Seminars in ultrasound, CT, and MR
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Intestinal malrotation is a congenital abnormal fixation of the bowel that predisposes to life threatening midgut volvulus. Most events of midgut volvulus occur in the first month of life with decreased risk with age. ⋯ For optimal results, the study should be well planned with meticulous attention to technique. In equivocal cases, small bowel follow-through study and ultrasound can be performed.
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Semin. Ultrasound CT MR · Aug 2014
ReviewImaging of lumps and bumps in pediatric patients: an algorithm for appropriate imaging and pictorial review.
Superficial lumps and bumps are a common presenting complaint in the pediatric patient population. Although encountered frequently, the path to a definitive diagnosis is not always a straightforward one. ⋯ This will not only allow the radiologist to serve as a valuable asset to the treating physician in choosing the most appropriate imaging modality but also help in accurate diagnosis, all while ensuring the "image gently" principle. An algorithm for imaging in the pediatric patient with lumps and bumps has been presented in this article and a few example entities along with their imaging findings have also been reviewed.
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Semin. Ultrasound CT MR · Apr 2014
ReviewCongenital genetic inborn errors of metabolism presenting as an adult or persisting into adulthood: neuroimaging in the more common or recognizable disorders.
Numerous congenital-genetic inborn errors of metabolism (CIEMs) have been identified and characterized in detail within recent decades, with promising therapeutic options. Neuroimaging is becoming increasingly utilized in earlier stages of CIEMs, and even in asymptomatic relatives of patients with a CIEM, so as to monitor disease progress and treatment response. This review attempts to summarize in a concise fashion the neuroimaging findings of various CIEMs that may present in adulthood, as well as those that may persist into adulthood, whether because of beneficial therapy or a delay in diagnosis. ⋯ The focus of this review is their appearance on routine magnetic resonance imaging sequences, with some basic attention to the findings of such CIEMs on specialized neuroimaging, based on recent or preliminary research. The general classes of disorders covered in this complex review are: peroxisomal disorders (adrenoleukodystrophy), lysosomal storage disorders (including metachromatic leukodystrophy, Krabbe or globoid cell leukodystrophy, Fabry, Niemann-Pick, GM1, GM2, Gaucher, mucopolysaccharidoses, and Salla diseases), mitochondrial disorders (including mitochondrial encephalomyopathy with lactic acidosis and strokelike episodes, myoclonic epilepsy with ragged red fibers, Leigh disease, and Kearns-Sayre syndrome), urea cycle disorders, several organic acidemias (including phenylketonuria, maple syrup urine disease, 3-hydroxy-3-methylglutaryl colyase deficiency, glutaric acidurias, methylmalonic academia, proprionic academia, 3-methylglucatonic aciduria, and 2-hydroxyglutaric acidurias), cytoskeletal or transporter molecule defects (including Alexander or fibrinoid leukodystrophy, proteolipid protein-1 defect or Pelizaeus Merzbacher, Wilson, and Huntington diseases), and several neurodegenerative disorders of brain iron accumulation. Additionally, an arbitrary "miscellaneous" category of 5 recognizable disorders that may present in or persist into adulthood is summarized, which include megalencephalic leukoencephalopathy with subcortical cysts (megancephalic leukoencephalopathy with subcortical cysts or van der Knaap disease), polymerase-III gene defect ("4H syndrome"), childhood ataxia with central nervous system hypomyelination ("vanishing white matter disease"), striopallidodentate calcinosis ("Fahr disease"), and Cockayne syndrome.
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Semin. Ultrasound CT MR · Feb 2014
ReviewChronic hypersensitivity pneumonitis and pulmonary sarcoidosis: differentiation from usual interstitial pneumonia using high-resolution computed tomography.
The distinction of chronic hypersensitivity pneumonitis (HP) or advanced-stage sarcoidosis from idiopathic pulmonary fibrosis or usual interstitial pneumonia is important because each disease is managed differently and may have a different prognosis. The analyses of pattern and distribution of lung parenchymal abnormalities on high-resolution computed tomography scans help differentiate among the 3 diseases. In chronic HP, the presence of lobular areas of decreased attenuation and centrilobular small nodules and the absence of lower lung zone predominance are characteristically observed. ⋯ Lung bases are usually spared. In idiopathic pulmonary fibrosis or usual interstitial pneumonia, however, the presence of honeycombing with lower lung zone predominance and the absence of centrilobular small nodules are important findings that allow us to differentiate the disease from chronic HP or advanced-stage sarcoidosis. In the 3 diseases, most important prognosis-predicting factor is the extent of fibrotic score (the extent of honeycombing and reticulation) calculated on high-resolution computed tomography scans or fibrosis estimated on chest radiographs.