[Febrile osseous pain in children with sickle cell disease: MRI findings]. / Douleurs osseuses fébriles chez l'enfant drépanocytaire: apport de l'IRM.

PURPOSE: The aim of this study was to report the MRI findings that can suggest a vaso-occlusive crisis in cases of febrile osseous pain in children suffering from sickle cell disease. MATERIALS AND METHODS: MRI (T1 and T2 weighted sequences and T1 weighted sequence with fat saturation before and after gadolinium injection) was performed in 10 children with sickle cell disease, presenting with febrile osseous pain. The diagnosis of vaso-occlusive crisis was made after fast improvement due to symptomatic treatment and negative bacteriological result. RESULTS: MRI was abnormal in all cases. A multifocal localisation was found in 2 cases. Bone marrow abnormalities were constant. In 10 cases, high T1 and T2 signal and metaphyso-diaphysial lesions were noted. Heterogeneous medullar enhancement with "ink stain" feature was constant. Early periosteal abnormalities were noted in 8 cases with inflammatory or stratified features. Cortical thinning was found in 1 case. Soft tissue abnormalities were observed in 5 cases with inflammatory features in 4. CONCLUSION: Multifocal synchronous localisation, medullar abnormalities resulting from hemoglobin degradation, heterogeneous enhancement, early periosteal abnormalities and associated soft tissues swelling are MRI findings suggesting acute vaso-occlusive disease.

Distinctive new form of spondyloepimetaphyseal dysplasia with severe metaphyseal changes similar to Jansen metaphyseal chondrodysplasia.

We report a boy with a unique, "new" form of spondyloepimetaphyseal dysplasia. The conspicuous features of the spinal changes were the delay in ossification of the cervical spine and posterior elements of the thoraco-lumbar spine. The vertebral bodies were of abnormal shape but of normal size and well ossified. The hallmark of epiphyseal changes was markedly delayed ossification (bone age). The severely disturbed metaphyseal ossification was similar to Jansen metaphyseal dysplasia. This pattern of changes has not yet been described in spondyloepimetaphyseal dysplasia.

A novel syndrome resembling Desbuquois dysplasia.

We report on three Tunisian siblings with a rare assortment of clinical and radiographic abnormalities closely resembling Desbuquois dysplasia. However, the siblings have had normal facies, normal hands, and were mentally normal. There were severe musculo-skeletal distinguishing features such as joint stiffness, severe kyphoscoliosis, and multiple large joint dislocations. Moreover, the patients had an additional remarkable radiographic feature not reported in Desbequois dysplasia-multiple carpal ossification centers. The diagnosis of Desbuquois dysplasia is more difficult in older children and adults as the characteristic facial features of early childhood may recede, and the metaphyseal growth plates obliterate. This condition of these patients represents a novel Desbuquois-like syndrome.

[Acute osteomyelitis in children: early MRI diagnosis]. / Douleurs osseuses fébriles chez l'enfant: apport de l'IRM.

PURPOSE: To assess the value of MRI for early diagnosis of osteomyelitis in children presenting with sepsis and acute onset of musculoskeletal pain. MATERIALS AND METHODS: MRI including fat suppressed T2W, and fat suppressed pre- and postcontrast T1W sequences was performed within 48 hours of admission in 26 children with clinical (fever and acute musculoskeletal pain) and biological (elevated WBC count and ESR in all cases, and elevated CRP in 12 patients) suspicion of acute osteomyelitis. None of the patients had sickle cell disease. RESULTS: MRI was normal in 7 children (26%). Bone marrow signal abnormality was noted in 19 cases (74%) consistent with acute osteomyelitis in 18 cases and metastatic neuroblastoma in 1 case. CONCLUSION: MRI is useful for evaluation of children presenting with sepsis and acute musculoskeletal pain, early diagnosis of osteomyelitis and to prevent unnecessary hospital admission and work-up.

[Tuberculosis of the knee: MRI findings in two pediatric cases]. / Tuberculose ostéo-articulaire du genou: aspects IRM de deux cas pédiatriques.

Osteo-articular tuberculosis is rare in infants. The MRI findings reported for adolescents and young adults mainly relate to spinal involvement. Two cases of osteo-articular tuberculosis of infants located at the knee are presented. Vaccination has been correctly done. Skin test and chest radiography were normal. Evolution was insidious for one case. Osseous, medullary, cartilaginous and soft tissue abnormalities revealed by MRI were suspicious for tuberculosis. Diagnosis was confirmed at histology for both cases and bacteriology for one case. The aim of this study is to report the MRI features of osteo-articular tuberculosis in pediatric patients.