Resonancia Magnética/métodos , Inflamación , Espectroscopía de Resonancia Magnética
3.
Pediatr Radiol ; 53(7): 1420-1442, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35982340

RESUMEN

Whole-body MRI is increasingly used in the evaluation of a range of oncological and non-oncological diseases in infants, children and adolescents. Technical innovation in MRI scanners, coils and sequences have enabled whole-body MRI to be performed more rapidly, offering large field-of-view imaging suitable for multifocal and multisystem disease processes in a clinically useful timeframe. Together with a lack of ionizing radiation, this makes whole-body MRI especially attractive in the pediatric population. Indications include lesion detection in cancer predisposition syndrome surveillance and in the workup of children with known malignancies, and diagnosis and monitoring of a host of infectious and non-infectious inflammatory conditions. Choosing which patients are most likely to benefit from this technology is crucial, but so is adjusting protocols to the patient and disease to optimize lesion detection. The focus of this review is on protocols and the elements impacting image acquisition in pediatric whole-body MRI. We consider the practical aspects, from scanner and coil selection to patient positioning, single-center generic and indication-specific protocols with technical parameters, motion reduction strategies and post-processing. When optimized, collectively these lead to better standardization of whole-body MRI, and when married to systematic analysis and interpretation, they can improve diagnostic accuracy.


Asunto(s)
Imagen por Resonancia Magnética , Neoplasias , Lactante , Adolescente , Niño , Humanos , Imagen por Resonancia Magnética/métodos , Imagen de Cuerpo Entero/métodos , Espectroscopía de Resonancia Magnética
4.
J Magn Reson Imaging ; 55(6): 1696-1707, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35312203

RESUMEN

BACKGROUND: Evaluation of structural lung abnormalities with magnetic resonance imaging (MRI) has previously been shown to be predictive of clinical neonatal outcomes in preterm birth. MRI during free-breathing with phase-resolved functional lung (PREFUL) may allow for complimentary functional information without exogenous contrast. PURPOSE: To investigate the feasibility of structural and functional pulmonary MRI in a cohort of neonates and infants with no cardiorespiratory disease. Macrovascular pulmonary blood flows were also evaluated. STUDY TYPE: Prospective. POPULATION: Ten term infants with no clinically defined cardiorespiratory disease were imaged. Infants recruited from the general population and neonatal intensive care unit (NICU) were studied. FIELD STRENGTH/SEQUENCE: T1 -weighted VIBE, T2 -weighted BLADE uncorrected for motion. Ultrashort echo time (UTE) and 3D-flow data were acquired during free-breathing with self-navigation and retrospective reconstruction. Single slice 2D-gradient echo (GRE) images were acquired during free-breathing for PREFUL analysis. Imaging was performed at 3 T. ASSESSMENT: T1 , T2 , and UTE images were scored according to the modified Ochiai scheme by three pediatric body radiologists. Ventilation/perfusion-weighted maps were extracted from free-breathing GRE images using PREFUL analysis. Ventilation and perfusion defect percent (VDP, QDP) were calculated from the segmented ventilation and perfusion-weighted maps. Time-averaged cardiac blood velocities from three-dimensional-flow were evaluated in major pulmonary arteries and veins. STATISTICAL TEST: Intraclass correlation coefficient (ICC). RESULTS: The ICC of replicate structural scores was 0.81 (95% CI: 0.45-0.95) across three observers. Elevated Ochiai scores, VDP, and QDP were observed in two NICU participants. Excluding these participants, mean ± standard deviation structural scores were 1.2 ± 0.8, while VDP and QDP were 1.0% ± 1.1% and 0.4% ± 0.5%, respectively. Main pulmonary arterial blood flows normalized to body surface area were 3.15 ± 0.78 L/min/m2 . DATA CONCLUSION: Structural and functional pulmonary imaging is feasible using standard clinical MRI hardware (commercial whole-body 3 T scanner, table spine array, and flexible thoracic array) in free-breathing infants. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 1.


Asunto(s)
Nacimiento Prematuro , Niño , Estudios de Factibilidad , Femenino , Humanos , Imagenología Tridimensional , Recién Nacido , Pulmón , Imagen por Resonancia Magnética , Embarazo , Estudios Prospectivos , Estudios Retrospectivos
5.
AJR Am J Roentgenol ; 218(5): 781-792, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-34549607

RESUMEN

Fistulizing perianal disease, a frequent, chronic, and often debilitating manifestation of Crohn disease (CD) in adults and children, has been relatively refractory to treatment in the past. The advent of biologic agents such as anti-tumor necrosis factor-α and cellular therapies, used in conjunction with a range of surgical interventions, has greatly improved disease outcomes, although complete remission can still be elusive. This Special Series review considers current perianal imaging options, specifically pelvic MRI and endoanal and transperineal ultrasound, as well as their roles in the diagnosis, management, and assessment of treatment response. Pelvic MRI is the first-line modality for imaging perianal CD, given the complexity of fistulas encountered in CD. MRI technical acquisition parameters for adults and children and an approach to MRI interpretation and reporting are provided. Anatomic classification systems for fistulizing peri-anal disease are presented. We also explore the history, current landscape, and future developments of MRI features of perianal disease as imaging biomarkers, to quantify activity and severity and to consider CD MRI-based inflammatory activity scores. We discuss the reliability and validation of a number of indexes (including the pediatric MRI-based perianal CD [PEMPAC] index, the MR novel index for fistula imaging in CD [MAGNIFI-CD], the Van Assche index, and the modified Van Assche index), their potential to quantify treatment response, and possible prognostic capabilities.


Asunto(s)
Enfermedad de Crohn , Fístula Rectal , Adulto , Niño , Enfermedad de Crohn/diagnóstico por imagen , Enfermedad de Crohn/patología , Humanos , Inflamación , Imagen por Resonancia Magnética/métodos , Fístula Rectal/diagnóstico por imagen , Fístula Rectal/etiología , Reproducibilidad de los Resultados
6.
Pediatr Radiol ; 52(2): 374-381, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33830290

RESUMEN

Given the increasing use of MRI in the pediatric population, the need for sedation in MRI performed in young children is a topic of growing importance. Although sedation is generally tolerated well by children, the financial and operational impacts of anesthesia on MRI workflow, as well as potential adverse effects of anesthetic medications, highlight the need to perform MRI in children without sedation whenever possible. This review focuses on current techniques to facilitate non-sedation MRI in children, including exam preparation with MRI simulation; asleep but not sedated techniques; awake and relaxed techniques using certified child life specialists, animal-assisted therapy, a child-friendly environment and in-scan entertainment; and non-sedated MRI protocol modifications such as shorter scan time, prioritizing sequences, reducing motion artifact, noise reduction, limiting use of gadolinium, employing an open MRI and modifying protocols.


Asunto(s)
Anestesia , Terapia Asistida por Animales , Artefactos , Niño , Preescolar , Sedación Consciente , Gadolinio , Humanos , Imagen por Resonancia Magnética
7.
Pediatr Radiol ; 52(2): 401-417, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33791839

RESUMEN

Pediatric patients with cancer predisposition syndromes are at increased risk of developing malignancies compared with their age-matched peers, necessitating regular surveillance. Screening protocols differ among syndromes and are composed of a number of elements, imaging being one. Surveillance can be initiated in infants, children and adolescents with a tumor known or suspected of being related to a cancer predisposition syndrome or where genetic testing identifies a germline pathogenic gene variant in an asymptomatic child. Pre-symptomatic detection of malignant neoplasms offers potential to improve treatment options and survival outcomes, but the benefits and risks of screening need to be weighed, particularly with variable penetrance in many cancer predisposition syndromes. In this review we discuss the benefits and risks of surveillance imaging and the importance of integrating imaging and non-imaging screening elements. We explore the principles of surveillance imaging with particular reference to whole-body MRI, considering the strategies to minimize false-negative and manage false-positive whole-body MRI results, the value of standardized nomenclature when reporting risk stratification to better guide patient management, and the need for timely communication of results to allay anxiety. Cancer predisposition syndrome screening is a multimodality, multidisciplinary and longitudinal process, so developing formalized frameworks for surveillance imaging programs should enhance diagnostic performance while improving the patient experience.


Asunto(s)
Detección Precoz del Cáncer , Neoplasias , Adolescente , Niño , Predisposición Genética a la Enfermedad , Humanos , Lactante , Imagen por Resonancia Magnética , Neoplasias/diagnóstico por imagen , Neoplasias/genética , Síndrome
8.
Pediatr Radiol ; 52(7): 1283-1295, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35391548

RESUMEN

BACKGROUND: The Toronto protocol for cancer surveillance in children with Li-Fraumeni syndrome has been adopted worldwide. OBJECTIVE: To assess the diagnostic accuracy of the imaging used in this protocol. MATERIALS AND METHODS: We conducted a blinded retrospective review of imaging modalities in 31 pediatric patients. We compared imaging findings with the reference standards, which consisted of (1) histopathological diagnosis, (2) corresponding dedicated imaging or subsequent surveillance imaging or (3) clinical outcomes. We individually analyzed each modality's diagnostic performance for cancer detection and assessed it on a per-study basis for chest and abdominal regional whole-body MRI (n=115 each), brain MRI (n=101) and abdominal/pelvic US (n=292), and on a per-lesion basis for skeleton/soft tissues on whole-body MRI (n=140). RESULTS: Of 763 studies/lesions, approximately 80% had reference standards that identified 4 (0.7%) true-positive, 523 (85.3%) true-negative, 5 (0.8%) false-positive, 3 (0.5%) false-negative and 78 (12.7%) indeterminate results. There were 3 true-positives on whole-body MRI and 1 true-positive on brain MRI as well as 3 false-negatives on whole-body MRI. Sensitivities and specificities of tumor diagnosis using a worst-case scenario analysis were, respectively, 40.0% (95% confidence interval [CI]: 7.3%, 83.0%) and 38.2% (95% CI: 29.2%, 48.0%) for skeleton/soft tissues on whole-body MRI; sensitivity non-available and 97.8% (95% CI: 91.4%, 99.6%) for chest regional whole-body MRI; 100.0% (95% CI: 5.5%, 100.0%) and 96.8% (95% CI: 90.2%, 99.2%) for abdominal regional whole-body MRI; sensitivity non-available and 98.3% (95% CI: 95.3, 99.4) for abdominal/pelvic US; and 50.0% (95% CI: 2.7%, 97.3%) and 93.8% (95% CI: 85.6%, 97.7%) for brain MRI. CONCLUSION: Considerations for optimizing imaging protocol, defining criteria for abnormalities, developing a structured reporting system, and practicing consensus double-reading may enhance the diagnostic accuracy for tumor surveillance.


Asunto(s)
Síndrome de Li-Fraumeni , Niño , Detección Precoz del Cáncer/métodos , Humanos , Síndrome de Li-Fraumeni/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Radiofármacos , Sensibilidad y Especificidad
9.
Eur Radiol ; 31(3): 1494-1504, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32880696

RESUMEN

OBJECTIVES: To assess the concordance of whole-body MRI (WB-MRI) and an FDG-PET/CT-based reference standard for the initial staging in children with Hodgkin lymphoma (HL) METHODS: Children with newly diagnosed HL were included in this prospective, multicentre, international study and underwent WB-MRI and FDG-PET/CT at staging. Two radiologists and a nuclear medicine physician independently evaluated all images. Discrepancies between WB-MRI and FDG-PET/CT were assessed by an expert panel. All FDG-PET/CT errors were corrected to derive the FDG-PET/CT-based reference standard. The expert panel corrected all reader errors in the WB-MRI DWI dataset to form the intrinsic MRI data. Inter-observer agreement for WB-MRI DWI was calculated using overall agreement, specific agreements and kappa statistics. Concordance for correct classification of all disease sites and disease stage between WB-MRI (without DWI, with DWI and intrinsic WB-MRI DWI) and the reference standard was calculated as primary outcome. Secondary outcomes included positive predictive value, negative predictive value and kappa statistics. Clustering within patients was accounted for using a mixed-effect logistic regression model with random intercepts and a multilevel kappa analysis. RESULTS: Sixty-eight children were included. Inter-observer agreement between WB-MRI DWI readers was good for disease stage (κ = 0.74). WB-MRI DWI agreed with the FDG-PET/CT-based reference standard for determining disease stage in 96% of the patients versus 88% for WB-MRI without DWI. Agreement between WB-MRI DWI and the reference standard was excellent for both nodal (98%) and extra-nodal (100%) staging. CONCLUSIONS: WB-MRI DWI showed excellent agreement with the FDG-PET/CT-based reference standard. The addition of DWI to the WB-MRI protocol improved the staging agreement. KEY POINTS: • This study showed excellent agreement between WB-MRI DWI and an FDG-PET/CT-based reference standard for staging paediatric HL. • Diffusion-weighted imaging is a useful addition to WB-MRI in staging paediatric HL. • Inter-observer agreement for WB-MRI DWI was good for both nodal and extra-nodal staging and determining disease stage.


Asunto(s)
Fluorodesoxiglucosa F18 , Enfermedad de Hodgkin , Niño , Imagen de Difusión por Resonancia Magnética , Enfermedad de Hodgkin/diagnóstico por imagen , Enfermedad de Hodgkin/patología , Humanos , Imagen por Resonancia Magnética , Estadificación de Neoplasias , Tomografía Computarizada por Tomografía de Emisión de Positrones , Estudios Prospectivos , Estándares de Referencia , Imagen de Cuerpo Entero
10.
Pediatr Radiol ; 51(5): 724-735, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33860861

RESUMEN

The use of sedation and general anesthesia has facilitated the significant growth of MRI use among children over the last years. While sedation and general anesthesia are considered to be relatively safe, their use poses potential risks in the short term and in the long term. This manuscript reviews the reasons why MRI examinations require sedation and general anesthesia more commonly in the pediatric population, summarizes the safety profile of sedation and general anesthesia, and discusses an amalgam of strategies that can be implemented and can ultimately lead to the optimization of sedation and general anesthesia care within pediatric radiology departments.


Asunto(s)
Anestesia General , Servicio de Radiología en Hospital , Anestesia General/efectos adversos , Niño , Sedación Consciente/efectos adversos , Humanos , Imagen por Resonancia Magnética
11.
Pediatr Radiol ; 51(12): 2229-2252, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34431006

RESUMEN

Gray-scale and color/power Doppler ultrasound (US) are the first-line imaging modalities to evaluate the spleen, gallbladder and pancreas in children. The increasing use of contrast-enhanced ultrasound (CEUS) as a reliable and safe method to evaluate liver lesions in the pediatric population promises potential for imaging other internal organs. Although CEUS applications of the spleen, gallbladder and pancreas have been well described in adults, they have not been fully explored in children. In this manuscript, we present an overview of the applications of CEUS for normal variants and diseases affecting the spleen, gallbladder and pancreas. We highlight a variety of cases as examples of how CEUS can serve in the diagnosis and follow-up for such diseases in children. Our discussion includes specific examination techniques; presentation of the main imaging findings in various benign and malignant lesions of the spleen, gallbladder and pancreas in children; and acknowledgment of the limitations of CEUS for these organs.


Asunto(s)
Vesícula Biliar , Bazo , Adulto , Niño , Medios de Contraste , Vesícula Biliar/diagnóstico por imagen , Humanos , Páncreas/diagnóstico por imagen , Bazo/diagnóstico por imagen , Ultrasonografía
12.
Pediatr Radiol ; 51(5): 698-708, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33772641

RESUMEN

Quality in MR imaging is a comprehensive process that encompasses scanner performance, clinical processes for efficient scanning and reporting, as well as data-driven improvement involving measurement of key performance indicators. In this paper, the authors review this entire process. This article provides a framework for establishing a successful MR quality program. The collective experiences of the authors across a spectrum of pediatric hospitals is summarized here.


Asunto(s)
Garantía de la Calidad de Atención de Salud , Mejoramiento de la Calidad , Niño , Humanos , Imagen por Resonancia Magnética , Control de Calidad
13.
Ophthalmology ; 127(11): 1549-1557, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32422154

RESUMEN

PURPOSE: To generate recommendations for long-term follow-up of adult survivors of heritable retinoblastoma. DESIGN: We convened a meeting of providers from retinoblastoma centers around the world to review the state of the science and to evaluate the published evidence. PARTICIPANTS: Retinoblastoma is a rare childhood cancer of the retina. Approximately 40% of retinoblastoma cases are heritable, resulting from a germline mutation in RB1. Dramatic improvements in treatment and supportive care have resulted in a growing adult survivor population. However, survivors of heritable retinoblastoma have a significantly increased risk of subsequent malignant neoplasms, particularly bone and soft tissue sarcomas, uterine leiomyosarcoma, melanomas, and radiotherapy-related central nervous system tumors, which are associated with excess morbidity and mortality. Despite these risks, no surveillance recommendations for this population currently are in place, and surveillance practices vary widely by center. METHODS: Following the Institute of Medicine procedure for clinical practice guideline development, a PubMed, EMBASE, and Web of Science search was performed, resulting in 139 articles; after abstract and full-text review, 37 articles underwent detailed data abstraction to quantify risk and evidence regarding surveillance, if available. During an in-person meeting, evidence was presented and discussed, resulting in consensus recommendations. MAIN OUTCOME MEASURES: Diagnosis and mortality from subsequent neoplasm. RESULTS: Although evidence for risk of subsequent neoplasm, especially sarcoma and melanoma, was significant, evidence supporting routine testing of asymptomatic survivors was not identified. Skin examination for melanoma and prompt evaluation of signs and symptoms of head and neck disease were determined to be prudent. CONCLUSIONS: This review of the literature confirmed some of the common second cancers in retinoblastoma survivors but found little evidence for a benefit from currently available surveillance for these malignancies. Future research should incorporate international partners, patients, and family members.


Asunto(s)
Predisposición Genética a la Enfermedad , Guías como Asunto , Neoplasias de la Retina/diagnóstico , Retinoblastoma/diagnóstico , Medición de Riesgo , Estudios de Seguimiento , Salud Global , Humanos , Incidencia , Neoplasias de la Retina/epidemiología , Neoplasias de la Retina/genética , Retinoblastoma/epidemiología , Retinoblastoma/genética , Factores de Riesgo
14.
Am J Med Genet A ; 182(6): 1496-1499, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32207556

RESUMEN

GATA6 pathogenic variants primarily manifest a phenotype with pancreatic agenesis and cardiac malformations. However, additional congenital malformations affecting the biliary system, congenital diaphragmatic hernia and developmental delay have been reported. We report a newborn, prenatally diagnosed with truncus arteriosus and intrauterine growth restriction, who was postnatally found to have pancreatic agenesis associated with neonatal diabetes and hepatobiliary abnormalities. Whole exome sequencing identified a de novo, heterozygous mutation in the GATA6 gene (c.1366C>T; p.Arg456Cys). Further investigations revealed abnormalities not previously associated with GATA6 mutation, including unilateral thyroid lobe agenesis associated with congenital hypothyroidism, absent gall bladder, possible adrenal insufficiency, thrombocytopenia, and neonatal stroke.


Asunto(s)
Hipotiroidismo Congénito/genética , Factor de Transcripción GATA6/genética , Cardiopatías Congénitas/genética , Páncreas/anomalías , Enfermedades Pancreáticas/congénito , Hipotiroidismo Congénito/complicaciones , Hipotiroidismo Congénito/patología , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/patología , Heterocigoto , Humanos , Lactante , Recién Nacido , Masculino , Mutación , Páncreas/patología , Enfermedades Pancreáticas/complicaciones , Enfermedades Pancreáticas/genética , Enfermedades Pancreáticas/patología , Fenotipo , Secuenciación del Exoma
15.
AJR Am J Roentgenol ; 215(4): 1002-1011, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-32809862

RESUMEN

OBJECTIVE. Whole-body MRI is a valuable tool in the surveillance of cancer predisposition syndromes (CPSs). Because it allows wide-FOV imaging without ionizing radiation, whole-body MRI is ideal in pediatric patients, enabling efficient assessment of different organ systems for multifocal disease. This article summarizes the use of whole-body MRI in pediatric patients with CPSs for earlier detection of malignancy, provides evidence where available, and offers guidance where lacking because of the rarity of CPSs. Protocol modifications and technique performance in specific CPSs are also considered. CONCLUSION. Whole-body MRI is the preferred imaging modality for surveillance of pediatric patients with CPSs, and the growing literature supports its importance in presymptomatic cancer detection.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Carcinoma Adenoide Quístico/diagnóstico por imagen , Síndrome de Li-Fraumeni/diagnóstico por imagen , Neoplasias Pulmonares/diagnóstico por imagen , Imagen por Resonancia Magnética , Imagen de Cuerpo Entero , Femenino , Predisposición Genética a la Enfermedad , Humanos , Lactante , Guías de Práctica Clínica como Asunto
17.
Radiographics ; 39(2): 516-534, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30681900

RESUMEN

Imaging is fundamental to diagnosis and management of pediatric patients with cancer and cancer predisposition syndromes (CPSs). Whole-body MRI has emerged as a versatile tool for pediatric oncologic imaging, with the potential to spare children from ionizing radiation imparted by conventional modalities such as CT and PET. Whole-body MRI also enables simultaneous high-resolution local-regional staging and wide field-of-view distant staging in the same imaging session, with superior evaluation of the brain, spine, liver, and marrow. Recent technical advances have reduced imaging times and enhanced image quality, with continued advances on the near horizon. Pulse sequences such as whole-body diffusion-weighted imaging have also broadened the range of diagnostic information obtainable. In addition, increasing identification of children with CPSs has compelled efforts to establish surveillance imaging strategies for affected individuals, with whole-body MRI playing a pivotal role in screening algorithms for several CPSs. In light of these emerging trends, a working knowledge of oncologic whole-body MRI applications and evolving CPS surveillance algorithms is vital to providers who participate in the care of pediatric patients affected by or predisposed to cancer. Recognizing both the strengths and limitations of whole-body MRI not only enables more thoughtful implementation but also improves the accuracy of image interpretation. ©RSNA, 2019 See discussion on this article by Khanna .


Asunto(s)
Imagen por Resonancia Magnética/métodos , Neoplasias/diagnóstico por imagen , Síndromes Neoplásicos Hereditarios/diagnóstico por imagen , Imagen de Cuerpo Entero/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Síndrome de Li-Fraumeni/diagnóstico por imagen , Masculino , Oncología Médica , Estadificación de Neoplasias/métodos , Neurofibromatosis/diagnóstico por imagen , Paraganglioma/diagnóstico por imagen , Pediatría , Feocromocitoma/diagnóstico por imagen
18.
Pediatr Radiol ; 49(9): 1201-1208, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-31203404

RESUMEN

BACKGROUND: Concern regarding gadolinium deposition in the brain after repeated administration of intravenous gadolinium-based contrast agents has prompted evaluation of imaging alternatives. OBJECTIVE: The study purpose was to determine if magnetic resonance imaging (MRI) using conventional sequences with diffusion-weighted imaging (DWI) instead of gadolinium-based contrast-enhanced MRI is valid for local staging and guiding biopsies in osseous sarcomas. MATERIALS AND METHODS: Initial pretreatment MRI with DWI and gadolinium-based contrast-enhanced images in patients ≤ 18 years with histopathologically proven osseous sarcomas were included. Two radiologists blinded to collated demographic and clinical data, independently reviewed conventional/DWI and conventional/gadolinium-based contrast-enhanced MRI then conventional sequences alone, recording tumor size, skip lesions, necrosis, neurovascular invasion, enlarged lymph nodes and diffusion restriction. Discrepancies were resolved by a third reader. A single reader measured apparent diffusion coefficient (ADC) values in non-necrotic tumors, then correlated minimum ADC values -- with and without normalization to skeletal muscle -- with relative enhancement. RESULTS: Twenty-one patients (mean age: 11.3±4.2 years, 15 [71%] females) had 14 osteosarcomas and 7 Ewing sarcomas, 50% centered in the femur. Conventional/DWI versus conventional/gadolinium-based contrast-enhanced MRI showed agreement for tumor size estimation with significant associations for necrosis (P=0.021), neurovascular involvement (P<0.001) and enlarged lymph nodes (P=0.005). Diagnostic accuracy of conventional/DWI is comparable to conventional/gadolinium-based contrast-enhanced MRI and superior to conventional sequences alone. Comparison between minimum ADC values and relative enhancement showed no correlation (P>0.05). CONCLUSION: Significant associations of key imaging features in the initial assessment of osseous sarcomas support DWI as an alternative to gadolinium-based contrast-enhanced MRI. The lack of association between ADC values and relative enhancement suggests that they measure independent constructs, DWI dependent upon tumor cellularity and perfusion.


Asunto(s)
Neoplasias Óseas/diagnóstico por imagen , Condrosarcoma/diagnóstico por imagen , Imagen de Difusión por Resonancia Magnética/métodos , Osteosarcoma/diagnóstico por imagen , Adolescente , Niño , Medios de Contraste , Femenino , Humanos , Masculino , Estudios Retrospectivos
19.
Pediatr Radiol ; 49(8): 1000-1009, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31030334

RESUMEN

BACKGROUND: Children undergoing magnetic resonance imaging (MRI) can experience negative emotions both before and during their scan, causing them to move and often necessitating the use of procedural sedation. Several strategies to improve patient compliance have been attempted. OBJECTIVE: This study was designed to evaluate the effectiveness of a non-pharmacological intervention to reduce anxiety in pediatric patients preparing for MRI using animal-assisted therapy. MATERIALS AND METHODS: An animal intervention pilot study was performed in patients who agreed in advance to interact with a dog. Patients and caregivers filled out questionnaires, including questions designed to capture changes in patient emotion before and after the intervention. MRI diagnostic quality was compared to age- and gender-matched control groups with and without general anesthesia. RESULTS: The intervention in 21 patients comparing pre- and post-scan surveys demonstrated a statistically significant improvement in patient anxiety levels (P<0.01). Diagnostic MRI scans were achieved in 19/21 (90%), with no significant difference in exam quality or times compared against control groups. The majority of caregivers and staff members agreed strongly that patients benefited from the therapy dog's presence. CONCLUSION: The use of animal-assisted therapy in a pilot group in our MRI division resulted in a beneficial effect on patients' emotional status, easing anxiety in preparation for scheduled scans, without impacting MRI quality or duration. Further randomized studies will be needed to demonstrate its significance in reducing sedation rates in children undergoing MRI.


Asunto(s)
Terapia Asistida por Animales/métodos , Ansiedad/prevención & control , Imagen por Resonancia Magnética/métodos , Adolescente , Animales , Niño , Preescolar , Perros , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética/psicología , Seguridad del Paciente , Proyectos Piloto , Mejoramiento de la Calidad , Estudios Retrospectivos , Medición de Riesgo , Encuestas y Cuestionarios
20.
J Magn Reson Imaging ; 47(6): 1638-1645, 2018 06.
Artículo en Inglés | MEDLINE | ID: mdl-29135086

RESUMEN

BACKGROUND: Pediatric Crohn's disease is associated with perianal disease (PAD). Magnetic resonance enterography (MRE) assesses small bowel involvement in pediatric inflammatory bowel disease (PIBD). Pelvic MRI (P-MRI) is the gold standard for assessing PAD. PURPOSE: To determine if MRE can accurately detect PAD in PIBD, distinguishing perianal fistulae (PAF) from perianal abscesses (PAA), referenced against P-MRI. STUDY TYPE: Retrospective. POPULATION: Seventy-seven PIBD patients, 27 females (mean age 14.1 years), with P-MRI and MRE within 6 months. FIELD STRENGTH/SEQUENCE: 1.5T and 3T; P-MRI: sagittal fat suppressed (FS) T2 fast spin-echo (FSE), coronal short tau inversion recovery, axial T1 FSE, coronal and axial postcontrast FS T1 FSE; MRE: coronal balanced steady-state free-precession (SSFP), coronal cine SSFP, coronal and axial single-shot T2 FS, axial SSFP, coronal ultrafast 3D T1 -weighted gradient echo FS (3D T1 GE), axial diffusion-weighted imaging, coronal and axial postcontrast 3D T1 GE FS. ASSESSMENT: Two radiologists independently, then by consensus, assessed randomized MRI exams, recording PAF number, location, and length; and PAA number, location, length, and volume. Sensitivity analysis used clinical disease as the gold standard, calculated separately for P-MRI and MRE. STATISTICAL TESTS: Comparing MRE and P-MRI consensus data, sensitivity, specificity, positive, and negative predictive values (P/NPV) were calculated. Inter- and intrareader reliability were assessed using kappa statistics. RESULTS: P-MRI and MRE were paired, detecting PAD in 73 patients, PAF in 63, and PAA in 31 P-MRI. MRE sensitivities, specificities, PPV, and NPV were: PAD 82%, 100%, 100%, 23%; PAF 74%, 71%, 92%, 38%; PAA 51%, 85%, 69%, 72%; clinical 82%, 22%, 37%, 69%; clinical P-MRI 96%, 8%, 37%, 80%. MRE interreader agreement for PAD was moderate (kappa = 0.51 [0.29-0.73]), fair for PAF and PAA. DATA CONCLUSION: Using a standard technique, MRE can detect PAD with high specificity and moderate sensitivity in PIBD, missing some PAF and small PAA. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1638-1645.


Asunto(s)
Enfermedades del Ano/diagnóstico por imagen , Colitis Ulcerosa/diagnóstico por imagen , Enfermedad de Crohn/diagnóstico por imagen , Imagen por Resonancia Magnética , Absceso , Adolescente , Niño , Preescolar , Femenino , Humanos , Inflamación , Masculino , Variaciones Dependientes del Observador , Pelvis/diagnóstico por imagen , Radiología , Reproducibilidad de los Resultados
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