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Background: Madelung deformity arises from a partial distal radial growth disturbance in combination with an abnormal hypertrophic ligament spanning the volar radius and carpus, termed, the Vickers ligament. The purpose of this study is to report long-term clinical and radiographic outcomes following Vickers ligament release and distal radial physiolysis in a population of skeletally immature patients with symptomatic Madelung deformity., Methods: Medical records were retrospectively reviewed of patients with Madelung deformity surgically treated between 1994 and 2005. All eligible patients who underwent a Vickers ligament release and distal radial physiolysis were contacted and invited to return to the clinic for follow-up., Results: Six patients (8 wrists) with Madelung deformity underwent Vickers ligament release and distal radial physiolysis. All were white females with a mean age at initial presentation of 11.4 years (10 to 12.8 y). Mean age at the time of initial surgery was 12.0 years (10.0 to 14.5 y). The median follow-up time was 10.6 years (5.8 to 21.9 y) and the average age at last follow-up was 23.1 years (17.5 to 32.2 y). Pain alone or in combination with concerns for deformity was the chief complaint in 6 of 8 of the wrists. At 1 year of clinical follow-up, 7 of 8 wrists were reported to be pain-free, and 6 of the 8 were noted to be completely pain-free at last follow-up. Motion in flexion, extension, pronation, supination, radial, or ulnar deviation was similar between the preoperative status and long-term follow-up. The average preoperative ulnar tilt was 35.1 degrees (SD: 8.5 degrees), average preoperative lunate subsidence was 1.9 degrees (SD: 1.8 degrees), and average preoperative palmar carpal displacement was 21.9 degrees (SD: 2.9 degrees). At the final follow-up, there was a large progression in lunate subsidence, but minimal change in ulnar tilt and palmar carpal displacement. At last clinical follow-up, 2 of the 6 patients had undergone a subsequent procedure including 1 radial dome osteotomy and 1 ulnar shortening osteotomy., Conclusion: In the skeletally immature patient population with Madelung deformity with growth potential remaining, distal radial physiolysis and Vickers ligament release is associated with relief of pain, preservation of motion, and, a reasonable rate of reoperation., Type of Study: This was a therapeutic study., Level of Evidence: Level II.

Background: Centralization is commonly utilized for treating the severely deviated wrist in radial longitudinal deficiency (RLD). Individuals with RLD have congenital shortening of the ulna and previous studies have shown that traditional centralization, in particular with notching of the carpus, results in additional ulnar growth retardation. At our institution, we use a technique of soft tissue release with bilobed flap. We examined if this technique preserves the growth potential of the distal ulna, therefore, avoiding an additionally shortened forearm., Methods: We retrospectively reviewed serial radiographs of 16 patients with 18 wrists who had at least 3 years of follow-up after a soft tissue release with bilobed flap. Radiographic lengths were measured using the method described by Heikel. Percentage of normal growth was calculated using normative data published by Maresh. Comparisons were made with preoperative, postoperative, and final follow-up studies., Results: The average length of follow-up was 9.2 years (range, 3 to 16.3 y) with an average age of 11.6 years (range, 5.2 to 17.5 y). The average age at the time of surgery was 27 months (range, 14 to 48 mo). A minimum of 3 radiographic studies were available for each subject. The average ulna length preoperatively was 63.9% of age-matched normal length (51.4% to 75.3%). The average ulna length at final follow-up was 61.9% of age-matched normal length (48.5% to 70.3%). The difference was not statistically significant. In addition, there were no distal ulnar physeal arrests., Conclusions: Soft tissue release with bilobed flap does not affect ulna growth like traditional centralization procedures can. This procedure has previously been shown to retain motion and have similar recurrence rates to formal centralizations. Therefore, we advocate that it be considered more widely for use in patients with RLD and significant wrist deviation to maximize growth and improve hand position. Soft tissue release with bilobed flap can be safely used on young children and preserve ulnar growth., Level of Evidence: Level IV-therapeutic.

Purpose: To report the hand position, range of motion, functional results, and radiographic outcomes associated with treating radial longitudinal deficiency with release of constricting or deforming soft tissue and resurfacing of the radial skin deficiency with a bilobed flap., Methods: We recalled and reviewed patients with at least a 3-year follow-up who had undergone soft-tissue release and coverage with a bilobed flap. The study group consisted of 16 patients and 18 wrists. All patients underwent follow-up examination and radiographs. Outcome measures using Pediatric Outcomes Data Collection Instrument (PODCI), Disabilities of the Arm, Shoulder, and Hand (DASH), and visual analogue scale (VAS) scores were recorded., Results: At a mean of 9.2 years follow-up, the average final resting wrist radial deviation angle was 64° compared with 88° preoperatively. The average active wrist flexion-extension arc was 73°. Average DASH score was 27 (range, 5-54). PODCI global was 88 (range, 75-97), PODCI happiness was 86 (range, 70-100), and VAS overall satisfaction (range, 0-10) was 1.2 (range, 0-8). At final follow-up, no physeal growth arrests were noted on radiographs, and no patients to date have required ulnocarpal arthrodesis., Conclusions: Soft-tissue release and coverage with a bilobed flap should be considered in the treatment algorithm for patients with radial longitudinal deficiency. Outcome measures show that these patients maintain useful active motion, and along with their parents, are satisfied with both the appearance and function. Some recurrence of radial deviation was noted, which was similar to results previously reported following centralization/radialization procedures, although with a lower inherent risk of both physeal injury to the ulna and stiffness. In addition, potential future procedures are not compromised by this surgical approach., Type of Study/level of Evidence: Therapeutic IV., (Copyright © 2015 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Background: Wrist flexion and ulnar deviation deformity is a common presentation in children with amyoplasia congenita. Multiple surgical procedures have been reported to correct the deformity to enhance functional independence and improve quality of life. We performed a retrospective review to detail our long-term results with carpal wedge osteotomy in these patients., Methods: Medical records of all patients with the amyoplasia form of arthrogryposis who underwent carpal wedge osteotomy between 1994 and 2008 were reviewed. Patients with a follow-up of two years or less were excluded. Preoperative and postoperative resting position and range of motion of the wrist were recorded. Interviews and questionnaires were completed to assess the mean overall satisfaction level of the parent or guardian with the outcome of surgery, function, and task completion with use of parent-guardian surveys, the Manual Ability Classification System, and the ABILHAND-Kids measure of manual ability., Results: Seventy-five wrists in forty-six patients who met the inclusion criteria were reviewed. The average age of the patients at the time of surgery was 4.3 years (range, nine months to eighteen years; median, 2.7 years). The average duration of follow-up was 5.7 years (range, two to 10.3 years; median, 5.3 years). The average resting position of the wrist postoperatively (11° of flexion) was significantly different from that measured preoperatively (55° of flexion) (p < 0.001). The arc of wrist motion measured preoperatively (32°) did not differ significantly from that measured postoperatively (22°) (p = 0.4903). The location of the motion arc was significantly improved to a more functional position. The average active extension of the wrist changed from -37° of extension preoperatively to -11° of extension postoperatively (p < 0.001). Active wrist flexion also significantly changed from 69° preoperatively to 33° postoperatively (p < 0.001). Parent-guardian surveys indicated that the mean overall satisfaction score after surgery was 9.1 of 10 possible points and that the mean ranking for task completion in activities of daily living was 4 (easier following surgery)., Conclusions: Long-term outcomes reveal that surgical correction of wrist flexion posture in children with amyoplasia congenita results in improvement that is sustained over time. The surveys and questionnaires completed by parents or guardians indicated that they were satisfied with the results of the operation.

Purpose: Madelung's deformity is usually recognized near the completion of skeletal growth and corrective osteotomy of the radius is frequently performed to treat the deformity and reduce pain. This study reviewed the clinical and radiographic results of a volar approach, ligament release, and dome osteotomy technique for treatment of this deformity., Methods: Between 1990 and 2000, 26 wrists in 18 patients were treated with a volar ligament release and dome osteotomy of the distal radius. The average age of the patients at the time of surgery was 13 years. All patients were available for review at an average of 23 months after surgery. Radiographs before surgery and at final follow-up evaluation were analyzed for the extent of correction., Results: All patients treated with this corrective osteotomy reported a reduction in pain and improved appearance. Patients showed improvements in forearm supination and wrist extension, with no loss of pronation or flexion. Improvements in the radiographic parameters of radial inclination and lunate subsidence also were observed. Four wrists required concurrent ulnar-sided surgery, and 3 additional wrists required staged ulnar shortening. One patient required a Darrach resection 3 years after the index procedure., Conclusions: The results of volar dome osteotomy provide improved range of motion, improved appearance, radiographic correction, and pain relief while preserving soft-tissue support for radial rotation around the distal ulna. Long-term follow-up evaluation is ongoing.

There are good options to maximize upper limb function for the child with arthrogryposis. Preservation of available elbow and wrist motion is essential. A comprehensive plan for upper limb treatment includes operative and nonoperative care.

A resurgence of interest in Madelung's deformity has developed recently because of improved operations for correction of the deformity, identification of the genetic loci for the condition in certain syndromal variants, identification of an anterior ligamentous structure tethering the carpus, and preventive treatments in growing children. The process is reviewed in this article and a new surgical technique is presented. The procedure is performed by way of an anterior incision that is more cosmetically appealing. The release of an anterior ligamentous structure described by Vickers is performed simultaneously with a dome shaped osteotomy of the radius. The fragments, once alignment is corrected, are stablized with temporary pin fixation and a long arm cast until the bone has healed.