Cases reported "Osteosclerosis"

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1/4. Raine dysplasia: a Brazilian case with a mild radiological involvement.

    We report a preterm male infant, the first child of a young consanguineous couple, whose physical examination revealed craniofacial disproportion with microcephaly, wide fontanelles, exophthalmos, low nasal root and hypoplastic nose, long philtrum, small mouth, high arched and narrow palate, micrognathia, dysplastic, low-set and rounded ears, short neck and, arthrogryposis. Postmortem findings included hypoplastic lungs. Radiological examinations showed mild and localized increased of bone density in the cranial vault and skull base and facial bones and undermodelled in the long bones. The above findings are characteristics of Raine dysplasia but the case reported here presents a mild bone involvement with only a localized bone sclerosis and absence of prenatal fractures. We discuss the possibility that this case represents an allelic mutation of the Raine gene. The consanguinity of the parents reinforces the hypothesis of autosomal recessive inheritance for this entity.
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2/4. Condensing osteitis of the clavicle. Report of two cases and review of the literature.

    Condensing osteitis of the clavicle, better defined as aseptic enlarging osteosclerosis of the clavicle, is a rare and benign idiopathic lesion. It is probably of degenerative or mechanical origin, and is most commonly seen in middle-aged women as a tender swelling over the medial one-third of the clavicle. Although the clinical features may be confusing and nonspecific, the typical radiographic and histopathological findings will mostly lead to a correct diagnosis of this disorder. The differential diagnosis is quite extensive. Most difficult to differentiate are: avascular necrosis of the medial clavicular epiphysis, sternoclavicular orsteoarthritis, low-grade chronic osteomyelitis, sternocostoclavicular hyperostosis and tietze's syndrome. The authors recommend a thorough physical examination and technical tests, not only in subjects with pain of the clavicle but also in those with shoulder pain only, especially in women who are in their fourth decade. Treatment with analgesic and anti-inflammatory medications may be variably effective. In refractory cases excisions of the medial one-third of the clavicle may be indicated to offer better relief of symptoms as well as to exclude malignancy.
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3/4. Osteomesopycnosis. A new case.

    A 10-year old, mentally and physically normally developed girl complained of back pain following an accident. Radiological findings revealed an inhomogeneous, coarse osteosclerosis along the endplates and posterior parts of the ovoid shaped vertebral bodies, in the proximal and distal femora-, proximal humeri and tibia epiphyses, and along the lateral aspects of the femoral necks. The pelvis presented a mottled appearance of the trabecular pattern at the symphysis pubis and the lateral parts of ischii and sclerosis in the acetabular region. The only laboratory abnormality was a high serum parathormone level. The family history revealed an autosomal dominant inheritance. The name proposed by Maroteaux for this skeletal disorder is osteomesopycnosis, as the lesions were localized to the spine, pelvis and sometimes proximal femur. The skull, ribs, long bones, hands and feet were not involved in the reported 12 cases. Radiographs of this patient showed in addition changes in the hand. This disease must be distinguished from atypical axial osteomalacia.
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4/4. A 42-year-old woman with low back pain.

    The following case is presented to illustrate the roentgenographic and clinical findings of a condition of interest to orthopedic surgeons. The initial history, physical findings, and roentgenographic examinations are noted on this page. The clinical and roentgenographic diagnoses are presented on the following pages.
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