Whole-body magnetic resonance imaging: an essential tool for diagnosis and work up of non-oncological systemic diseases in children.

AIM: Whole-body MRI (WBMRI) is a non-irradiating imaging technique, suitable to investigate the extent of multisystemic diseases in pediatric patients. However its real impact on diagnosis and management of non-oncological pediatric diseases remains unclear. We present our experience of pediatric WBMRI in various pathologies. METHODS: We retrospectively reviewed medical files of all non- oncologic patients who underwent WBMRI from January 2008 to November 2012. The MRI protocol included T1 weighted and 3D SPACE Inversion Recovery (IR) and fat saturated diffusion weighted (DWI) sequence. We reviewed medical records in order to determine if performing WBMRI added useful information for diagnostic purposes and/or changed clinical management. RESULTS: Forty-two children were included in the study (19 F, 23 M) (median age 4.7 years). Twenty-one children underwent general anesthesia. WBMRI was a useful tool to provide correct diagnosis in chronic recurrent multifocal osteomyelitis (CRMO), and to identify the origin of fever or arthralgia of unknown etiology. WBMRI allowed to determine the extent of disease in juvenile idiopathic arthritis (JIA), chronic granulomatous disorder, enchondromatosis, Langerhans cell histiocytosis, and in the assessment of tumor burden in neurofibromatosis type I. For the battered child syndrome, the influence on management was rather minimal. For each of these pathologies we performed a review of recent literature. CONCLUSION: WBMRI is easy to perform in children and allows the assessment of systemic diseases or discovery of lesions ignored by clinical examination. WBMRI influenced the decisional process and clinical management of various pathologies in our series.

Brain magnetic resonance imaging (MRI) and neurological changes after a single high altitude climb.

PURPOSE: Neurological impairment, mental dysfunction, and brain imaging changes caused by severe hypoxia have been described by several authors. However, the occurrence of transitory, long lasting, or permanent brain damage has been debated. Although climbing to 8000 m is reserved to a small number of climbers, there are hundreds of lowlanders spending relatively short holidays climbing peaks up to 6000 m in the Andes or in the Himalayas. They are usually not well acclimated and often suffer from acute mountain sickness (AMS). The aim of this study was to examine the effect of a single high altitude exposure on the changes in brain MRI and neuropsychological testing in climbers. METHODS: Brain MRI, medical history, and a battery of neuropsychological tests were obtained in eight male climbers between 31 and 48 yr of age a few days before and between 5 and 10 d after returning to sea level following ascent to altitudes of over 6000 m without oxygen. RESULTS: The mean AMS symptom score recorded at 5500 m was three in all climbers, headache being the predominant symptom. CONCLUSION: We did not observe the changes in brain imaging and in neuropsychological testing observed by other authors. The residual central nervous system impairment following return from high altitude was not observed in our study, and the good results in neuropsychological testing were well correlated with the unchanged brain MRI imaging.

Magnetic resonance imaging helps in the early diagnosis of myositis ossificans in children.

Two cases on myositis ossificans circumscripta (MOC) in the arm are reported. Plain X-ray films and magnetic resonance imaging (MRI) were performed in both cases. In the first, an intramuscular tumor-like mass without calcifications was found on MRI with soft-tissue edema extension. In the second, MRI disclosed additional bone-marrow edema. The diagnosis of MOC was confirmed by biopsy in one case and by follow-up in the other. MOC is a benign soft-tissue lesion that is rare in children, with an acute course and usually spontaneously favorable evolution. The differential diagnosis from an infection or a malignant tumor remains difficult. The best imaging modalities are conventional radiography and MRI. The MRI patterns of MOC are typical but not pathognomonic; typical MRI findings in conjunction with clinical symptoms during the early phase of MOC permit the postponement of a biopsy or aggressive surgical procedures. Surgery is indicated for cases not showing typical MOC calcifications at a later stage.

Reliability of color Doppler and power Doppler sonography in the evaluation of intussuscepted bowel viability.

In two similar cases of irreducible intussusception with wall necrosis and perforation, symptoms had begun 24 h before admission and the plain radiographs showed signs of small-bowel obstruction. The sonographic (US) appearances, however, were different: color Doppler (CD) US showed vascular flow in the intussuscepted bowel in one case and no flow in the other. After radio-clinical assessment, both children underwent surgery and an intestinal resection had to be performed, manual reduction being impossible. The reliability of the US findings and the prognostic value of CD and power Doppler US in determining the viability of the intussuscepted bowel are discussed with a review of the literature.

Achalasia-alacrima-ACTH insensitivity syndrome (Triple-A-syndrome).

Achalasia is a rare pathology in infancy. It is still more infrequent when associated with adrenal insufficiency and alacrima, a disorder known as Allgrove's syndrome, the etiology of which remains unclear. We describe a 9-year-old girl who presents with glucocorticoid insufficiency, partial mineralocorticoid deficiency, achalasia, and alacrima.