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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European radiology 9 (1999), S. 1348-1353 
    ISSN: 1432-1084
    Keywords: Key words: Currarino triad ; Ultrasonography ; MR imaging ; Constipation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The aim of this study was to evaluate the spectrum of findings and the efficacy of different imaging modalities in order to formulate recommendations for diagnostic imaging of Currarino triad (ASP syndrome), including screening of relatives. The imaging films of five female patients (age range 6 weeks to 12 months) were analysed retrospectively. The studied material consisted of US and MRI of the lower spine (5 patients each), lumbosacral plain radiography (4 patients), contrast enema (4 patients), urinary US (2 patients), genitography (1 patient) and myelo-CT (1 patient). Depiction of pathological findings with different imaging modalities was reviewed and validated with special respect to their demonstrability by US. Ultrasonography detected the sacral bony defect as well as the presacral pathology (meningocele and/or tumour) and thereby gave the basic diagnosis in all of the cases. It also depicted tethered cord and urinary tract abnormalities correctly. Magnetic resonance imaging gave a more distinct visualization of pre- and intraspinal pathology with additional demonstration of intraspinal lipoma in two cases. Regarding anorectal and genital malformations, radiographic contrast agent studies had been used in all patients. Two blind-ending retrorectal fistulas, depicted by enema, were missed by MRI. Patients with congenital or early infancy obstipation, anorectal malformations and complex urinary tract malformations should have spinal and pelvic sonography first. A plain film of the sacrum is recommended in equivocal cases. The need for MRI and contrast agent studies depends on the individual pathology, whereas presently MRI has made further radiographic imaging increasingly dispensable. A screening program with lumbosacral US or plain radiography for families with Currarino triad should be obligatory.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European radiology 4 (1994), S. 371-376 
    ISSN: 1432-1084
    Keywords: Contrast media ; Gastrointestinal tract ; Infants ; Iotrolan ; Radiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Over the past 5 years we have used the new dimeric, non-ionic, water-soluble contrast agent iotrolan (Isovist-300) in 370 studies of the paediatric gastrointestinal tract. Iotrolan is by far the best accepted and tolerated contrast agent for young children. Because of its iso-osmolality it can be used without dilution. No ill effects were reported in cases of aspiration or perforation. In all studies very high contrast images were obtained and the required diagnostic information was provided. The only disadvantage of iotrolan is its present high price. However, in view of the small volumes required in children (10 ml is sufficient in 59% and 20 ml sufficient in 92% of investigations) this should not be a major obstacle to its use. Iotrolan promotes a much greater readiness among paediatricians and paediatric surgeons to have gastrointestinal studies performed on very small and very sick babies.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Canavan disease (CD) is a rare leukodystrophy which is lethal in infancy or early childhood. The underlying biochemical abnormality in CD is a hereditary deficiency ofN-aspartoacylase transmitted in an autosomal recessive fashion. We report on the ultrasound (US), CT, and MRI findings of three unrelated boys with biochemically confirmed CD. At 6 and 9 months of age, two CD patients with rapid neurological deterioration showed markedly enhanced acoustic attenuation of the white matter with the exception of the corpus callosum, giving the appearance of a reversed pattern of echogenicity of cortical gray and subcortical white matter. While gyri and sulci had an almost normal US appearance, the periventricular gray matter featured prominently with increased echogenicity. In contrast another CD patient with a more protracted course had ventricular enlargement when examined by US at 5 and 9 months but no alteration in white matter echogenicity. MRI showed impaired myelinization in all three patients with Canavan disease.
    Type of Medium: Electronic Resource
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