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This report concerns another case of striped bone disease (Osteopathia striata) together with sclerosis of the base of the skull. The mode of inheritance of this rare genetic disorder is still unknown. The clinical characteristics of 20 cases reported in the international literature are summarized and compared with our own findings.
A hyperdense zone in the 3rd lumbal vertrebra grew during four years. Bone scans showed no augmentation of activity in this region. Differentialdiagnosis includes a local hyperostosis and enostoma.
Not only for giving the diagnosis "Perthes' disease" but also with regard to prognostic statements the knowledge of the radiological stages of Perthes' disease is essential. The present treatise demonstrates the radiological course of Perthes' disease, how it results from the evaluation of the courses of illness of 116 patients, which were treated in the period from 1965 to 1980 at the Orthopedic University Hospital Hamburg-Eppendorf because of a genuine Perthes' disease. The following schema turned out to be appropriate to the assessment: I. Initial stage, I a Early period, I b Late period. II. Florid stage, II a Sclerosing period, II b Fragmental period, II c Osteolytic period. III. Stage of regeneration. IV. Final stage.
Based on 57 fibrous metaphyseal defects the conventional radiologic signs are described as diagnostically reliable including asymptomatic patients as well as patients with a history of pain and lesions with positive scintigraphy. The radiologic morphology of the fibrous endosteal and medullary defects differs with the age of the patient. Beyond the age of 18 years there is a sclerosis of the lesion and a less marked arrosion of the corticalis. The differential diagnosis depends on the cortical, endosteal of medullary site of the fibrous defect as well as on the age of patient and includes the cortical metastasis, the juxta-articular osteoid-osteoma, the periosteal chondroma, the cortical irregularity, the solitary bone cyst, the fibrous dysplasia, the chronic osteomyelitis and the osteosclerotic osteosarcoma.
The conventional radiographs and CT examinations of 112 patients with primary and secondary bone tumours of the spine have been compared. Two hundred and sixty-eight vertebrae were shown to be involved, using CT; conventional radiographs showed correctly in 88% an involvement of the vertebral bone and in 66% of the different parts of the vertebrae. Of the intraspinal and paravertebral tumour extensions shown by CT, only 23% and 33% respectively were correctly diagnosed by plain radiography. Only 51% of the vertebrae showing a reduction in height on conventional radiographs could be diagnosed on horizontal CT cuts. The type of lesion could be diagnosed in only very few cases by either method.
The type, frequency and location of lumbar vertebral lesions that may interfere with quantitative computed tomographic (QCT) estimation of bone mineral content were analysed in 1166 cases. All examinations were carried out with a standard protocol on the second, third and fourth vertebral body. A total of 130 lesions were identified, the majority of which included degenerative sclerosis of L4 and compression fractures of L2. Evaluation of 28 QCTs with compression fracture in one vertebral body showed that an accurate BMC value was obtained with an interendplate distance of 13 mm or more.
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We have compared the value of MRI with conventional radiography and CT in 40 predominantly sclerotic skeletal lesions. We have found that sclerosis and other changes in the bone structure can be demonstrated by MRI in much the same way as with conventional radiography and CT. Using high resolution surface coils, it is possible to demonstrate small areas of new bone formation such as periosteal new bone or the calcification within the nidus of an osteoid osteoma. MRI is particularly appropriate if it is necessary to show soft tissue changes within an area of sclerosis, which may be responsible for the new bone formation and which may be masked on radiographs and CT by the surrounding sclerosis.
The osseous manifestations of osteochondrosis dissecans are well demonstrated by conventional and computerised tomography. Beyond that, magnetic resonance imaging (MRI) is effective in evaluating the vitality and loosening of an osseous dissecate. Subchondral cavities and cartilaginous defects are detected with high accuracy. Further, MRI seems to be a useful method in childhood to differentiate a variant irregularity of the osseous articular surface from definite osteochondrosis dissecans.
Four cases of multifocal osteomyelitis of unknown origin in childhood are reported. The variable clinical and radiographic appearances of the disease are illustrated and the diagnostic difficulties in the early stages of the disease are stressed.
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Two case reports of recurrent multifocal periostitis in two girls aged 15 and 6 are added to the eight cases already reported in the literature. The disease is characterized clinically by recurrent mesomelic swelling of the extremities and radiologically by periosteal thickening and sclerosis of underlying bone. Hyperglobulinaemia is the most constant biochemical finding. The bone biopsy shows no typical features. The possibility of a viral etiology is discussed.
The findings at bone marrow biopsies in 5,000 patients with systemic haematological diseases were compared with the radiographic appearances. Haemoblastic conditions produce diffuse involvement of the bone marrow, whereas conditions characterised by limited infiltration, such as Waldenström's disease or Brill-Symmers' disease, produce some osteoporosis and only rarely bone destruction. Haemoblastic diseases with additional focal proliferation, such as myelofibrosis or myelosclerosis, multiple myeloma, highly malignant lymphomas and malignant granulomatosis produce circumscribed changes in the spongiosa which are readily recognised radiologically. In addition to generalised osteoporosis or sclerosis, one finds local areas of osteolysis or osteoplasia. Bone marrow biopsies in above-mentioned conditions indicate that they progress in a series of stages which correlate well with the radiological findings.
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A retrospective analysis of spleen irradiation in 25 patients with progressive idiopathic myelosclerosis (agnogenic myeloid metaplasia), treated between 1948 and 1983, shows that there is a place for the mechanical effect of irradiation being an indication for its use as a palliative measure. A decrease in spleen size of varying extent was achieved in 84% of patients, while the symptoms of abdominal compression improved in 88%. Remission period averaged 10 months. On the other hand, in all seven patients in whom a decrease in the size of the spleen might have been expected to signal an improved blood picture, the frequency of transfusion remained unchanged.
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