Fig. 33.1
Characteristic aesthetic appearance of Madelung’s deformity
Multiple operative treatment options have been proposed, principally corrective osteotomies. However, owing to its rarity, the literature is sparse and there is little evidence to support definitive management strategies.
What Is the Natural History of Madelung’s Deformity?
Several hundred cases of MD have been presented in the literature since its first description. However, no published reports exist on the actual frequency of MD in the population or natural history [8]. This is not a surprise given the wide spectrum of clinical presentation; from completely asymptomatic in some individuals to quite disabling symptoms in others (Zebala et al. [28]). Reports have linked the osseous deformity to progressive ulnocarpal abutment, distal radio-ulna joint instability and progressive radiocarpal arthritis [8]. Three cases of extensor tendon rupture have been reported in severe and chronic cases due to abrasion on the prominent ulna head (Rondier et al. [21]; Ducloyer et al. [6]).
Is There a Role for Nonoperative Treatment?
Optimal treatment of MD is controversial particularly if patients were asymptomatic or not sufficiently symptomatic to warrant surgical treatment. The non-surgical management of patients with MD is analgesia and activity modification [26].
What Are the Operative Options?
Multiple surgical approaches have been described in the literature. Broadly, these can be considered in three categories: those involving the radius or the ulna in isolation and those involving both bones in combination. Surgery to the radius aims to address the primary pathology and/or correct the resultant deformity. Isolated procedures to the ulna treat ulna sided wrist pain that occurs secondary to ulnocarpal impingement. However, there is little clarity and no consensus within the literature to enable the development of a definitive management strategy. Evidence is limited to small case series and frequently the surgical techniques employed are not uniform and therefore drawing definitive conclusions from heterogeneous groups can be difficult. Additionally, the optimal timing of surgical intervention is not known. Surgery before physeal closure may lessen the development of secondary degenerative changes in the carpus and allow some remodelling, whereas intervention after skeletal maturity decreases the likelihood of recurrence.
Surgery to the Radius
Physiolysis
Vicker’s and Nielsen describe a physiolysis procedure with release of Vicker’s ligament, to address both proposed pathoanatomical causes of the deformity. The procedure described (also referred to as Langenskiöld technique) excises the abnormal physis in the ulna and volar portion of the distal radius with the interposition of fat. Improvements in range of motion and pain were seen in a cohort of 24 wrists in 17 patients who underwent the procedure at a mean age of 12 years. The authors advocate the need for early surgery as remodelling potential is necessary [27]. Subsequently, Ogino (Ogino et al. [19]) reported outcomes of 3 wrists in 2 patients with a mean age of 12 years. However, in 1 patient palmer shift of the carpus and radial inclination worsened. Most recently Paes described the long term (12 year) outcomes of 5 wrists in 3 patients who underwent the procedure at a mean age of 12 years. Results were poor with pain and a restriction in range of motion seen at long term follow up in all patients. Consequently, the author no longer advocates physiolysis questioning the applicability of the Langenskiöld technique, which was initially described for physeal arrest post trauma, to Madelung’s where the physis is pathological. Concerns have also been raised regarding distal radio-ulna joint degeneration that results from operating on the ulna side of the distal radius (Paes et al. [7]).
Isolated Radial Osteotomy
Isolated radial osteotomies have been described with the rationale to correct the volar and ulna angulation and lengthen the distal radius. Murphy (Murphy et al. [18]) described an opening wedge osteotomy performed through a volar approach which enabled a biplane deformity correction. Corticocancellous graft from iliac crest was used and the abnormal volar ligaments were excised. The outcomes of 12 wrists in 11 patients who underwent the procedure at a mean age of 16 years were reported. Patients were subjectively pleased with the function and cosmetic appearance of the wrist and ulna variance and radioulnar inclination also improved although no statistical analysis was provided. A subset of the cohort however also underwent an ulna osteotomy or more proximal radial osteotomy. Mallard (Mallard et al. [15]) advocates the use of a reverse wedge osteotomy in skeletally mature patients. A bone wedge is cut from the excess radial and dorsal cortical bone, reversed and placed into an osteotomy on the ulna side of the distal radius. In a case series of 11 wrists, range of motion improved and patients were satisfied with the outcome. However, 30% required an additional ulna osteotomy for ulna-carpal impingement. An llizarov technique has also been described. This facilitates lengthening of the radius in addition to correction of the angular deformity. Houshain reported favourable outcomes with regard to pain, grip strength and range of motion in two patients (Houshian et al. [11]). Similar findings are also reported by de Billy in a case series of 5 wrists (de Billy et al. [4]).
Dome osteotomies have the theoretical advantage over the above procedures of being able to achieve deformity correction in all three planes. Harley performed a radial dome osteotomy with release of Vicker’s ligament in 26 wrists from 18 patients at a mean age of 13 years. At a mean follow up of 2 years improvements in supination and extension range of motion were seen in addition to ulna tilt and lunate subsidence (Harley et al. [10]). Steinman reported the long term (11 year) follow up of the above series and concluded that radiographic deformity correction was maintained in addition to a good to excellent functional outcomes (Steinman et al. [25]).
Imai recently reported a case study of a 12 year old female with a bilateral deformity corrected by a novel customised cylindrical distal radius osteotomy. The axis of the deformity and the degree of rotation required to correct it were determined from pre-operative CT scans which allowed customised osteotomy templates to be developed. At 28 month follow up the patient was pain free and both grip strength and ROM had improved (Imai et al. [13]).
Surgery to the Ulna
Epiphysiodesis
Epiphysiodesis of the ulna can be performed in conjunction with a radial osteotomy to prevent ulna positive variance. Bak described a case of a 14 year old female who developed an early acquired Madelung like deformity from repetitive injury during training who was subsequently able to return to high level competition [1].
Isolated Ulna Osteotomy
Previously Darrach advocated resection of the ulna head either alone (or in combination with a radial osteotomy) to manage ulna sided wrist pain. However, subsequent lateral subluxation of the caprus caused concern (Ranawat et al. [20]). Schroven undertook a segmental resection of a portion of ulna shaft, just distal to the distal radio-ulna joint, to create a pseudoarthrosis with the rationale of restoring stability while retaining the ulna head (Schroven et al. [23]).
Isolated osteotomies of the ulna have been presented in the literature. These are indicated to treat ulna sided wrist pain in skeletally mature patients with a mild MD. Ulna head prominence is addressed preventing ulnocarpal abutment. Bruno performed an isolated ulna shortening osteotomy in 9 wrists in adults with symptomatic ulnocarpal impingement. At a mean follow up of 42 months ulna variance was significantly reduced (from 3.3mm to -1.1mm) and all patients were asymptomatic (Bruno et al. [2]). Subsequently, on a similar subset of patients with a mild MD, Glard performed a shortening osteotomy of the ulna with anterior angulation, the aim being to reduce the distal radioulnar joint. Post-operatively, pain and range of motion in pronation and supination improved and all patients were satisfied with the cosmetic effect. The mean volar angulation at ulna osteotomy was 17o (Glard et al. [9]).
Surgery Combined to the Radius and Ulna
Combined corrective osteotomies of the radius and ulna aim to correct the radial deformity while shortening the ulna to achieve reduction of the distal radio-ulna joint. Dos Reis described a wedge subtraction osteotomy of the radius and shortening osteotomy of the ulna. Grip strength and range of motion significantly improved at 53 month follow up in 18 patients who underwent the above procedure at a mean age of 22 years (dos Reis et al. [5]). Similarly, a closing wedge osteotomy of the radial metaphysis and shortening ulna osteotomy with slight volar angulation to reduce distal radio-ulna joint was described by Salon. Improvements in pain and range of motion were seen at 9 year follow up in 11 wrists who underwent the procedure at a mean age of 14 years. Remodelling of the distal radio-ulna joint was seen in all patients (Salon et al. [22]). Recently McCarrol presented encouraging early results from a very distal radial osteotomy with ulna shortening. The distal nature of the radial osteotomy enabled flexibility to independently correct the three plane deformity [16].