Madelung Deformity Treatment & Management

Nonoperative management may be helpful in skeletally mature individuals with MD and mild-to-moderate short-term wrist pain. If pain is predominantly DRUJ pain, then a sugar-tong–type splint may relieve joint irritation from overactivity. If radiocarpal pain is the primary problem, then a volar splint may decrease symptoms. Patients may eventually decrease their manual activity levels to a point that will keep their symptoms manageable without surgery. In contrast, the younger and skeletally immature patient with clear evidence of MD has pain that is caused by the tension within Vickers ligament and splintage will most likely not have a satisfactory result.

The surgical decision is based on 4 factors, as follows:

• Patient's age and the growth remaining in the distal radius
• Severity of the deformity
• Severity of the symptoms
• Clinical and radiographic findings

Operative treatment can be divided into those procedures that correct the primary deformity of the radius, those that attempt to decrease pain and increase range of motion by making a compensatory change in the ulna, and those that address both. Multiple procedures address the deformity in the radius. Conceptually, these can be broken into those that change the growth or anatomy at the physis, those that change the bony anatomy of the metaphysis, and those considered salvage-type joint-sacrificing procedures. The goal of ulnar procedures is to change the relationship of the relatively long ulna to the radius.

Vickers physiolysis

When the deformity is noticed early and significant growth remains, changing the growth pattern of the distal radial physis to correct the deformity is possible. In 1992, Vickers and Nielsen described the lesion in the volar and ulnar distal radius as both bony and ligamentous, and they stated that it is an inherent failure of focal growth and structural tethering of further growth. They described an ulnar-volar release for MD of the physis, called physiolysis, as seen in the images below. This allows, then, normal and compensatory growth to correct the deformity, much like the bony bridge resection of Langenskiöld. Vickers and Nielsen used the procedure of resecting the bony and ligamentous lesion (the first to correct MD using remaining growth to correct the deformity). They were the first to describe a ligamentous lesion as part of the pathology and also were the first to use the volar approach to address it.

Through a volar flexor carpi radialis (FCR) approach, the pronator is mobilized, and the distal radius is exposed. An osteotome is used to make a longitudinal split 5 mm from the DRUJ on the radius, and parallel transverse 1 mm sections are made until normal physis is identified. Some physis is left to overhang the metaphysis. Fat harvested from the forearm is packed into the bone defect. Completely excising the tethering ligament (Vickers ligament) between the lunate and the radius is critical. See the images below.

The Vickers ligament originates on the radius in a fossa that is seen radiographically as a flame-shaped radiolucency distal to a bone spur on the ulnar aspect of the distal metaphysis, as seen in the images below. Dannenberg noted this characteristic as an area of osteopenia in his original radiographic criteria in 1938. Carter and Ezaki noted it in 91% of their cases. It is fibrous and fibrocartilaginous and 5-7 mm thick. It inserts into the anterior surface of the lunate and the anterior radioulnar ligament portion of the triangular fibrocartilage complex (TFCC). Most likely, it is a secondary pathologic structure to a primary bony derangement and may even be a coalescence of normal structures. This ligament may be an etiologic factor in MD pain. Following operative release of this ligament, patients state soon after surgery that their pain is largely relieved.

Osteotomy of radius

If the deformity has progressed in an older child and remaining growth is insufficient, several procedures can be used to correct the position of the distal radiocarpal joint surface. They generally consist of a biplane osteotomy, either closing or opening wedge, which corrects the position of the joint surface and brings the radius and ulna into a more proper position. If a positive ulnar variance remains, an ulnar shortening procedure can be performed.

A less commonly used method is distraction histogenesis with the Ilizarov technique. Several authors have described experience with the technique of radial osteotomy and subsequent angular and length correction over time. The goal is slow correction of the distal radius to make a more congruous DRUJ. Children treated with this method are either skeletally mature or nearly mature. Half pins are commonly selected over tensioned thin-wires to allow better range of motion during distraction. Three or 4 rings are used, with fewer complications and less pain when 3 are used. The first goal after fixation placement and radial osteotomy is angular correction over a 2-week period. The second goal is lengthening of approximately 1.5 cm over the next 2 weeks, followed by consolidation for 3 weeks. The entire process in the distractor takes 8 weeks.

In 2000, Carter and Ezaki reported a combined procedure using a Vickers ligament release and a dome-shaped osteotomy of the radius to correct all of the aspects of the radial deformity, including the radial and volar translation of the distal metaphysis. This procedure is performed with a volar approach, and the Vickers ligament is excised entirely. A dome-shaped osteotomy is made in the metaphysis so that the articular surface not only decreases in its radial inclination but also translates as it rotates. This translation reestablishes articular support to the lunate. A second maneuver translates the articular surface dorsally. The prominent volar bone is then removed, and the osteotomy is pinned into position with 2 Steinman pins. It not only corrects the deformity but also decreases pain and increases range of motion. See the images below.

Radioulnar length adjustment

In MD, the ulna grows normally and becomes longer than the radius. Because the radius is volar, the ulna appears to be subluxed dorsally. The incongruence at the distal radioulnar joint and the impingement of the radius on the ulna in supination may cause pain and contribute to decreased range of motion in supination. To allow unrestricted rotation, several ulnar procedures have been described. These include ulnar shortening, ulnar head resection and a Sauve-Kapandji-type (Lauenstein) DRUJ arthrodesis, and ulnar pseudoarthrosis. Several authors have advocated both radial and ulnar procedures.

Ulnar resection

The Darrach procedure long has been a treatment option for MD. This construct in isolation may leave the carpus unstable, especially in light of the increased ulnar and volar slope of the radial articular surface. The carpus therefore tends to slide off of the ulnar side of the wrist. Several authors have devised procedures to solve this problem. The Sauve-Kapandji (Lauenstein) procedure may be a viable option for MD in that when the ulnar head is preserved, less chance exists for ulnar migration of carpus.

In 1975, Ranawat studied 13 wrists in 8 patients with MD. His operative indications were pain and limitation of motion. He did not consider deformity alone to be an indication for operation. Mild deformity was treated with a Darrach procedure, and severe deformity was treated with Darrach plus a biplane radial osteotomy. In patients in whom a Darrach procedure was performed, the carpus tended to translate ulnarward. A radial osteotomy was theorized to improve the muscle balance about the wrist and to provide better axial bone support for the carpus on the radius in the absence of the ulnar head.

In 1993, Watson et al described another combined procedure in which the radial osteotomy was performed with both a closing wedge technique on the radial aspect of the metaphysis and an opening wedge on the ulnar aspect with the radial bone wedge. This technique preserved radial length but also required an ulnar head resection. Pain was significantly relieved in all 15 wrists.

White and Weiland described a combination procedure in a series of wrists with MD of posttraumatic etiology. The procedure included a volar closing wedge osteotomy along with a distal radioulnar arthrodesis and ulnar pseudoarthrosis proximal to the arthrodesis. The osteotomy of the radius improved the mechanics of the radiocarpal joint and prevented radiocarpal arthritis. This procedure alone may not improve pain on supination/pronation due to the incongruent distal radioulnar joint. Resection of the distal ulna as in the Darrach procedure may solve this problem, but ulnar translation of the carpus may result. Fusing the DRUJ maintained carpal stability. Increase in the patient's range of motion was minimal in any plane, but pain was significantly relieved, the carpus was stable, and the cosmetic result was satisfactory.

In the skeletally mature patient who presents with pain and in whom physical examination demonstrates limitation of motion and severe radiocarpal joint incongruity, a salvage procedure may be indicated. When incongruity is severe, any surgical manipulation of the joint surfaces would be difficult and the joint would not be stable for smooth articulation. For long-term pain relief and a stable joint, a radiocarpal arthrodesis and distal ulna resection is indicated.

Postoperative management depends on what is done operatively. Six to 8 weeks of cast immobilization is necessary following an osteotomy of the radius or ulna to allow for bony healing. Hand therapy is necessary in children who are not able to regain range of motion on their own after 2-3 months without restrictions.

Monitor these children until surgical issues are resolved and then yearly until skeletal maturity is reached. Following a physiolysis procedure, yearly radiographs can be used to document improvement in the position of the distal radial articular surface with growth.

In the author's experience, no specific complications after operative correction have arisen as problematic. However, isolated cases of postoperative wound infections, reflex sympathetic dystrophy, and recurrent deformity after continued growth are reported in the literature.

Goals for surgical correction of MD consist primarily of pain relief and correction of the cosmetic deformity. A secondary goal is to increase range of motion. Most patients who undergo biplane osteotomy with ulnar length adjustment or the crescentic radial osteotomy of Carter and Ezaki attain both primary goals.^[19] Range of motion usually is only moderately improved at best. It should be noted that pain relief can be substantial and can be achieved quickly following release of the volar Vickers ligament.

Genetic links of MD to the SHOX gene on the X chromosome are emerging. No simple Mendelian inheritance pattern exists, so more than one gene most likely is involved. The interaction of the SHOX locus with other genes and transcription factors is not known and will be an area of future research. Results may provide a more complete answer to the link between dyschondrosteosis and MD.

Historically, surgical treatment of MD has been a closing wedge biplane osteotomy in conjunction with an ulnar shortening osteotomy of the distal ulna. Recently, a new procedure that addresses the deformity with a dome-shaped osteotomy, a release of Vickers ligament, and a reduction of the DRUJ without an ulnar procedure has been successfully used. This procedure shows great promise.