IS Case 574: Madelung deformity in Turner's syndrome
Imaging Sciences URMC 2010
Publication Date: 2011-11-17
Patient is a 13-year-old girl being evaluated for short stature. Imaging of her left wrist demonstrated features consistent with Madelung deformity. The patient later tested positive for Turner's syndrome.
Madelung deformity in Turner's syndrome
Madelung deformity describes a variety of conditions where there is premature fusion of the ulnar aspect of radial epiphysis. Typical features include ulnar angulation of the distal radius, decreased carpal angle, dorsal subluxation of ulna, triangulation of distal radial epiphysis, and wedged in lunate with widened distal radioulnar interosseous space. Etiologies includes post-traumatic, dysplasias, genetic, and idiopathic causes. Bone dysplasias associated with with Madelung deformity include multiple hereditary cartilaginous exostoses, Ollier disease, achondroplasia, and mucopolysaccharidoses including Hurler syndrome. There is also an association with mesomelic dwarfism, e.g., dyschondrosteosis, and Turner's syndrome.
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