Inflammatory Myofibroblastic Tumor of the Kidney and Bilateral Lung Nodules in a Child Mimicking Wilms Tumor With Lung Metastases.

Renal inflammatory myofibroblastic tumor (IMT) is an extremely rare lesion especially in children. This report describes a case of renal IMT accompanied by multiple lung nodules mimicking Wilms tumor with lung metastasis in a 3-year-old boy. To our knowledge, this is a unique case of IMT which has not been reported in the literature previously.

Evaluation of pediatric thoracic disorders: comparison of unenhanced fast-imaging-sequence 1.5-T MRI and contrast-enhanced MDCT.

OBJECTIVE: The purpose of this study was to investigate the efficacy of thoracic MRI with fast imaging sequences without contrast administration at 1.5 T for evaluating thoracic abnormalities by comparing MRI findings with contrast-enhanced MDCT findings. SUBJECTS AND METHODS: A prospective study included consecutively registered pediatric patients who from December 2009 to January 2012 underwent thoracic MDCT followed within 2 days by MRI for evaluation of thoracic abnormalities. The final study sample consisted of 71 children (36 boys, 35 girls; mean age, 8.6 ± 4.5 years; range, 2 months-16 years) and 71 paired thoracic MRI and MDCT studies. Thoracic MRI was performed in the axial and coronal planes with the following fast imaging sequences: T1-weighted fast-field echo inversion prepulse, T2-weighted balanced fast-field echo multiple 2D, T1- and T2-weighted turbo spin-echo cardiac-triggering parallel imaging technique without cardiac monitoring, and STIR. Thoracic MDCT was performed with i.v. contrast administration. Two pediatric radiologists independently reviewed each MRI and MDCT study for abnormalities in the lung, large airways, and mediastinal, pleural, and musculoskeletal structures. The sensitivity, specificity, and overall accuracy of MRI were calculated. Interobserver agreement was measured with the kappa coefficient. RESULTS: With MDCT as the reference standard, 51 of 71 (72%) patients had abnormal findings on MDCT studies, including infections in 21 (42%) cases, neoplasms in 19 (37%) cases, interstitial lung disease in seven (14%) cases, pleural effusion in three (6%) cases, and congenital bronchogenic cyst in one (2%) case. The overall diagnostic accuracy, sensitivity, and specificity of MRI for detecting thoracic abnormalities were 69 of 71 (97%), 49 of 51 (96%), and 20 of 20 (100%). Two undiagnosed findings with MRI that were detected with MDCT were mild bronchiectasis and small pulmonary nodule (3 mm). Almost perfect interobserver agreement was found between two reviewers with 70 of 71 agreements (κ = 0.97; 95% CI, 0.92-1.00; p < 0.001). CONCLUSION; MRI with fast imaging sequences without contrast administration is comparable to contrast-enhanced MDCT for detecting thoracic abnormalities in pediatric patients. Use of MRI with fast imaging sequences without contrast administration as a first-line cross-sectional imaging study in lieu of contrast-enhanced MDCT has the potential to benefit this patient population owing to reduced radiation exposure and i.v. contrast administration.

Magnetic resonance enterography in pediatric celiac disease.

OBJECTIVE: To assess if magnetic resonance enterography is capable of showing evidence/extent of disease in pediatric patients with biopsy-proven celiac disease by comparing with a control group, and to correlate the magnetic resonance enterography findings with anti-endomysial antibody level, which is an indicator of gluten-free dietary compliance. METHODS: Thirty-one pediatric patients (mean age 11.7±3.1 years) with biopsy-proven celiac disease and 40 pediatric patients as a control group were recruited in the study. The magnetic resonance enterography images of both patients with celiac disease and those of the control group were evaluated by two pediatric radiologists in a blinded manner for the mucosal pattern, presence of wall thickening, luminal distention of the small bowel, and extra-intestinal findings. Patient charts were reviewed to note clinical features and laboratory findings. The histopathologic review of the duodenal biopsies was re-conducted. RESULTS: The mean duration of the disease was 5.6±1.8 years (range: 3-7.2 years). In 24 (77%) of the patients, anti-endomysial antibody levels were elevated (mean 119.2±66.6RU/mL). Magnetic resonance enterography revealed normal fold pattern in all the patients. Ten (32%) patients had enlarged mesenteric lymph nodes. CONCLUSION: Although a majority of the patients had elevated anti-endomysial antibody levels indicating poor dietary compliance, magnetic resonance enterography did not show any mucosal abnormality associated with the inability of magnetic resonance enterography to detect mild/early changes of celiac disease in children. Therefore, it may not be useful for the follow-up of pediatric celiac disease.

Susceptibility-Based Differentiation of Intracranial Calcification and Hemorrhage in Pediatric Patients.

Differential diagnosis of intracranial hemorrhage versus calcification on conventional magnetic resonance images (MRIs) is often challenging. Although computed tomography (CT) confirms calcification, phase information obtained during susceptibility-weighted imaging can be useful in distinguishing between 2 pathologies. Fourteen patients previously diagnosed to have hemorrhage or calcification with imaging were included in the study retrospectively. Phase shift values of hemorrhage and calcification were compared by using Student t test. The pathologies identified were tuberous sclerosis, Sturge-Weber syndrome, craniopharyngioma, congenital cytomegalovirus, subependymal hemorrhages, and hemorrhagic microembolic infarction. Calcifications appeared hypointense whereas hemorrhages were hyperintense on phase maps (left-handed magnetic resonance system). Statistical comparison of phase shift values yielded significant difference between hemorrhage versus calcification (P < .01). Phase maps were found to offer valuable data to differentiate 2 pathologies when used complementary to conventional magnetic resonance images. Considering the relatively higher risks of radiation exposure in children, susceptibility-weighted imaging with phase maps may help to waive radiation exposure from CT.

Magnetic resonance enterography in pediatric celiac disease / A enterografia por ressonância magnética na doença celíaca pediátrica

Abstract Objective: To assess if magnetic resonance enterography is capable of showing evidence/extent of disease in pediatric patients with biopsy-proven celiac disease by comparing with a control group, and to correlate the magnetic resonance enterography findings with anti-endomysial antibody level, which is an indicator of gluten-free dietary compliance. Methods: Thirty-one pediatric patients (mean age 11.7 ± 3.1 years) with biopsy-proven celiac disease and 40 pediatric patients as a control group were recruited in the study. The magnetic resonance enterography images of both patients with celiac disease and those of the control group were evaluated by two pediatric radiologists in a blinded manner for the mucosal pattern, presence of wall thickening, luminal distention of the small bowel, and extra-intestinal findings. Patient charts were reviewed to note clinical features and laboratory findings. The histopathologic review of the duodenal biopsies was re-conducted. Results: The mean duration of the disease was 5.6 ± 1.8 years (range: 3-7.2 years). In 24 (77%) of the patients, anti-endomysial antibody levels were elevated (mean 119.2 ± 66.6 RU/mL). Magnetic resonance enterography revealed normal fold pattern in all the patients. Ten (32%) patients had enlarged mesenteric lymph nodes. Conclusion: Although a majority of the patients had elevated anti-endomysial antibody levels indicating poor dietary compliance, magnetic resonance enterography did not show any mucosal abnormality associated with the inability of magnetic resonance enterography to detect mild/early changes of celiac disease in children. Therefore, it may not be useful for the follow-up of pediatric celiac disease.

Resumo Objetivo: Avaliar se a enterografia por ressonância magnética (ERM) consegue comprovar/mostrar a extensão da doença em pacientes pediátricos com doença celíaca (DC) comprovada por biópsia, comparar com um grupo de controle e correlacionar os achados da ERM com o nível de anticorpo antiendomísio (EMA) indicador de dieta sem glúten. Métodos: Foram recrutados 31 pacientes pediátricos (idade média entre 11,7 ± 3,1 anos) com DC comprovada por biópsia e 40 pacientes pediátricos em um grupo de controle. As imagens da ERM dos pacientes com DC e no grupo de controle foram avaliadas por dois radiologistas pediátricos às cegas para o padrão da mucosa, presença de espessamento da parede, dilatação luminal do intestino delgado e achados extraintestinais. Os prontuários dos pacientes foram revisados para anotação de características clínicas e achados laboratoriais. A avaliação histopatológica das biópsias duodenais foi feita novamente. Resultados: A duração média da doença foi 5,6 ± 1,8 anos (faixa de 3-7,2 anos). Em 24 (77%) dos pacientes, os níveis EMA estavam elevados (média 119,2 ± 66,6 RU/mL). A ERM revelou um padrão de pregas normal em todos os pacientes; 10 (32%) dos pacientes apresentaram gânglios linfáticos mesentéricos aumentados. Conclusão: Apesar de a maioria dos pacientes ter níveis elevados de EMA, o que indica uma dieta pobre, a ERM não mostrou anomalia na mucosa associada à incapacidade de a ERM detectar alterações leves/precoces de DC nas crianças. Portanto, ela pode não ser útil no acompanhamento da DC pediátrica.