This patient is a 15-year-old, right-hand-dominant female who presented to the orthopedic service at the age of 10 for evaluation of short stature and "odd-looking wrists."
Age 11, prior to surgery. The entire radius is abnormal, particularly the volar-ulnar portion of the distal radius. This is consistent with Madelung's deformity. (Figure 1)
She was diagnosed with a skeletal dysplasia (Leri-Weill syndrome) and Madelung's deformity of both wrists (Figure 1). She did not have wrist pain at that time.
Post-op distal radius osteotomy, age 12. The shape of the distal radius is more normal. The K-wires visualized on the radiographs are maintaining the osteotomy in place while it heals. (Figure 2)
At age 11, her left wrist was treated with an osteotomy of the distal radius and division of an abnormal ligament (Vicker's ligament), correcting the volar subluxation of the carpus (Figure 2). Several months later, the right wrist was treated with a similar procedure.
She was pain-free for two years when she developed discomfort along the ulnar side of the wrists, especially when moving the forearm from pronation to supination and vice versa. The patient identified her pain as emanating from the region of the distal radioulnar joint (DRUJ), with the right wrist more symptomatic than the left. Wrist immobilization with splints and over-the-counter oral anti-inflammatories palliated her symptoms, but she continued to have pain, even with activities such as writing.
Exam of both wrists demonstrated prominence of the ulnar heads dorsally, with the right being worse than the left. On the right side, elbow flexion and extension were normal, with full extension and 135 degrees of flexion. Forearm rotation was painful and limited, with supination of 60 degrees and pronation of 45 degrees. Extension of the left wrist was 20 degrees, and flexion 30 degrees, both less than normal, but not painful. Sensation was intact to light touch in all digits, and capillary refill was less than two seconds. There was discomfort at the DRUJ with forearm rotation, especially when this motion was performed while holding objects that weighed more than two to three kilograms.
Pre-op DRUJ reconstruction, age 15. The ulna is longer than the radius and it is dorsally subluxated. The DRUJ is widened and appears abnormal secondary to Madelung's deformity as well as arthritis. (Figure 3)
Posteroanterior x-rays (radiographs) of the left wrist reveal that the ulna is 10 mm longer then the radius (radius equal to ulna is normal). The DRUJ is widened, and both the ulna and radius show joint abnormalities. The volar-ulnar aspect of the distal radius is deficient, which results in an increased ulnar and volar slant of the joint. On the lateral x-ray, the ulna is subluxed dorsally and the carpus is subluxed volarly. The growth plates are closed. These are all typical findings for Madelung's deformity (Figure 3).
Posteroanterior x-rays of the right show an ulnar head that is approximately five mm longer than the radius. There is degeneration of the DRUJ, as seen by the flattening of the joint both on the ulnar and radial sides. The volar-ulnar aspect of the distal radius is deficient, and the growth plates are closed. On the lateral view, the ulna is dorsally subluxed.
Post-op DRUJ reconstruction, age 15. The distal ulna has been resected, including the portion that articulates with the radius. The space between the ulna and radius is filled with allograft tendon, which is woven around the ulna and attached to the radius with the two suture anchors that are visible on radiographs. (Figure 4)
The goal of treatment for Madelung's deformity is to provide a stable wrist joint with pain-free motion. Because this patient's right wrist was more painful than the left, the recommendation for surgical reconstruction of the right wrist was made. This consisted of: 1. An ulnar shortening osteotomy, and 2. Reconstruction of the DRUJ. Specifically, the ulna was shortened by excising the distal one centimeter of the ulna, resulting in more normal ulnar variance. The ulnar head subluxation was addressed by removing the abnormal articular surface and reconstructing the joint by first interposing a spacer of allograft tendon rolled on itself and then using the tails of the graft to reinforce the restraining ligaments of the joint. The repair was protected in a long arm cast for six weeks (Figure 4). After six weeks, the patient was allowed to slowly get back to unrestricted activities. No therapy or further splinting was needed.
At her most recent visit following the right wrist reconstruction, her wrist pain and function had improved. She no longer uses pain medication or protective wrist splints. She is able to perform all activities of daily living and play musical instruments, including the piano. Her DRUJ is stable, and her joint motion, though unchanged from preoperatively, is pain-free. She does have diffuse "hand pain" with prolonged writing, and the limitations in wrist motion preclude doing exercises such as push-ups.
Due to the success of the surgery on the right wrist, the patient opted for a similar left wrist reconstruction. The early results of this procedure are similar to those of her right wrist.
Normal wrist on the left, Madelung's deformity on the right. In Madelung's deformity, the ulnar portion of the radius is deficient, as can be seen by the abnormal slope of the distal radius compared to the x-ray on the left. (Figure 5)
Madelung's deformity is a rare condition affecting the wrist. It is more common in females and can occur in association with Leri-Weill skeletal dysplasia. The condition usually becomes symptomatic during adolescence. The basic problem is a growth disturbance of the volar-ulnar portion of the distal radius growth plate (Figure 5). There is an associated capsular restraint named for the surgeon who first described it, "Vicker's ligament." This structure is also located on the volar-ulnar aspect of the wrist and, in combination with the abnormal growth, alters wrist mechanics. Patients typically exhibit decreased motion, particularly in supination, extension and ulnar deviation. Although patients may initially have minimal pain, the progression to degenerative changes inevitably occurs over time.
Treatment for Madelung's deformity is tailored to the age of the patient and severity of the deformity. Nonoperative measures, including splinting, nonsteroidal anti-inflammatory drugs (NSAIDs) and activity modification, palliate but do not prevent progression. These modalities should be the first line of treatment but are rarely the definitive treatment. In cases that are detected early and that demonstrate steady progression of the deformity, surgical treatment is directed at removing the tethering capsule, "Vicker's ligament," and correcting the alignment of the radius bone with an osteotomy. Unfortunately, the osteotomy does not completely correct the abnormal anatomy, and further surgery is typically necessary, as it was in this case. In skeletally mature patients, surgical treatment is directed toward addressing the most symptomatic area. Specifically, for patients with symptoms isolated to the radiocarpal joint, osteotomies of the distal radius may be effective, although in the most severe cases wrist fusion is the only treatment that will relieve pain. For many patients, the DRUJ is more painful than the radiocarpal joint. This is a reflection of the normal growth of the ulna in the presence of abnormal growth of the radius. This mismatch results in an ulna that appears longer than the radius and subluxates dorsally, such as in the patient discussed here. This can be treated with procedures directed at the DRUJ, including ulnar shortening, distal ulna resection with or without interposition and DRUJ stabilization, such as performed in this case. For those with severe deformity, more than one procedure will likely be necessary over a lifetime. Unfortunately, some of these patients may eventually require distal radioulnar fusion or replacement.
Dubey A, Fajardo M, Green S, Lee SK. Madelung's deformity: a review. Hand Surg Eur Vol. 2010 Mar;35 (3):174-81.
Watt AJ, Chung KC. Generalized skeletal abnormalities. Hand Clinics. 2009 May;25 (2):265-76.
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