The patient with Madelung’s deformity has a characteristic position of the upper extremity. In most cases, the ulna appears prominent with the carpus and hand volarly translated. Pain over the ulna may be present with some decrease in forearm supination and wrist extension. The forearms themselves may be short. Plain radiographs can show a spectrum of deformity ranging from minimal increase in radial inclination to substantially increased radial inclination along with lunate subsidence (Fig. 3a, b). McCarroll et al. (2010) reported that radiographic criteria for the diagnosis included ulnar tilt of 33° or greater, lunate subsidence of 4 mm or more, lunate fossa angle of 40° or greater, and palmar carpal displacement of 20 mm or more. In the skeletally immature patient, premature closure of the volar-ulnar radial physis is noted. Radiographs of the entire forearm to include the elbows are important as whole-bone involvement of the radius has been documented and has implications on eventual outcomes (Zebala et al. 2007; Fig. 4). CT and MRI imaging can add a three-dimensional view of the deformity, but is usually not necessary to establish or treat the Madelung’s deformity patient.

Vickers and Nielsen (1992) described a thick fibrous structure (the so-called Vickers’ ligament ), which begins on the ulnovolar metaphyseal region of the radius and attaches to the lunate and triangular fibrocartilage. Whether this anomalous structure is truly an additional structure or just prominence of the volar wrist ligamentous structures is subject of debate, but its presence has been reported in up to 91 % of patients (Vickers and Nielsen 1992; Nielsen 1977). Some authors believe that release of this structure during operative intervention is an integral part of the surgery (Vickers and Nielsen 1992; Nielsen 1977; Harley et al. 2006; Steinman et al. 2013). However, it is doubtful that the potential tethering effect of the “ligament” is the primary cause of deformity. Madelung’s deformity is not seen in the child less than 3 years of age (a period where rapid growth takes place).

Symptoms that warrant consideration for surgery in most patients include pain and unacceptable deformity. Early reports on operative techniques centered solely on the ulna with varying success (Ranawat et al. 1975; Glard et al. 2007; Bruno et al. 2003). Most surgical treatment now more logically addresses the radius as the deformity appears to be inherent to this bone (Vickers and Nielsen 1992; Harley et al. 2002, 2006; Steinman et al. 2013; Murphy et al. 1996). Age plays an important role with respect to which procedure is chosen. Vickers and Nielsen (1992) have reported excellent success with ligament release and physiolysis in those skeletally immature patients who have considerable growth potential remaining. In the patient with substantial deformity and not much forearm growth remaining, corrective radial osteotomy with ligament release is warranted.

After induction of general anesthesia and under tourniquet control, a longitudinal incision is placed over the flexor carpi radialis tendon (FCR) overlying the distal radius. Next, dissection is carried down to the pronator quadratus where the distal portion is divided to allow exposure of the abnormal radio-lunate ligament (Vickers’). It is easily identified in a concavity in the metaphyseal portion of the radius. This is carefully released from proximal to distal until the tethering of the lunate is released. This is usually readily apparent and results in being able to visualize the undersurface of the lunate. Next, physiolysis is performed as described initially by Langenskiold and later by Vickers (Vickers and Nielsen 1992). The down-turned metaphysis at the distal volar-ulnar radius is carefully trimmed to expose the remaining growth plate. Using a burr or rongeur, the abnormal physis is removed until more normal-appearing physeal cartilage is identified. Adequate resection is imperative to the success of this procedure. At this point, the area is then packed with a generous amount of autogenous fat that can be harvested from a number of donor sites (Fig. 6). It is important for this fat to make contact with the entire cavity that was created. Layered closure is performed and the extremity is immobilized for 3–4 weeks. After removal of the cast, range-of-motion (ROM) exercises are instituted and serial radiographs are performed to access growth and alignment (Fig. 7a, b, c).