Clinical History
A 25 year old lady started complaining of increasing pain and deformity of both wrist over a few months. No history of trauma was evident. She was referred to the orthopaedic outpatient clininc by her GP.
Imaging Findings
25-year-old lady presented to the outpatient clinic complaining of increasing pain and deformity of both wrists. Inspection revealed the prominence of the distal ulna and bowing of the radius on both sides. Examination revealed the presence of marked tenderness over the distal radioulnar and wrist joints. Both active and passive joint movements were limited on both sides. Physical examination revealed short stature. Standard AP and Lateral plain X-ray projections of both forearms were taken.
Discussion
This condition known as carpus curves results from epiphyseal growth arrest on the volar and ulnar half of the distal radius, causing the articular surface to be directed ulnar and volarward. It is inherited as an autosomal dominant condition with variable expressivity. It shows a predominantly female distribution with a 4:1 ratio. It is bilateral in two thirds of patients.
It is caused by the complete absence or underdevelopment of the ulnar portion of the radius growth plate, thus failing to contribute to the linear growth of the corresponding border of the radial diaphysis. The precise mechanism of this disorder has not been determined however hemiarthropathy of the distal radius epiphysis, abnormal muscular insertions and disturbances to the vascular supply of the epiphysis have all been suggested.
It is associated with Madelung’s dyschondrosteosis and mesomelic dwarfism should be suspected if the condition is bilateral.
Clinically it is characterised by insidious onset of pain in one wrist, then in other, and increasing prominence of the dorsal ulnar head and bowing of distal radius. Pain from radioulnar subluxation or radiolunate impingement usually becomes less severe as the patient grows older. However when the deformity becomes stabilised, the incongruity of the joint surfaces in the wrist may lead to the recurrence of painful symptoms. Deformity progresses until growth plate of the distal radius closes. Wrist motion, particularly extension and supination, is limited. Although Madelung's deformity is considered a congenital deformity, it does not become manifest until in late childhood or early adolescence.
Treatment options include non operative conservative measures indicated early on in the condition especially when the patient is asymptomatic. Various surgical procedures are available in the treatment plan for this condition.
Differential Diagnosis List
