Thirteen patients with dyschondrosteosis from eight families are reviewed and their clinical and radiographic variation noted. Inheritance is likely to be autosomal dominant but with only 50 per cent penetrance. Stature was moderately reduced, due to shortening of the bones of the leg. Radio-ulnar shortening could either involve both bones equally or the radius predominantly, in which case a typical Madelung deformity was seen. Tibio-fibular disproportion was present in half the patients, two of them having severe deformity associated with tibia varum and a long fibula. The treatment of one of these patients is described. It is recommended that patients with dyschondrosteosis should be kept under surveillance during the growing period. Problems in the limbs, especially the legs, may require operations to equalise the length of the two bones.
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Article Synopsis
- - This paper reviews research on Madelung's deformity, a condition affecting the distal radius where the joint tilts and causes wrist and elbow issues, often linked to hereditary and environmental factors.
- - The deformity is more prevalent in females, usually affects both arms, and can be linked to other conditions like Leri-Weill dyschondrosteosis, with symptoms including wrist pain, stiffness, and deformity.
- - Diagnosis relies primarily on X-rays, with MRI used for early detection, and treatment options vary from non-invasive monitoring to several surgical procedures, depending on severity and symptoms.
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