A 13-year-old premenarchal female presented with a 1-year history of left wrist pain and progressive flexion deformity. She described the pain as a dull ache, predominantly on the ulnar side, that increased throughout the day. The pain was moderately severe and, apart from NSAIDs, which provided only minimal relief, nothing made the pain better or worse. The patient was left-handed, and the pain was limiting her ability to write in school. She denied any numbness or tingling in the wrist but said she was experiencing weakness in the wrist secondary to pain. Other than the progressively worsening wrist pain, the patient had been in her usual state of health.
History The patient had no history of trauma to the wrist, no recent weight loss or infection, and no surgical history. Her only significant history was seasonal allergies, for which she took loratadine (Claritin) as needed. She lived in an urban area with her parents and two sisters, who were healthy. There was no family history of genetic disorders or musculoskeletal pathology. The patient was in the seventh grade and not active in any sports.
Physical examination The patient weighed 115 pounds and was 63 inches tall. The left wrist was in a flexed position with a prominent ulna, but no ecchymosis, erythema, edema, or muscle wasting was noted. The left wrist did have an obvious flexion deformity: excessive active palmar flexion to 100 degrees and active dorsiflexion to 70 degrees (normal wrist range of motion [ROM] is 75 degrees of flexion and 70 degrees of extension). Passive ROM measurements were the same. Supination of the left forearm was 80 degrees, and pronation was 70 degrees (normal supination is 85 degrees; normal pronation, 70 degrees). The distal ulna was tender to palpation. The radial, ulnar, and median nerves were intact. Capillary refill was less than 2 seconds (normal capillary refill is 2-3 seconds). The right wrist had no gross deformity; there was active palmar flexion to 90 degrees and active dorsiflexion to 80 degrees, with no limitations in supination or pronation and intact nerve function. Plain radiographs of the affected wrist were obtained (see Figure 1).
Diagnosis
This patient had a Madelung's deformity (MD), which is excessive radial and palmar angulation of the distal radius caused by a growth disturbance of the palmar and ulnar portion of the distal radial physis.1 The disturbance is due to a combination of factors, including a bony lesion in the ulnar portion of the distal radial physis and an abnormal palmar ligament (known as Vickers' ligament), which tethers the lunate to the radius just proximal to the physis.1
Radiographic evidence Multiple abnormalities may be seen on radiographs of patients with MD, depending on the severity of the deformity (see Table 1). Not all signs can be detected in every patient. Furthermore, there is no correlation between the number and extent of radiographic signs and the patient's complaints of pain and decreased ROM. In this case, the radiographs demonstrated increased width between the distal radius and ulna, positive ulnar variance, triangularization of the distal radial physis, and wedging of the carpus between the deformed radius and the protruding ulna. Bone age on the radiograph was approximately 12 years, which correlated with the patient's chronological age of 13 years. The diagnosis of MD was made by progressive deformity without trauma and by the clinical and radiographic appearance.
Treatment
Surgery was the best option for this patient because of her proximity to skeletal maturity, degree of pain, decreased function, and radiographic findings. After a discussion of options, the patient and her mother decided to proceed with surgery to alleviate the pain and correct the cosmetic deformity (see Figure 2).
The patient underwent left wrist dome osteotomy with Vickers' ligament release and epiphysiodesis of the distal ulna. Fixation was accomplished with two 0.062 Kirschner wires. The patient was placed in a long-arm cast for 3 weeks, followed by a short-arm cast for 3 weeks. She subsequently underwent pin removal, which was conducted in the operating room secondary to migration of the most proximal Kirschner wire beneath the skin. The patient wore a wrist splint for another 4 weeks. At her 3-month follow-up visit, she had no pain and the wrist had an anatomically normal appearance with normal ROM in both flexion and extension (see Figure 3).
