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Radial opening wedge osteotomy in Madelung's deformity
Radial Opening Wedge Osteotomy in Madelung's Deformity Michael S. Murphy, MD, Ronald L. Linscheid, MD, James H. Dobyns, MD, Hamlet A. Peterson, MD, Rochester, MN Radiopalmar opening wedge osteotomy was used in 11 female patients (12 wrists) with Madelung's deformity who ranged in age from 9 to 31 years. Five patients met the radiographic criteria for generalized dyschondrosteosis. Clinically, there was dissatisfaction with the appearance of the wrist. The principal motion deficits were in radiocarpal extension and forearm pronosupination associated with varying degrees of discomfort with sustained activity. All had a decreased radioulnar angle, lunate subluxation shown radiographically, and
various degrees of dorsal subluxation of the ulnar head. Indications for surgery were pain, limited motion, cosmetic appearance, and progressive deformity in two immature patients. Biplanar corrective opening wedge osteotomy of the radius was performed with iliac crest graft. Three patients had an added ulnar recession. Fixation was with a plate or Kirschner wire. Supplementary distracting external fixation was used in six cases. The follow-up period averaged 48 months. Pain relief in all patients was satisfactory. Range of motion and grip strength were not influenced. The radioulnar angle improved by 11~ but lunate subluxation was minimally changed. Length of the forearm and bowed appearance of the forearm were improved. Reduction of the radioulnar joints was improved but remained incongruous. (J Hand Surg 1996;21A:1035-1044.)
Madelung's deformity is a congenital abnormality that is often the localized manifestation of a systemic syndrome caused by a growth disturbance of the ulnopalmar portions of the distal radius epiphysis and physis, a Madelung is credited with the original description in 1878, 2,3 although he noted earlier descriptions by Dupuytren in 1834 and Malgaigne in 1855. Management of symptomatic Madelung's deformity of the wrist can be a formidable problem. No ideal treatment has been described. Conservative measures directed toward activity modification and splinting have not prevented progression of symptoms or deformity. Treatments based on ulnar shortening, 4 Milch cuff resection in children, 5 or Darrach
From the Department of Orthopedics, Mayo Clinic and Mayo Foundation, Rochester, MN. Received for publication Oct. 18, 1994; accepted in revised form April 1, 1996. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of the article. Reprint requests: Ronald L. Linscheid, MD, Mayo Clinic, 200, First Street SW, Rochester, MN 55905.
resection in adults 6 are traditional forms of treatment. Long-term problems with these procedures, such as ulnar carpal translation, have been recognized. 7 We report our experience with biplanar radial opening wedge osteotomy as a treatment modality. This procedure, rather than being ablative, is directed toward restoring a more normal anatomy in order to relieve pain, aid function, and improve appearance.
Materials and Methods Clinical Presentation Eleven female patients (12 wrists) were selected for corrective osteotomy on the basis of symptoms and the degree of wrist deformity. All had a history of progressively disabling wrist pain. The average time from diagnosis to operation was 5 years (range, 1-11 years). The average age of patients was 16 years (range, 9 to 31 years). Bilateral involvement and a family history of Madelung's deformity were present in all. Five patients met the radiographic criteria for a generalized dyschondrosteosis. This included short stature and diminished tibial length as The Journal of Hand Surgery
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1036 Murphy et al. / Madelung's Deformity well as shortened forearms and the typical wrist deformities. Two patients also had antecedent radius fractures at ages 6 and 7 years and two patients were skeletally immature. One patient with bilateral Madelung's deformity underwent a preliminary resection of a distal projecting ulnar and pahnar corner of the radius incorrectly assumed to be radial osteochondroma, but subsequent progressive deformity resulted in a corrective osteotomy. Clinically, the forearm was foreshortened and the hand appeared ulnarly displaced. In the more severe cases, the distal radius was bowed ulnarly and palmarly and the ulnar head was displaced dorsodistally in relation to the ulnar aspect of the carpus. A decreased range of motion was found in all cases. Madelung's deformity was confirmed with the radiographic criteria suggested by Dannenberg et al. a These included radial shortening with a palmar and radial bowing, dysplasia of the lunate fossa with secondary remodeling of the carpus to fit the altered radioulnar contours, ulnar-sided epiphyseodesis of the radius, subluxation of the ulnar head dorsodistally, and distortion and widening of the ulnar head. Changes in the proximal radius, including widening of the radial head, are uncommon and were noted only once in this series. In addition, the diagnostic variables of radioulnar angle, third metacarpal angle, and lunate subluxation, as outlined by Ranawat et al., 7 were abnormal (Fig. 1). With this technique] the position of the lunate in relation to the articular cartilage of the radius is deemed normal when there is contact between at least 50% of the proximal lunate surface and the ossified portion of the radius. We measured the percentage of lunate in contact with the articular surface of the radius. The ulnar border of the radius was used as the point of reference on a standard posteroanterior forearm film. The percentage of lunate radial to this axis was considered to be radiolunate contact. Preoperative radioulnar inclination was determined radiographically as the angle between the articular surface of the radius and a line drawn down the longitudinal axis of the radius as seen on a standard posteroanterior film. A value of 60 ~ or greater is considered normal. 7 Forearm alignment relative to the third metacarpal is considered normal if the angle is less than 50.7 Preoperatively, the third metacarpal angle averaged 11 ~ (range, 8~176 This measurement appears to be of little consequence, and further use is not planned. The position of the lunate in the normal wrist is considered adequate if there is contact between at least half of its proximal articular surface and the
Figure 1. Determination of the radioulnar inclination angle.
radius. 7 Preoperatively, the average contact was 30%. Ulnar variance was difficult to measure because of the distortion of both the ulnar heads and radial articular surfaces. A line tangent to the ulnar head and a line intersecting the articular slope of the radius at its ulnar margin were drawn perpendicular to the longitudinal axis of the ulna. The distance between the lines was then measured. Criteria for surgery included cosmetic deformity; foreshortened forearms; limitation of motion, particularly supination; pain with activity; dorsal ulnar projection; and progressive deformity in a skeletally immature patient.
Surgical Technique Exposure was performed through a longitudinal palmar approach, exploiting the interval between the flexor carpi radialis and the radial artery or the interval between the ulnar neurovascular bundle and the finger flexors. The latter exposure allowed both the reflection of the pronator quadratus fi'om the ulna and the exposure of the ulnopalmar aspect of the distal radius. A large aberrant muscle thought to be a pronator quadratus appeared to tether the ulnar and palmar aspects of the radial epiphysis in one case. 9 In others, a stout fibrous cord appeared to tether the ulnar and palmar aspects of the epiphysis, but whether this was an etiologic restraint to physeal
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B A
C Figure 2. Corrective osteotomy and trapezoidal graft. (A) Palmar exposure reflecting pronator quadratus. (B) Alignment osteotomy cuts in mature individuals. Osteotomies are spread open with an osteotome. (C) Biplanar trapezoidal iliac crest bone grafts are inserted and bone plates are applied. (By permission of Mayo Foundation.)
growth or a remnant of periosteal sleeve that did not attenuate during physeal growth is indeterminate. 3 The metaphyseal area was cleared to the midpoint of the radius. In the skeletally immature patient, the physeal line was identified. An osteotome was placed parallel and 1 cm proximal to the rim of the lunate fossa and angled dorsodistally at the angle of the lunate fossa, as seen radiographically. A Kirschner wire (K-wire) was drilled into the appropriate spot for confirmation under the image intensifier, if doubt existed as to position. The osteotomy was then performed under fluoroscopic control and the epiphysis was gently wedged open to produce a biplanar correction. In 6 of 11 patients, external fixation provided radiocarpal distraction and facilitated graft insertion. A corticocancellous trapezoidal graft was fashioned from the iliac crest 1~(Fig. 2). The correction of the radioulnar inclination and the palmar tilt was checked radiographically. K-wires and AO buttress plates were used for fixation. In two cases, ulnar recession was performed to correct excessive ulna-positive variance. This allowed the dorsal overriding of the ulnar head on the carpus and the dorsal subluxation of the distal radioulnar joint to be corrected. The ulnas were fixed with six-hole AO plates. Three patients had bowing of the radius sufficient to justify an additional, more proximal osteotomy. Two of these patients were skeletatly immature and had
severe deformity as the result of a generalized dyschondrosteosis. In these two patients, the initial osteotomy was placed under the rim of the radius,
MAYO 9 t~,4
Figure 3. Technique for physeal osteotomy. In the immature radius, the physis is identified as a thin white line. The osteotomy is directed from the ulnopalmar cul-de-sac to the ulnar aspect of the physis before wedging open with interpositional material. (By permission of Mayo Foundation.)
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Murphy et aL. / Madelung's Deformity
aiming for the ulnar aspect of the identifiable intact physis or physeal scar. Silicone blocks were fashioned in a trapezoidal configuration to achieve biplanar correction (Fig. 3). These silicone blocks were inserted as temporary supports to the lunate fossa while radiophyseal growth occurred (Fig. 4). These were abutted against the ulna in an attempt to prevent their extrusion. A second more proximal opening wedge osteotomy was then created through the shaft of the radius to help correct radial bowing and to enhance radial length. Standard corticocancellous iliac crest grafts were placed in these more proximal osteotomies. Fixation was achieved with AO plates.
Results The follow-up period averaged 48 months (range, 13-97 months). All patients were examined clinically and radiographically. They were generally pleased with the pain relief and indicated the arm had increased functional usefulness in daily activities. They were also pleased with the improved appearance of the forearm, although the scars in a few instances were widened enough to be of concern to them. The prominence of the ulnar head was diminished in each instance. Congruity of the distal radioulnar joint was seldom achieved because of the developmental distortion of the ulnar head and dysplasia of the sigmoid notch area. However, the excessive translation of the ulnar head during pronosupina-
tion was diminished. Only one patient complained of mild discomfort at this joint with strenuous activity (Figs. 5, 6). Preoperatively, the largest deficit was in dorsiflexion, which averaged 47 ~ (range, 30~176 Palmar flexion averaged 54 ~ (range, 20~176 Radial deviation averaged 10~ (range, 0~176 Ulnar deviation averaged 41 ~ (range, 20~176 Pronation averaged 56 ~ (range, 10~ ~ and supination averaged 66 ~ (range, 10~176 Normalized grip strength averaged 68% of the expected (range, 48%-100%). Postoperative range of motion and grip strength values were essentially unchanged from preoperative values. Preoperatively, radioulnar inclination averaged 34 ~ (range, 23~ ~) and postoperatively, 45 ~ (range, 30~176 Preoperatively, the radiolunate contact area averaged 30% (range, 10%-50%) and postoperatively, 35% (range, 15%-50%). In one immature patient, a 7mm growth spurt of the radius was noted by the third postoperative month. Ulnar variance preoperatively ranged from +3 to +15 mm; postoperatively, this improved to being at or between neutral and +6 mm. In the more severe cases, the distortion of the ulnar head made precise ulnar variance correction less important because the sloped distal ulnar surface helped support the carpus. Complications There were no nonunions or deep infections. Two skeletally immature patients underwent repeat
A B Figure 4. (A) Radiographic appearance of classic Madelung's deformity in a 9-year-old girl with dyschondrosteosis. (B) Clinical appearance of the forearm. (Figure continues)
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g D Figure 4. (continued) (C) The pronator teres has an abnormal insertion onto the ulnopalmar corner of the radial epiphysis, which appeared to be tethering that side of the physis. This is represented by the muscle mass lying above the lower retractor. (D) A double osteotomy with external fixator in place. A triangular piece of silicone block was inserted in the physeal cut and anchored with a Kirschner wire. (Figure continues)
opening wedge osteotomy as progressive loss of correction occurred over 3- and 4-year periods with growth. These were the only skeletally immature patients in the series. In the initial operation, both had required double osteotomies for adequate correction, with the distal osteotomy distracted by a silicone wedge. In each case, there was gradual loss of correction, recurrent deformity with growth, and silicone wedge extrusion. Uncomplicated corrective osteotomy was repeated through the previous site after skeletal maturity. One of these patients required subsequent hardware removal, for a total of two additional procedures. One patient who had opening wedge osteotomy of the radius with accompanying ulnar recession subsequently had ulnar plate removal. A displaced fracture occurred through the osteotomy site, which required open reduction, bone grafting, repeat plating, and later plate extraction. One patient required a radiolunar arthrodesis as a salvage procedure. Initially, she underwent an excision of a supposed distal radius osteochondroma with resection of a distal radius physeal bar. The painful deformity progressed. A biplanar corrective osteotomy with corticocancellous bone grafting was performed when she was 12 years old. In spite of initial satisfactory correction, the graft collapsed and inadequate lunate support allowed palmar lunate subluxation. This subluxation may have been abetted by capsular release at the index procedure. An isolated radiolunate arthrodesis with an iliac strut graft
restored alignment, improved length, and relieved her discomfort.
Discussion The distal radius ossific nucleus appears with some degree of variability.11 At 6 years of age, the epiphysis begins to flatten, and at 8 years of age, it extends radially to form the styloid, which is usually fully ossified by age 10 years. From this point, the ulnar portion of the epiphysis is thinner than the radial but continues to gain depth until skeletal maturity. 1,12 In Madelung's deformity, the normal wedge configuration of the distal radius is greatly accentuated. In many instances, its ulnar portion will fail to ossify. 12 The increasing obliquity of the articular slope of the radius induces the lunate to displace ulnarly and palmarly. Secondary developmental deformation of the lunate occurs as the result of inadequate proximal support. An epiphysiodesis of the ulnopalmar aspect of the physis becomes apparent. The continued growth of the radial aspect is responsible for the increasing deformity. The age at onset of this epiphysiodesis determines the severity of the deformity (ie, more proximal bowing and greater radioulnar length disparity). Dorsal ulnar head displacement is the combined effect of the ulnopalmar displacement of the carpus and the overriding of the ulnar head with continued growth.
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E Figure 4. (continued) (E) Radiographs of the same patient taken 11 years 8 months and 6 weeks after a second diaphyseal open wedge osteotomy.
The pathologic process that causes this disturbance in physeal growth is as yet unrecognized.~ Vickers and Nielsen 13 have identified a fibrous tether
A
at the ulnopalmar aspect of the radius extending into the radiocarpal ligaments. They also analyzed pathologic specimens from the areas of physeal arrest. They described a disorganized columnar pattern similar to that seen in achondroplasia. In this series, both an abnormal pronator quadratus insertion and fibrous bands tethering the ulnopalmar rim of the radius were encountered. This has been compared with tibia vara of Blount's disease. Dannenberg et al. s described 12 diagnostic criteria frequently found with Madelung's deformity. The most constant are (1) increased dorsal and radial convexity of the distal radius, (2) exaggerated palmar and ulnar tilt of the distal articular surface of the radius, (3) pyramiding of the carpal bones, (4) a widened interosseous space, and (5) a relative dorsal position assumed by the ulnar head. Frequently, there is pyramiding of the carpal bones, with the apex situated on the proximally subluxed lunate. This deformity of the proximal carpal row is adaptive owing to the lack of central support from the radius as the lunate intrudes into the space between the deficient lunate fossa and the ulnar head. Madelung's deformity is a hereditary disorder transmitted as an autosomal-dominant trait with incomplete penetrance. Sporadic cases can occur.l, 14-16
B
Figure 5. (A) Preoperative clinical appearance of a 15-year-old student, her 11-year-old sister, and 39-year-old mother (grandfather also had Madelung's deformities bilaterally). Discomfort increased with supination and strenuous activities. (B) Preoperative radiographs show epiphysiodesis of the ulnar aspect of the radial physis, ulnar-plus development, radial shortening, and bowing. The radioulnar angle is 50~
The Journal of Hand Surgery/Vol. 21A No. 6 November 1996
It is m o s t c o m m o n l y seen as a bilateral process in young female patients. Madelung's deformity can be one of the local manifestations of dyschondrosteosis or Leri-Weill disease.la2a 7 This is the most c o m m o n form of mesomelic dwarfism and consists of mild shortness of stature, shortness of the middle seg-
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ments of the upper and lower limbs, and Madelung's deformity.l,naTa 8 Five patients in this series satisfied these criteria on a skeletal survey. Radial head subluxation, which is frequently seen in these patients as a result of the increased radial bowing, was excluded radiographically. 12
A
C
D
Figure 6. Same patient as ir/Figure 5. (A) A double osteotomy was performed with distraction by an external fixator and internal fixation with a buttress plate and a Kirschner wire. (B) Radiograph at 3 years after surgery still shows residual bowing, ulna-plus variance, 48 ~ radioulnar angle, and deformed carpus. The radioulnar joint has, however, remained reduced, the carpus is stable on the forearm, there is a full range of motion, and there is little or no discomfort with activities at 5 years after surgery. (C) Pronation. (D) Supination right forearm. (Figurecontinues)
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Murphy et al. / Madelung's Deformity
E Figure 6. (continued) (E) Appearance on right improved with decreased bowing, less prominence of ulnar head, and more length in forearm.
Other bone dysplasias that can lead a patient to present with similar radial deformity include multiple hereditary exostoses, multiple epiphyseal dysplasia, enchondromatosis (Ollier's disease), and Turner's XO
syndrome (gonadal dysgenesis).J Rickets, juvenile rheumatoid arthritis, and infection should also be eliminated as possibilities. Premature radial epiphysiodesis is increasingly seen in young women gymnasts as pseudo-Madelung's syndrome. 4 Mild Madelung's deformity after skeletal maturity can usually be managed by nonoperative means. Patients with severe wrist deformities and disabling pain are candidates for surgical intervention. Previous efforts toward correcting the disparity in radial and ulnar length have included ulnar resection, recession, or epiphysiodesis.3,6,7,9a 9 These procedures do not address the abnormal radiocarpal relationship and may promote ulnar carpal translation.I, 7 Radiocarpal and radioulnar arthrodesis, which sacrifice wrist motion, have been proposed as late surgical measures in the symptomatic patient.ll,12, 20 In this series, an isolated radiolunate arthrodesis provided a satisfactory salvage procedure. Other authors have suggested a radial or lateral closing wedge osteotomy of the radius, with or without ulnar resection.7,19, 20 This sacrifices already deficient radial length and usually requires ulnar resection to achieve a functional relationship at the sigmoid notch of the radius and ulnocarpal joint. A simple lateral closing wedge does not address the abnormal radiopalmar inclination. Ranawat et al. v addressed this by performing both a lateral and a dorsal closing wedge osteotomy in conjunction with a distal ulnar resection. This approach addresses
A B Figure 7. Osteotomy in a less severe deformity (a small plate holds the trapezoidal graft beneath the lunate fossa) in a 16-yearold gymnast and competitive athlete who has had increasing pain with activities. (A) Preoperative radiograph. Note deformity associated with premature epiphysiodesis of the lunate fossa portion of the physis. There was minimal carpal deformity but a 4-mm ulna-plus variance. (B) The lunate fossa was elevated through an ulnopalmar exposure and the trapezoidal iliac graft secured with a small buttress plate. Healing was uneventful and full activity was allowed at 12 weeks.
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both aspects of the deformity but sacrifices radial length and the stabilizing action of the distal ulna. ~~ Watson et al. 21 recently reported a balanced radial-sided closing and ulnar-sided opening wedge osteotomy in conjunction with matched ulnar arthroplasty. Follow-up evaluation was limited but satisfactory results were reported. This procedure does not address lost radial length or excessive palmar tilt. They reported fewer long-term problems with the distal radioulnar joint when a simultaneous matched arthroplasty was performed. By contrast, our experience suggests that preservation of the ulnar head and its relocation in a position of support under the carpus are desirable. Only one patient had mild radioulnar discomfort postoperatively. Ideally, one would like to initiate correction before physeal closure so that secondary adaptive deformity to the lunate and proximal row could be lessened. Vickers and colleagues 13,22-24 reported excellent results with early physiolysis. Their series consisted of a more immature population--one with an average age of 12 years. Through a transverse palmar incision, the abnormal ligamentous tethering was released and occasionally the physeal bar was excised. Twenty-four wrists in 17 patients were treated in this fashion. Three of the more mature patients required radial osteotomy as well. The follow-up period ranged from 15 months to 12 years, with 9 patients at skeletal maturity. Pain relief and cosmesis improved. Range of motion, particularly supination, improved. There were no significant complications. Partial restoration of growth in the physis proximal to the lunate fossa and rounding of the lunate were noted. The rationale for the present procedure is based on the premise that an ulnopalmar approach to the distal radius allows a biplanar osteotomy to restore the lunate fossa articular surface to a position where it can provide increased lunate support. This also allows resection of the soft tissue tether as well as inspection and restoration of the distal radioulnar joint. Radial length is enhanced rather than shortened. It also permits an additional more proximal osteotomy to decrease radial bowing in the severe deformities. Intraoperatively, the site of osteotomy was confirmed radiographically, because its position was critical to achieving adequate correction. If the osteotomy is too distal, the articular surface of the distal radius can be entered inadvertently (Fig. 7). Polyaxial tomography was often helpful in preoperative assessment.
104-3
Adequate contouring of the iliac graft requires preoperative planning to create a satisfactory biplanar correction. Complete restoration of normal anatomy is precluded by the marked abnormal bony architecture, but partial correction is attainable. Surgical judgment must be exercised and a reasonable compromise struck. It may not be possible to restore the active growth of the physis beneath the lunate fossa once partial epiphysiodesis has occurred. If an osteotomy is carried to the active physis proximal to the scaphoid fossa and bony closure is prevented by interposition of inert material, the extent of the deformity may be diminished. In the two cases in which this was performed by using silicone wedge grafts, continued longitudinal growth of the physis of the distal radius occurred; however, the improvement was partially lost as growth overtook the correction and the silicone block partially extruded. Therefore, it may be advantageous to block the correction open with a bone graft and consider a repeat procedure later if it is indicated. Alternatively, a Vickers procedure may be considered, a3,22-24 It is unlikely that remodeling of the carpus to normality could be achieved, but perhaps it could be improved. In the skeletally mature, restoration of the lunate fossa must introduce decreased radiocarpal congruency, but the diminished shear stress across the slope of the radiocarpal joint should better distribute joint compressive stress. It is also unlikely that normal congruency of the distal radioulnar joint can be restored, but fortunately this joint is quite tolerant of incongruency because of the minimal transverse compressive load. In the event that the ulna is more than 2-3 mm longer than the correction obtainable for the radius, ulnar recession offers a solution to correct the ulnocarpal and radioulnar dissociation. The triangular fibrocartilage is left intact as a support for the ulnar carpus. In six patients in our series, an external fixator was used to distract the carpus from the distally levered lunate fossa. This aided the healing of the bone graft. This external fixator was generally left in place for 6-8 weeks. In a few instances, the ulna was pinned to the radius with a 0.062 K-wire to facilitate the distal radioulnar joint reduction and capsular repair. The distal radioulnar relationship can often be restored even if the ulnar head is dorsally dislocated. Double osteotomy of the radius, ulnar recession, manipulation of the carpus, and temporary transfix-
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Murphy et al. / Madelung's Deformity
ion of the ulna and carpus aid correction. Biomechanical studies support the value of restoring the normal architecture of the radiocarpal articulationY, 26 Retention and maintenance of the ulnar head resists ulnar carpal translation and excessive stress across the radiocarpal joint. 4 Intraoperatively, the radioulnar joint is visualized and its congruity is assessed. Three patients in this series did require ulnar recession in order to reduce the radioulnar articulation. Biplanar opening wedge osteotomy is a reasonable surgical option in a select group of patients with Madelung's deformity who experience disabling pain at the radiocarpal and radioulnar joints. The surgical procedure is directed toward correction of the abnormal anatomy and is based on biomechanical principles. Length of the radius is improved by an opening wedge osteotomy that aids restoration of the disrupted distal radioulnar joint. In those cases that could not be adequately reduced, recession of the ulna helped maintain more normal mechanics. The procedure is technically demanding. The work of Vickers et al. n,22-24 supports the early aggressive use of physiolysis in the immature child. Skeletally mature patients have diminished potential for remodeling, but their procedures are technically easier. Release of the epiphysiodesis in the skeletally immature patient poses a problem in maintenance of the opening wedge but does release the tether to growth of the remaining active portion of the physis. A later repeat operation may be necessary. Correction of the secondary malformation of the carpus is always incomplete.
References I. Tachdjian MO. Pediatric orthopedics. Vol. 1, 2nd ed. Philadelphia: WB Saunders, 1994:186-189. 2. Christ E Ulno-volar bayonet hand: its differential diagnosis from Madelung's deformity. ROFO Fortschr Geb Rontgenstr Nuklearmedizin 1981 ;134:426-430. 3. Madelung V. Die spontane Subluxation der Hand nach vorne. Verb Dtsch Ges Chir 1878;7:259-76; Arch Klin Chir 1979;23:395-396. 4. Darrow JC Jr, Linscheid RL, Dobyns JH et al. Distal ulnar recession for disorders of the distal radioulnar joint. J Hand Surg 1985;10A:482-491. 5. Milch H. Cuff resection of the ulna for malunited Colles' fracture. J Bone Joint Surg 1941;23:311-313. 6. Darrach W. Habitual forward dislocation of the head of the ulna. Ann Surg 1913;57:928-930.
7. Ranawat CS, DeFiore J, Straub LR. Madelung's de~brmity: an end-result study of surgical treatment. J Bone Joint Surg Am 1975;57:772-775. 8. Dannenberg M, Anton JI, Spiegel MB. Madelung's deformity: consideration of its roentgenological diagnostic criteria. AJR Am J Roentgenol 1939;42:671-676. 9. Dobyns JH, Wood VE, Bayne LG. Congenital hand deformities. In: Green DR Hotchkiss RN, eds. Operative hand surgery. Vol. 1, 3rd ed. New York: Churchill Livingstone, 1993: 515-520. 10. Scheffer MM, Peterson HA. Opening-wedge osteotomy for angular deformities of long bones in children. J Bone Joint Surg 1994;76A:325-334. 11. Henry A, Thorburn MJ. Madelung's deformity: a clinical and cytogenetic study. J Bone Joint Surg 1967;49B:66-73. 12. Kelikian H. Congenital deformities of the hand and forearm. Philadelphia: WB Saunders, 1974: 753-779. 13. Vickers D, Nielsen G. Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg 1992;17B:401-407. 14. Beals RK, Lovrien EW. Dyschondrosteosis and Madelung's deformity: report of three kindreds and review of the literature. Clin Orthop 1976; 116:24-28. 15. Dawe C, Wynne-Davies R, Fulford GE. Clinical variation in dyschondrosteosis: a report on 13 individuals in 8 families. J Bone Joint Surg 1982;64B:377-381. 16. Golding JS, Blackburne JS. Madelung's disease of the wrist and dyschondrosteosis. J Bone Joint Surg 1976; 58B:350-352. 17. Leri A, Weill J. Une affection cong~nitale et symdtrique du d6veloppement osseux: la dyschondrostdose. Bull Mem Soc Med Hop Paris 1929;53:1491-1494. 18. Gelberman RH, Bauman T. Madelung's deformity and dyschondrosteosis. J Hand Surg 1980;5:338-340. 19. Burrows HJ. An operation for the correction of Madelung's deformity and similar conditions. Proc R Soc Med 1937:30:565-572. 20. Nielsen JB. Madelung's deformity: a follow-up study of 26 cases and a review of the literature. Acta Orthop Scand 1977;48:379-384. 21. Watson HK, Pitts EC, Herber S. Madelung's deformity: a surgical technique. ] Hand Surg 1993; 18B:601-605. 22. Vickers DW. Premature incomplete fusion of the growth plate: causes and treatment by resection (physiolysis) in fifteen cases. Aust N Z J Surg 1980;50:393-401. 23. Vickers D. Epiphysiolysis. Curr Orthop 1989;3:41-47. 24. Vickers D. Clinodactyly of the little finger: a simple operative technique for reversal of the growth abnormality. J Hand Surg 1987;12B:335-342. 25. Tsumura H, Himeno S, An K-N, Cooney WR Chao EYS. Biomechanical analysis of Kienb6ck's disease (abstract). Orthop Trans 1987;11:327. 26. Tsumura H, Himeno S, Morita H et al. The optimum correcting angle of wedge osteotomy at the distal end of the radius for Kienb6ck's disease. J Japanese Soc Surg Hand 1984; 1:435-439.
various degrees of dorsal subluxation of the ulnar head. Indications for surgery were pain, limited motion, cosmetic appearance, and progressive deformity in two immature patients. Biplanar corrective opening wedge osteotomy of the radius was performed with iliac crest graft. Three patients had an added ulnar recession. Fixation was with a plate or Kirschner wire. Supplementary distracting external fixation was used in six cases. The follow-up period averaged 48 months. Pain relief in all patients was satisfactory. Range of motion and grip strength were not influenced. The radioulnar angle improved by 11~ but lunate subluxation was minimally changed. Length of the forearm and bowed appearance of the forearm were improved. Reduction of the radioulnar joints was improved but remained incongruous. (J Hand Surg 1996;21A:1035-1044.)
Madelung's deformity is a congenital abnormality that is often the localized manifestation of a systemic syndrome caused by a growth disturbance of the ulnopalmar portions of the distal radius epiphysis and physis, a Madelung is credited with the original description in 1878, 2,3 although he noted earlier descriptions by Dupuytren in 1834 and Malgaigne in 1855. Management of symptomatic Madelung's deformity of the wrist can be a formidable problem. No ideal treatment has been described. Conservative measures directed toward activity modification and splinting have not prevented progression of symptoms or deformity. Treatments based on ulnar shortening, 4 Milch cuff resection in children, 5 or Darrach
From the Department of Orthopedics, Mayo Clinic and Mayo Foundation, Rochester, MN. Received for publication Oct. 18, 1994; accepted in revised form April 1, 1996. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of the article. Reprint requests: Ronald L. Linscheid, MD, Mayo Clinic, 200, First Street SW, Rochester, MN 55905.
resection in adults 6 are traditional forms of treatment. Long-term problems with these procedures, such as ulnar carpal translation, have been recognized. 7 We report our experience with biplanar radial opening wedge osteotomy as a treatment modality. This procedure, rather than being ablative, is directed toward restoring a more normal anatomy in order to relieve pain, aid function, and improve appearance.
Materials and Methods Clinical Presentation Eleven female patients (12 wrists) were selected for corrective osteotomy on the basis of symptoms and the degree of wrist deformity. All had a history of progressively disabling wrist pain. The average time from diagnosis to operation was 5 years (range, 1-11 years). The average age of patients was 16 years (range, 9 to 31 years). Bilateral involvement and a family history of Madelung's deformity were present in all. Five patients met the radiographic criteria for a generalized dyschondrosteosis. This included short stature and diminished tibial length as The Journal of Hand Surgery
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1036 Murphy et al. / Madelung's Deformity well as shortened forearms and the typical wrist deformities. Two patients also had antecedent radius fractures at ages 6 and 7 years and two patients were skeletally immature. One patient with bilateral Madelung's deformity underwent a preliminary resection of a distal projecting ulnar and pahnar corner of the radius incorrectly assumed to be radial osteochondroma, but subsequent progressive deformity resulted in a corrective osteotomy. Clinically, the forearm was foreshortened and the hand appeared ulnarly displaced. In the more severe cases, the distal radius was bowed ulnarly and palmarly and the ulnar head was displaced dorsodistally in relation to the ulnar aspect of the carpus. A decreased range of motion was found in all cases. Madelung's deformity was confirmed with the radiographic criteria suggested by Dannenberg et al. a These included radial shortening with a palmar and radial bowing, dysplasia of the lunate fossa with secondary remodeling of the carpus to fit the altered radioulnar contours, ulnar-sided epiphyseodesis of the radius, subluxation of the ulnar head dorsodistally, and distortion and widening of the ulnar head. Changes in the proximal radius, including widening of the radial head, are uncommon and were noted only once in this series. In addition, the diagnostic variables of radioulnar angle, third metacarpal angle, and lunate subluxation, as outlined by Ranawat et al., 7 were abnormal (Fig. 1). With this technique] the position of the lunate in relation to the articular cartilage of the radius is deemed normal when there is contact between at least 50% of the proximal lunate surface and the ossified portion of the radius. We measured the percentage of lunate in contact with the articular surface of the radius. The ulnar border of the radius was used as the point of reference on a standard posteroanterior forearm film. The percentage of lunate radial to this axis was considered to be radiolunate contact. Preoperative radioulnar inclination was determined radiographically as the angle between the articular surface of the radius and a line drawn down the longitudinal axis of the radius as seen on a standard posteroanterior film. A value of 60 ~ or greater is considered normal. 7 Forearm alignment relative to the third metacarpal is considered normal if the angle is less than 50.7 Preoperatively, the third metacarpal angle averaged 11 ~ (range, 8~176 This measurement appears to be of little consequence, and further use is not planned. The position of the lunate in the normal wrist is considered adequate if there is contact between at least half of its proximal articular surface and the
Figure 1. Determination of the radioulnar inclination angle.
radius. 7 Preoperatively, the average contact was 30%. Ulnar variance was difficult to measure because of the distortion of both the ulnar heads and radial articular surfaces. A line tangent to the ulnar head and a line intersecting the articular slope of the radius at its ulnar margin were drawn perpendicular to the longitudinal axis of the ulna. The distance between the lines was then measured. Criteria for surgery included cosmetic deformity; foreshortened forearms; limitation of motion, particularly supination; pain with activity; dorsal ulnar projection; and progressive deformity in a skeletally immature patient.
Surgical Technique Exposure was performed through a longitudinal palmar approach, exploiting the interval between the flexor carpi radialis and the radial artery or the interval between the ulnar neurovascular bundle and the finger flexors. The latter exposure allowed both the reflection of the pronator quadratus fi'om the ulna and the exposure of the ulnopalmar aspect of the distal radius. A large aberrant muscle thought to be a pronator quadratus appeared to tether the ulnar and palmar aspects of the radial epiphysis in one case. 9 In others, a stout fibrous cord appeared to tether the ulnar and palmar aspects of the epiphysis, but whether this was an etiologic restraint to physeal
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B A
C Figure 2. Corrective osteotomy and trapezoidal graft. (A) Palmar exposure reflecting pronator quadratus. (B) Alignment osteotomy cuts in mature individuals. Osteotomies are spread open with an osteotome. (C) Biplanar trapezoidal iliac crest bone grafts are inserted and bone plates are applied. (By permission of Mayo Foundation.)
growth or a remnant of periosteal sleeve that did not attenuate during physeal growth is indeterminate. 3 The metaphyseal area was cleared to the midpoint of the radius. In the skeletally immature patient, the physeal line was identified. An osteotome was placed parallel and 1 cm proximal to the rim of the lunate fossa and angled dorsodistally at the angle of the lunate fossa, as seen radiographically. A Kirschner wire (K-wire) was drilled into the appropriate spot for confirmation under the image intensifier, if doubt existed as to position. The osteotomy was then performed under fluoroscopic control and the epiphysis was gently wedged open to produce a biplanar correction. In 6 of 11 patients, external fixation provided radiocarpal distraction and facilitated graft insertion. A corticocancellous trapezoidal graft was fashioned from the iliac crest 1~(Fig. 2). The correction of the radioulnar inclination and the palmar tilt was checked radiographically. K-wires and AO buttress plates were used for fixation. In two cases, ulnar recession was performed to correct excessive ulna-positive variance. This allowed the dorsal overriding of the ulnar head on the carpus and the dorsal subluxation of the distal radioulnar joint to be corrected. The ulnas were fixed with six-hole AO plates. Three patients had bowing of the radius sufficient to justify an additional, more proximal osteotomy. Two of these patients were skeletatly immature and had
severe deformity as the result of a generalized dyschondrosteosis. In these two patients, the initial osteotomy was placed under the rim of the radius,
MAYO 9 t~,4
Figure 3. Technique for physeal osteotomy. In the immature radius, the physis is identified as a thin white line. The osteotomy is directed from the ulnopalmar cul-de-sac to the ulnar aspect of the physis before wedging open with interpositional material. (By permission of Mayo Foundation.)
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Murphy et aL. / Madelung's Deformity
aiming for the ulnar aspect of the identifiable intact physis or physeal scar. Silicone blocks were fashioned in a trapezoidal configuration to achieve biplanar correction (Fig. 3). These silicone blocks were inserted as temporary supports to the lunate fossa while radiophyseal growth occurred (Fig. 4). These were abutted against the ulna in an attempt to prevent their extrusion. A second more proximal opening wedge osteotomy was then created through the shaft of the radius to help correct radial bowing and to enhance radial length. Standard corticocancellous iliac crest grafts were placed in these more proximal osteotomies. Fixation was achieved with AO plates.
Results The follow-up period averaged 48 months (range, 13-97 months). All patients were examined clinically and radiographically. They were generally pleased with the pain relief and indicated the arm had increased functional usefulness in daily activities. They were also pleased with the improved appearance of the forearm, although the scars in a few instances were widened enough to be of concern to them. The prominence of the ulnar head was diminished in each instance. Congruity of the distal radioulnar joint was seldom achieved because of the developmental distortion of the ulnar head and dysplasia of the sigmoid notch area. However, the excessive translation of the ulnar head during pronosupina-
tion was diminished. Only one patient complained of mild discomfort at this joint with strenuous activity (Figs. 5, 6). Preoperatively, the largest deficit was in dorsiflexion, which averaged 47 ~ (range, 30~176 Palmar flexion averaged 54 ~ (range, 20~176 Radial deviation averaged 10~ (range, 0~176 Ulnar deviation averaged 41 ~ (range, 20~176 Pronation averaged 56 ~ (range, 10~ ~ and supination averaged 66 ~ (range, 10~176 Normalized grip strength averaged 68% of the expected (range, 48%-100%). Postoperative range of motion and grip strength values were essentially unchanged from preoperative values. Preoperatively, radioulnar inclination averaged 34 ~ (range, 23~ ~) and postoperatively, 45 ~ (range, 30~176 Preoperatively, the radiolunate contact area averaged 30% (range, 10%-50%) and postoperatively, 35% (range, 15%-50%). In one immature patient, a 7mm growth spurt of the radius was noted by the third postoperative month. Ulnar variance preoperatively ranged from +3 to +15 mm; postoperatively, this improved to being at or between neutral and +6 mm. In the more severe cases, the distortion of the ulnar head made precise ulnar variance correction less important because the sloped distal ulnar surface helped support the carpus. Complications There were no nonunions or deep infections. Two skeletally immature patients underwent repeat
A B Figure 4. (A) Radiographic appearance of classic Madelung's deformity in a 9-year-old girl with dyschondrosteosis. (B) Clinical appearance of the forearm. (Figure continues)
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g D Figure 4. (continued) (C) The pronator teres has an abnormal insertion onto the ulnopalmar corner of the radial epiphysis, which appeared to be tethering that side of the physis. This is represented by the muscle mass lying above the lower retractor. (D) A double osteotomy with external fixator in place. A triangular piece of silicone block was inserted in the physeal cut and anchored with a Kirschner wire. (Figure continues)
opening wedge osteotomy as progressive loss of correction occurred over 3- and 4-year periods with growth. These were the only skeletally immature patients in the series. In the initial operation, both had required double osteotomies for adequate correction, with the distal osteotomy distracted by a silicone wedge. In each case, there was gradual loss of correction, recurrent deformity with growth, and silicone wedge extrusion. Uncomplicated corrective osteotomy was repeated through the previous site after skeletal maturity. One of these patients required subsequent hardware removal, for a total of two additional procedures. One patient who had opening wedge osteotomy of the radius with accompanying ulnar recession subsequently had ulnar plate removal. A displaced fracture occurred through the osteotomy site, which required open reduction, bone grafting, repeat plating, and later plate extraction. One patient required a radiolunar arthrodesis as a salvage procedure. Initially, she underwent an excision of a supposed distal radius osteochondroma with resection of a distal radius physeal bar. The painful deformity progressed. A biplanar corrective osteotomy with corticocancellous bone grafting was performed when she was 12 years old. In spite of initial satisfactory correction, the graft collapsed and inadequate lunate support allowed palmar lunate subluxation. This subluxation may have been abetted by capsular release at the index procedure. An isolated radiolunate arthrodesis with an iliac strut graft
restored alignment, improved length, and relieved her discomfort.
Discussion The distal radius ossific nucleus appears with some degree of variability.11 At 6 years of age, the epiphysis begins to flatten, and at 8 years of age, it extends radially to form the styloid, which is usually fully ossified by age 10 years. From this point, the ulnar portion of the epiphysis is thinner than the radial but continues to gain depth until skeletal maturity. 1,12 In Madelung's deformity, the normal wedge configuration of the distal radius is greatly accentuated. In many instances, its ulnar portion will fail to ossify. 12 The increasing obliquity of the articular slope of the radius induces the lunate to displace ulnarly and palmarly. Secondary developmental deformation of the lunate occurs as the result of inadequate proximal support. An epiphysiodesis of the ulnopalmar aspect of the physis becomes apparent. The continued growth of the radial aspect is responsible for the increasing deformity. The age at onset of this epiphysiodesis determines the severity of the deformity (ie, more proximal bowing and greater radioulnar length disparity). Dorsal ulnar head displacement is the combined effect of the ulnopalmar displacement of the carpus and the overriding of the ulnar head with continued growth.
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Murphy et al. / Madelung's Deformity
E Figure 4. (continued) (E) Radiographs of the same patient taken 11 years 8 months and 6 weeks after a second diaphyseal open wedge osteotomy.
The pathologic process that causes this disturbance in physeal growth is as yet unrecognized.~ Vickers and Nielsen 13 have identified a fibrous tether
A
at the ulnopalmar aspect of the radius extending into the radiocarpal ligaments. They also analyzed pathologic specimens from the areas of physeal arrest. They described a disorganized columnar pattern similar to that seen in achondroplasia. In this series, both an abnormal pronator quadratus insertion and fibrous bands tethering the ulnopalmar rim of the radius were encountered. This has been compared with tibia vara of Blount's disease. Dannenberg et al. s described 12 diagnostic criteria frequently found with Madelung's deformity. The most constant are (1) increased dorsal and radial convexity of the distal radius, (2) exaggerated palmar and ulnar tilt of the distal articular surface of the radius, (3) pyramiding of the carpal bones, (4) a widened interosseous space, and (5) a relative dorsal position assumed by the ulnar head. Frequently, there is pyramiding of the carpal bones, with the apex situated on the proximally subluxed lunate. This deformity of the proximal carpal row is adaptive owing to the lack of central support from the radius as the lunate intrudes into the space between the deficient lunate fossa and the ulnar head. Madelung's deformity is a hereditary disorder transmitted as an autosomal-dominant trait with incomplete penetrance. Sporadic cases can occur.l, 14-16
B
Figure 5. (A) Preoperative clinical appearance of a 15-year-old student, her 11-year-old sister, and 39-year-old mother (grandfather also had Madelung's deformities bilaterally). Discomfort increased with supination and strenuous activities. (B) Preoperative radiographs show epiphysiodesis of the ulnar aspect of the radial physis, ulnar-plus development, radial shortening, and bowing. The radioulnar angle is 50~
The Journal of Hand Surgery/Vol. 21A No. 6 November 1996
It is m o s t c o m m o n l y seen as a bilateral process in young female patients. Madelung's deformity can be one of the local manifestations of dyschondrosteosis or Leri-Weill disease.la2a 7 This is the most c o m m o n form of mesomelic dwarfism and consists of mild shortness of stature, shortness of the middle seg-
1041
ments of the upper and lower limbs, and Madelung's deformity.l,naTa 8 Five patients in this series satisfied these criteria on a skeletal survey. Radial head subluxation, which is frequently seen in these patients as a result of the increased radial bowing, was excluded radiographically. 12
A
C
D
Figure 6. Same patient as ir/Figure 5. (A) A double osteotomy was performed with distraction by an external fixator and internal fixation with a buttress plate and a Kirschner wire. (B) Radiograph at 3 years after surgery still shows residual bowing, ulna-plus variance, 48 ~ radioulnar angle, and deformed carpus. The radioulnar joint has, however, remained reduced, the carpus is stable on the forearm, there is a full range of motion, and there is little or no discomfort with activities at 5 years after surgery. (C) Pronation. (D) Supination right forearm. (Figurecontinues)
1042
Murphy et al. / Madelung's Deformity
E Figure 6. (continued) (E) Appearance on right improved with decreased bowing, less prominence of ulnar head, and more length in forearm.
Other bone dysplasias that can lead a patient to present with similar radial deformity include multiple hereditary exostoses, multiple epiphyseal dysplasia, enchondromatosis (Ollier's disease), and Turner's XO
syndrome (gonadal dysgenesis).J Rickets, juvenile rheumatoid arthritis, and infection should also be eliminated as possibilities. Premature radial epiphysiodesis is increasingly seen in young women gymnasts as pseudo-Madelung's syndrome. 4 Mild Madelung's deformity after skeletal maturity can usually be managed by nonoperative means. Patients with severe wrist deformities and disabling pain are candidates for surgical intervention. Previous efforts toward correcting the disparity in radial and ulnar length have included ulnar resection, recession, or epiphysiodesis.3,6,7,9a 9 These procedures do not address the abnormal radiocarpal relationship and may promote ulnar carpal translation.I, 7 Radiocarpal and radioulnar arthrodesis, which sacrifice wrist motion, have been proposed as late surgical measures in the symptomatic patient.ll,12, 20 In this series, an isolated radiolunate arthrodesis provided a satisfactory salvage procedure. Other authors have suggested a radial or lateral closing wedge osteotomy of the radius, with or without ulnar resection.7,19, 20 This sacrifices already deficient radial length and usually requires ulnar resection to achieve a functional relationship at the sigmoid notch of the radius and ulnocarpal joint. A simple lateral closing wedge does not address the abnormal radiopalmar inclination. Ranawat et al. v addressed this by performing both a lateral and a dorsal closing wedge osteotomy in conjunction with a distal ulnar resection. This approach addresses
A B Figure 7. Osteotomy in a less severe deformity (a small plate holds the trapezoidal graft beneath the lunate fossa) in a 16-yearold gymnast and competitive athlete who has had increasing pain with activities. (A) Preoperative radiograph. Note deformity associated with premature epiphysiodesis of the lunate fossa portion of the physis. There was minimal carpal deformity but a 4-mm ulna-plus variance. (B) The lunate fossa was elevated through an ulnopalmar exposure and the trapezoidal iliac graft secured with a small buttress plate. Healing was uneventful and full activity was allowed at 12 weeks.
The Journal of Hand Surgery/Vol. 21A No. 6 November 1996
both aspects of the deformity but sacrifices radial length and the stabilizing action of the distal ulna. ~~ Watson et al. 21 recently reported a balanced radial-sided closing and ulnar-sided opening wedge osteotomy in conjunction with matched ulnar arthroplasty. Follow-up evaluation was limited but satisfactory results were reported. This procedure does not address lost radial length or excessive palmar tilt. They reported fewer long-term problems with the distal radioulnar joint when a simultaneous matched arthroplasty was performed. By contrast, our experience suggests that preservation of the ulnar head and its relocation in a position of support under the carpus are desirable. Only one patient had mild radioulnar discomfort postoperatively. Ideally, one would like to initiate correction before physeal closure so that secondary adaptive deformity to the lunate and proximal row could be lessened. Vickers and colleagues 13,22-24 reported excellent results with early physiolysis. Their series consisted of a more immature population--one with an average age of 12 years. Through a transverse palmar incision, the abnormal ligamentous tethering was released and occasionally the physeal bar was excised. Twenty-four wrists in 17 patients were treated in this fashion. Three of the more mature patients required radial osteotomy as well. The follow-up period ranged from 15 months to 12 years, with 9 patients at skeletal maturity. Pain relief and cosmesis improved. Range of motion, particularly supination, improved. There were no significant complications. Partial restoration of growth in the physis proximal to the lunate fossa and rounding of the lunate were noted. The rationale for the present procedure is based on the premise that an ulnopalmar approach to the distal radius allows a biplanar osteotomy to restore the lunate fossa articular surface to a position where it can provide increased lunate support. This also allows resection of the soft tissue tether as well as inspection and restoration of the distal radioulnar joint. Radial length is enhanced rather than shortened. It also permits an additional more proximal osteotomy to decrease radial bowing in the severe deformities. Intraoperatively, the site of osteotomy was confirmed radiographically, because its position was critical to achieving adequate correction. If the osteotomy is too distal, the articular surface of the distal radius can be entered inadvertently (Fig. 7). Polyaxial tomography was often helpful in preoperative assessment.
104-3
Adequate contouring of the iliac graft requires preoperative planning to create a satisfactory biplanar correction. Complete restoration of normal anatomy is precluded by the marked abnormal bony architecture, but partial correction is attainable. Surgical judgment must be exercised and a reasonable compromise struck. It may not be possible to restore the active growth of the physis beneath the lunate fossa once partial epiphysiodesis has occurred. If an osteotomy is carried to the active physis proximal to the scaphoid fossa and bony closure is prevented by interposition of inert material, the extent of the deformity may be diminished. In the two cases in which this was performed by using silicone wedge grafts, continued longitudinal growth of the physis of the distal radius occurred; however, the improvement was partially lost as growth overtook the correction and the silicone block partially extruded. Therefore, it may be advantageous to block the correction open with a bone graft and consider a repeat procedure later if it is indicated. Alternatively, a Vickers procedure may be considered, a3,22-24 It is unlikely that remodeling of the carpus to normality could be achieved, but perhaps it could be improved. In the skeletally mature, restoration of the lunate fossa must introduce decreased radiocarpal congruency, but the diminished shear stress across the slope of the radiocarpal joint should better distribute joint compressive stress. It is also unlikely that normal congruency of the distal radioulnar joint can be restored, but fortunately this joint is quite tolerant of incongruency because of the minimal transverse compressive load. In the event that the ulna is more than 2-3 mm longer than the correction obtainable for the radius, ulnar recession offers a solution to correct the ulnocarpal and radioulnar dissociation. The triangular fibrocartilage is left intact as a support for the ulnar carpus. In six patients in our series, an external fixator was used to distract the carpus from the distally levered lunate fossa. This aided the healing of the bone graft. This external fixator was generally left in place for 6-8 weeks. In a few instances, the ulna was pinned to the radius with a 0.062 K-wire to facilitate the distal radioulnar joint reduction and capsular repair. The distal radioulnar relationship can often be restored even if the ulnar head is dorsally dislocated. Double osteotomy of the radius, ulnar recession, manipulation of the carpus, and temporary transfix-
1044
Murphy et al. / Madelung's Deformity
ion of the ulna and carpus aid correction. Biomechanical studies support the value of restoring the normal architecture of the radiocarpal articulationY, 26 Retention and maintenance of the ulnar head resists ulnar carpal translation and excessive stress across the radiocarpal joint. 4 Intraoperatively, the radioulnar joint is visualized and its congruity is assessed. Three patients in this series did require ulnar recession in order to reduce the radioulnar articulation. Biplanar opening wedge osteotomy is a reasonable surgical option in a select group of patients with Madelung's deformity who experience disabling pain at the radiocarpal and radioulnar joints. The surgical procedure is directed toward correction of the abnormal anatomy and is based on biomechanical principles. Length of the radius is improved by an opening wedge osteotomy that aids restoration of the disrupted distal radioulnar joint. In those cases that could not be adequately reduced, recession of the ulna helped maintain more normal mechanics. The procedure is technically demanding. The work of Vickers et al. n,22-24 supports the early aggressive use of physiolysis in the immature child. Skeletally mature patients have diminished potential for remodeling, but their procedures are technically easier. Release of the epiphysiodesis in the skeletally immature patient poses a problem in maintenance of the opening wedge but does release the tether to growth of the remaining active portion of the physis. A later repeat operation may be necessary. Correction of the secondary malformation of the carpus is always incomplete.
References I. Tachdjian MO. Pediatric orthopedics. Vol. 1, 2nd ed. Philadelphia: WB Saunders, 1994:186-189. 2. Christ E Ulno-volar bayonet hand: its differential diagnosis from Madelung's deformity. ROFO Fortschr Geb Rontgenstr Nuklearmedizin 1981 ;134:426-430. 3. Madelung V. Die spontane Subluxation der Hand nach vorne. Verb Dtsch Ges Chir 1878;7:259-76; Arch Klin Chir 1979;23:395-396. 4. Darrow JC Jr, Linscheid RL, Dobyns JH et al. Distal ulnar recession for disorders of the distal radioulnar joint. J Hand Surg 1985;10A:482-491. 5. Milch H. Cuff resection of the ulna for malunited Colles' fracture. J Bone Joint Surg 1941;23:311-313. 6. Darrach W. Habitual forward dislocation of the head of the ulna. Ann Surg 1913;57:928-930.
7. Ranawat CS, DeFiore J, Straub LR. Madelung's de~brmity: an end-result study of surgical treatment. J Bone Joint Surg Am 1975;57:772-775. 8. Dannenberg M, Anton JI, Spiegel MB. Madelung's deformity: consideration of its roentgenological diagnostic criteria. AJR Am J Roentgenol 1939;42:671-676. 9. Dobyns JH, Wood VE, Bayne LG. Congenital hand deformities. In: Green DR Hotchkiss RN, eds. Operative hand surgery. Vol. 1, 3rd ed. New York: Churchill Livingstone, 1993: 515-520. 10. Scheffer MM, Peterson HA. Opening-wedge osteotomy for angular deformities of long bones in children. J Bone Joint Surg 1994;76A:325-334. 11. Henry A, Thorburn MJ. Madelung's deformity: a clinical and cytogenetic study. J Bone Joint Surg 1967;49B:66-73. 12. Kelikian H. Congenital deformities of the hand and forearm. Philadelphia: WB Saunders, 1974: 753-779. 13. Vickers D, Nielsen G. Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg 1992;17B:401-407. 14. Beals RK, Lovrien EW. Dyschondrosteosis and Madelung's deformity: report of three kindreds and review of the literature. Clin Orthop 1976; 116:24-28. 15. Dawe C, Wynne-Davies R, Fulford GE. Clinical variation in dyschondrosteosis: a report on 13 individuals in 8 families. J Bone Joint Surg 1982;64B:377-381. 16. Golding JS, Blackburne JS. Madelung's disease of the wrist and dyschondrosteosis. J Bone Joint Surg 1976; 58B:350-352. 17. Leri A, Weill J. Une affection cong~nitale et symdtrique du d6veloppement osseux: la dyschondrostdose. Bull Mem Soc Med Hop Paris 1929;53:1491-1494. 18. Gelberman RH, Bauman T. Madelung's deformity and dyschondrosteosis. J Hand Surg 1980;5:338-340. 19. Burrows HJ. An operation for the correction of Madelung's deformity and similar conditions. Proc R Soc Med 1937:30:565-572. 20. Nielsen JB. Madelung's deformity: a follow-up study of 26 cases and a review of the literature. Acta Orthop Scand 1977;48:379-384. 21. Watson HK, Pitts EC, Herber S. Madelung's deformity: a surgical technique. ] Hand Surg 1993; 18B:601-605. 22. Vickers DW. Premature incomplete fusion of the growth plate: causes and treatment by resection (physiolysis) in fifteen cases. Aust N Z J Surg 1980;50:393-401. 23. Vickers D. Epiphysiolysis. Curr Orthop 1989;3:41-47. 24. Vickers D. Clinodactyly of the little finger: a simple operative technique for reversal of the growth abnormality. J Hand Surg 1987;12B:335-342. 25. Tsumura H, Himeno S, An K-N, Cooney WR Chao EYS. Biomechanical analysis of Kienb6ck's disease (abstract). Orthop Trans 1987;11:327. 26. Tsumura H, Himeno S, Morita H et al. The optimum correcting angle of wedge osteotomy at the distal end of the radius for Kienb6ck's disease. J Japanese Soc Surg Hand 1984; 1:435-439.