J Bone Joint Surg Br
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J Bone Joint Surg Br · May 1983
Sarcoma complicating Paget's disease of bone. A clinicopathological study of 62 cases.
Out of 21 900 cases filed at the Latin-American Registry of Bone Pathology between April 1940 and July 1981, there were 987 with Paget's disease (4.51 per cent); 62 of these (6.28 per cent) were complicated by sarcoma and two were associated with giant-cell tumours of bone (osteoclastoma) without signs of malignancy. There was a slight predominance of men and the ages ranged from 45 to 87 years, with an average of 66 years. The most frequent sites were the femur (23 cases), the humerus (nine), the pelvis (10), and the tibia (nine). ⋯ The histological features of the osteosarcomata were characteristic, with large numbers of osteoclast giant cells, alternating with atypical osteoblasts, thus exaggerating the anarchic remodelling process of Paget's disease. The neighbouring areas of the pagetic bone showed an increased number of osteoclasts. These facts suggest a possible pathogenetic relationship between sarcoma and Paget's disease; the possibility of both processes having a viral aetiology is discussed.
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J Bone Joint Surg Br · Jan 1983
Osteogenesis imperfecta with dominant inheritance and normal sclerae.
Most patients with dominantly inherited osteogenesis imperfecta have blue sclerae and relatively mild symptoms. However, in a small group of families the patients have normal sclerae and this disorder has been classified as Type 4 osteogenesis imperfecta. This paper reports the clinical and radiographical features of 48 patients from 16 families with Type 4 osteogenesis imperfecta and compares the findings with those of the classical disorder with blue sclerae (Type 1 osteogenesis imperfecta). ⋯ In Type 4 the first fracture more commonly occurs at birth, dentinogenesis imperfecta is more frequent than in Type 1 and bruising and nose-bleeds are less common. As in Type 1, the radiographic appearances of the bones may be normal. It is important that Type 4 osteogenesis imperfecta should be recognised because of the need for competent genetic counselling, because the management may be different from that appropriate for Type 1 and because it may be mistaken for idiopathic juvenile osteoporosis or child abuse.
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Four infants between 2 and 13 weeks of age developed vertebral osteomyelitis. Their symptoms were different from those of children with discitis in that our patients were systemically ill, there was almost complete dissolution of involved vertebral bodies with either normal or nearly normal adjacent vertebral endplates, and three of the four children had recurrence of infection. ⋯ Years later the radiographic appearance of these children can be identical to congenital kyphosis with either anterior failure of segmentation or posterior hemivertebrae. The treatment should be the same as for congenital kyphosis with early bracing in extension and early fusion for progressive kyphosis.