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A long-term follow-up (average, eight years) of surgical treatment of thirteen cases of Madelung's deformity in eight patients showed that the Darrach procedure was successful when there was mild deformity and that biplane osteotomy of the radius combined with the Darrach procedure was needed for severe deformity. Subluxation of the lunate bone ulnarward occurred after the Darrach procedure but was not clinically symptomatic.

1 . A classification of Madelung's deformity on an etiological basis is suggested. 2. Seven patients with "idiopathic" Madelung's deformity and one occurring in association with Turner's syndrome are discussed. 3. Cytogenetic studies of five patients are recorded and their significance discussed. 4. Arthrodesis of the radio-carpal joint by the "wedge" technique is preferred for those patients whose symptoms warrant treatment.

MADELUNG'S deformity of the wrist is a rare condition, usually bilateral, developing at the distal end of the forearm of young individuals between the ages of 8 and 20 years—80 per cent of such deformities appear between the ages of 11 and 15 years. The deformity is essentially an anterolateral curvature of the distal third of the radius, with dorsal prominence of the joint end of the ulna. Contrary to early descriptions, it is not associated with an irreducible dislocation of the wrist. The lesion progresses slowly and by the end of one or two years the deformity is complete. During the active developmental stage, pains may or may not be present. If there is discomfort from pain or ache, it may be aggravated by forced movement, chiefly by extension. The anatomical changes are best seen in roentgenograms which include the hand and the entire forearm. There is bowing of the lower third of the radius, usually dorsal and lateral. The articular surface is tilted medially toward the volar side and slightly rot...

Seven cases of dyschondrosteosis are reported. This is a hereditary bone dysplasia with an autosomal dominant mode of transmission characterized by a typical deformity of the distal radius and ulna and proximal carpal bones and mesomelic dwarfism. The wrist deformity has been widely reported in the medical literature under the eponym, Madelung's disease.

Dyschondrosteosis is a dominant hereditary bone dysplasia resulting in wrist deformity and mesomelic dwarfism. No previous cases of this condition have been described from North America. This report also includes the first documented case in a male. European authors have distinguished between dyschondrosteosis and Madelung's disease. Clinical and radiologic findings in 5 patients in 2 families presented here and a review of the literature strongly suggest that these two conditons are in fact identical. Dyschondrosteosis would appear to be the preferred designation since, historically, it has included all of the features of the condition.

The writers have presented a case of bilateral dyschondroplasia of the distal radial epiphysis (Madelung's deformity) with fusion of the semilunar and triangular bones.

THE dyschondrosteosis of Leri-Weill and the condition known as Madelung's deformity seem to be related entities. 1 Dyschondrosteosis was first described in 1929 in the French literature by Leri and Weill. 2 However, Madelung, a German surgeon, has described the deformity in a patient presented to the seventh congress of surgeons in Germany in 1878. 3 A review of the available literature shows few reports of this deformity in recent years. Those in the European medical journals are more readily available. Stetten, in 1909, was the first to report the condition in the American literature with a complete review of its history. 4 The condition of Leri-Weill is characterized by short stature of variable degree. The forearms and lower extremities are characteristically short (Fig 1). There is a posterior subluxation of the ulna and elbow with limited wrist motion, with or without genuvarus. X-ray film findings are those of an

IN 1878, MADELUNG (8) described a painful abnormality of the wrist and forearm which characteristically began in adolescence. The radiographic alterations of the radius and ulna were described later. The most complete review of the subject is that of Anton et al. (1), who collected and summarized the literature through 1938. Considering this clinical and radiographic abnormality a deformity rather than a syndrome, these workers distinguished it from Madelung's disease, which is characterized by diffuse symmetrical lipomatosis of the upper part of the back, shoulders, and neck. They also suggested that the deformity of the wrist and forearm be considered a dyschondroplasia of the distal radial epiphysis. While this may be a logical name for the entity, it has not found wide usage, Madelung's deformity remaining the preferred term. In 1929, Leri and Weill (7) reported a case of dwarfism and radiographic alterations of the forearm and wrist similar to Madelung's deformity, although no reference was made to M...