Osteoarthritis associated with osteopetrosis treated by total hip replacement arthroplasty

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IN 1904 Heinrich Albers-Schönberg,1 a German radiologist, reported a case of generalized osteosclerosis and multiple fractures in a 26-year-old patient. The condition constituted a new disease entity which now bears his name. On the basis of the roentgenographic appearance of the bones, Albers-Schönberg described the disorder as “Marmor-Knochen Krankheit” (marble bone disease). The triad of findings considered to be pathognomonic were generalized osteosclerosis, anemia, and lymphadenopathy with hepato-splenomegaly.

Laurell and Wallgren,2 in 1920, focused attention on the multiple pathologic fractures characteristic of marble bone disease and introduced the term osteosclerosis fragilis generalisata. Karshner,3 in 1926, introduced the term osteopetrosis to describe the petrified nature of the affected bones. Since then the disease has been reviewed by several authors, and approximately 300 cases have been reported. In a number of instances another disease has been associated with osteopetrosis,4 for example, leukemia, Hodgkin’s disease, sarcoma, coarctation of the aorta, and rickets. However, there has been no detailed description of degenerative joint disease in association with osteopetrosis.

The fragile quality of the dense bone in osteopetrosis causes increased fractures, particularly of the femoral neck in the proximal third of the femoral shaft and the proximal third of the tibia. Despite this fragility and the variability of the healing of fractures, there is little information in regard to difficulties encountered in internal fixation of such fractures.4, 5 The purpose of this report is to describe a total hip replacement arthroplasty in a patient with Albers-Schönberg disease, and to emphasize the technical difficulties and complications . . .



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