Early Distal Radius Remodeling after Radius Dome Osteotomy in Madelung's Deformity.

Background Distal radius realignment procedures such as the dome osteotomy have been shown to reliably correct the geometry of this three-dimensional deformity. However, it has not been clarified whether the correction is durable over time. We thus reviewed a prospective Madelung's database and evaluated all patients who received a radius dome osteotomy to see whether there was any loss of correction over time. Patients and Methods A total of 16 patients with 19 operated wrists were included. Mean age at surgery was 14 years; the most recent radiograph that was considered for analysis was obtained at a mean of 20 months after the index surgery. Three established radiographic deformity parameters (ulnar tilt, lunate subsidence, palmar carpal displacement) were repetitively measured by an independent observer over the course of the postoperative follow-up and compared using the analysis of variance statistics. Results A mild but nonsignificant worsening of deformity parameters was observed in six patients (32%). Overall, ulnar tilt improved from 50 to 40 degrees after surgery and was 43 degrees at latest follow-up. The values for lunate subsidence were -5.5, 0, and -1.6 mm, respectively. Palmar carpal displacement did not recur after correction (21, 17, and 16 mm, respectively). Conclusions Frontal plane parameters remodeled to a certain degree, although not in a statistically significant manner, soon after corrective osteotomies in Madelung's deformity. Although mild surgical overcorrection may be warranted in younger patients, it remains unclear if a concomitant distal radial epiphysiodesis would generally be beneficial. Level of Evidence Level IV.

Patient and Caregiver Impressions of the Impact of Madelung Deformity: A CoULD Registry Analysis.

PURPOSE: This study seeks to investigate demographics of patients with Madelung deformity in a large, geographically diverse sample and understand patient and caregiver perceptions of the impact of this condition. We hypothesized that patients with untreated Madelung deformity have greater pain and lower function compared to the normal population but are less affected than the chosen control group, namely, patients with proximal radioulnar synostosis (PRUS). METHODS: This retrospective study queried the Congenital Upper Limb Differences (CoULD) Registry, a multicenter registry of patients treated in tertiary care pediatric hospitals. We searched patients enrolled as of July 2022 and identified 3,980 total patients and 66 (1.7%) with a diagnosis of Madelung deformity. We reviewed demographics and Patient-Reported Outcomes Measurement Information System (PROMIS; peer relations, depressive symptoms, pain interference, and upper extremity function domains) scores at time of enrollment. We used a matched cohort comparison with propensity scoring for 50 patients with Madelung deformity and 50 patients with PRUS (control cohort). RESULTS: Patients with Madelung deformity presented at an average age of 13.1 years (± 2.1 years). Ninety-eight percent were female, and 82% were White. Seventy-four percent had distal radius-only deformity. Upper extremity PROMIS scores in both the Madelung deformity and the PRUS groups were significantly "worse" than normal, confirming our hypothesis. The Madelung deformity and PRUS cohort scores were not consistently different from one another. PROMIS scores from all other domains, including pain interference, were similar to, or better than normal for both groups, disproving the second part of our hypothesis. CONCLUSIONS: Patients with Madelung deformity averaged 13 years of age and were nearly all female, and the majority had only distal involvement of the radius. Patients with Madelung deformity had lower function based on PROMIS scores, similar to the control cohort, whereas all other PROMIS measures were similar to or better when compared to normal values. Pain interference scores in both cohorts were lower than normal values. Patients with Madelung deformity have decreased function, similar to the comparative cohort of patients with PRUS, but do not present with increased pain. TYPE OF STUDY/LEVEL OF EVIDENCE: Symptom prevalence III.

Assessment of 3.0 Tesla magnetic resonance imaging in Madelung's deformity: findings and implications.

OBJECTIVE: The aim of the study was to investigate the 3.0 Tesla magnetic resonance imaging (MRI) features of Madelung's deformity. MATERIALS AND METHODS: The wrist MRI scans of 19 patients clinically diagnosed with Madelung's deformity and 20 patients without deformity were consecutively selected from Beijing Jishuitan Hospital between April 2019 and December 2022 for observation, in the case group and control group, respectively. Multiple linear regression was used to analyze the factors affecting tilting angle and width of central disc (CD, also termed as triangular fibrocartilage, the main component of triangular fibrocartilage complex), while the chi-square test was used to compare the occurrences of CD (radial) attachment displacement, VL, and RTL. p < 0.05 indicated statistical significance. RESULTS: Madelung's deformity significantly contributed to the tilting and thickening of the CD. In the case group, the tilting angle and thickness of CD were (51.46 ± 1.33)° and (0.23 ± 0.01) cm, respectively, which was statistically significant (p < 0.05); the radial attachment of the CD significantly shifted away from the distal articular surface level (χ2 = 39.00, p < 0.001), with a mean displacement of (0.97 ± 0.38) cm. Furthermore, the cases demonstrated abnormally developed Vickers ligament (χ2 = 35.19, p < 0.001) and radiotriquetral ligament (χ2 = 25.66, p < 0.001). CONCLUSION: MRI provides a notable advantage in diagnosing Madelung's deformity. Compared with the control group, patients with Madelung's deformity exhibited tilting and thickening of the CD. Additionally, the radial attachment of the CD was significantly shifted proximally with abnormal development of Vickers and radiotriquetral ligaments.

Restoring Wrist Harmony: A Case Report of Ulnar Osteotomy for Correcting the Radius Malunion.

A 41-year-old female patient sought medical attention due to a malunited distal radius fracture with a positive ulnar variance, experiencing wrist pain and limited range of motion. The patient was successfully treated with an isolated ulnar osteotomy and bone grafting, resulting in significant alleviation of symptoms and improved wrist mobility. Various surgical methods have been proposed to address malunited radius fractures, and ulnar osteotomy has shown promise as an effective technique for such cases.

Advances in diagnosis and treatment of Madelung's deformity.

In this paper, a review of the literature was performed to critically evaluate relevant published research on diagnosis and treatment of Madelung's deformity. Madelung's deformity is a metaphyseal dysplasia of the distal radius, where the distal radial joint tilts to the volar and ulnar sides, combined with distal ulnar subluxation and elbow dislocation. The main pathogenic factors of this disease include idiopathic, hereditary and acquired factors. So far, it is believed that Madelung's deformity is mainly associated with trauma, epiphyseal dysplasia, nutritional disorders, and gene deletion or mutation. It is more common in females, and is an autosomal dominant inheritance disease. Most patients suffer from this disease bilaterally. Madelung's deformity may occur as a complication of Leri-Weill dyschondrosteosis. Most patients usually have no clinical symptoms in the early stage, and some patients come to the hospital due to wrist pain, stiffness, deformity and a shorter forearm. X-ray film is the main diagnostic method for this disease. Magnetic resonance imaging can show local soft tissue and bone abnormalities in the early stage, so it is used for the early diagnosis of this disease. The ulnar angle can be classified into different types based on the size of the distal radius palmar angle. For severe deformity and symptoms, surgical treatment is often required, including soft tissue release, distal radius osteotomy, ulnar shortening osteotomy, distal ulnar resection, and distal radioulnar joint fusion. Some procedures have better clinical results in relieving pain and improving mobility.

State of the art review: The pathogenesis and management of Madelung deformity.

Madelung deformity remains a fascinating yet unresolved challenge. There is an increasing awareness for early diagnosis by healthcare providers with improvement in diagnostic modalities, however, the exact mechanisms for the development of the deformity have still to be clarified. While some corrective procedures have been described to effectively address an established deformity, the existing literature lacks clear and evidence-based treatment guidelines on how to proceed in daily practice. This review article aims to summarize the current best evidence on this topic, including particular areas of controversy and areas with need for future research.

[Analysis of the effectiveness of sequential plate internal fixation in correction of Madelung deformity after ulnar osteotomy and shortening].

Objective: To investigate the effectiveness of sequential plate internal fixation in the correction of Madelung deformity after ulnar osteotomy and shortening. Methods: The clinical data of 13 patients with Madelung deformity admitted between September 2015 and July 2021 were retrospectively analyzed. There were 5 males and 8 females with an average age of 18.3 years ranging from 17 to 23 years. The disease duration ranged from 12 to 24 months, with an average of 17 months. Three cases had a clear history of trauma. All patients had external radial deviation deformity and limited movement of the ulnar deviation, and the ulnar impact pain was significant during ulnar deviation movement; 9 patients had limited wrist joint supination movement, and the supination movement was normal. In the first stage, ulnar osteotomy and shortening combined with external fixator were used to correct wrist deformity in 13 patients. After operation, bone transfer was performed 6 times per day, with adjustments made every 4 hours, which was 1 mm per day. After the osteotomy was in place, the ulnar plate internal fixation was performed to reconstruct the ulnar stability in the second stage. The Cooney wrist joint score was used to assess the pain, function, range of motion, flexion and extension range of motion, and grip strength of the wrist joint before operation and before the removal of internal fixator. The subjective feeling and appearance satisfaction of patients were recorded. Results: After the second-stage operation, all the 13 patients were followed up 10-22 months, with an average of 15 months. The deformity of wrist joint disappeared after operation, and the flexion, extension, and ulnar deviation were basically normal. There was no complication such as ulnar impingement sign, nonunion or infection. Wrist function, pain, and range of motion were significantly improved after operation, except for 1 patient who had no significant improvement in rotation and pain. The ulnar internal fixator was removed at 10-18 months after the second-stage operation. The scores of pain, function, range of motion, flexion and extension range of motion, and grip strength in the Cooney wrist score before removal of internal fixator significantly improved when compared with those before operation ( P<0.05). Subjective and appearance satisfaction of patients were excellent in 9 cases, good in 3 cases, and fair in 1 case. Conclusion: Ulnar osteotomy and shortening with sequential plate internal fixation for correction of Madelung deformity, with mild postoperative pain, can effectively avoid bone nonunion, improve wrist joint function, and have significant effectiveness.

Benign paroxysmal positional vertigo in a patient with persistent hypoglossal artery and bilateral madelung deformity.

We explore a case of Benign Paroxysmal Positional Vertigo in the context of Persistent Hypoglossal Artery (PHA) and bilateral Madelung Deformity (MD). PHA is associated with a raft of major adverse cardiovascular events. MD can result from manifold conditions such as Turner's Syndrome and mesomelic dwarfism. In this case, the patient's positive family history of MD across generations is suggestive of inherited mutation in the Short Stature Homeobox (SHOX) Gene. We discuss the putative impact of SHOX on the genesis of Benign Paroxysmal Positional Vertigo (BPPV) in a patient with PHA and bilateral MD.

#MadelungDeformity: Insights Into a Rare Congenital Difference Using Social Media.

BACKGROUND: Madelung deformity is a rare congenital hand difference with little known regarding the patient perspective. In this cross-sectional survey study, we harnessed the global reach of social media to understand the clinical spectrum of Madelung deformity and its impact on physical, mental, and social health. METHODS: A survey was developed based on a previously published protocol and multiple Patient-Reported Outcomes Measurement Information System (PROMIS) short forms. The survey was distributed on several Madelung deformity communities on Facebook and Instagram. T-scores were calculated, interpreted, and compared between patients who underwent surgery and those who did not. Correlations between scores were calculated using the Spearman rank correlation coefficient. RESULTS: Mean PROMIS scores for adults were as follows: pain intensity, 4.9 ± 2.8; pain interference, 57.6 ± 10.0; upper extremity, 35.2 ± 8.1; depression, 53.8 ± 11.1; anxiety, 55.4 ± 11.4; and ability to participate in social roles and activities, 42.5 ± 7.7. Mean scores for children were as follows: pain intensity, 5.0 ± 2.8; pain interference, 55.7 ± 11.3; upper extremity function, 24.6 ± 10.4; depressive symptoms, 57.7 ± 11.3; anxiety, 57.3 ± 11.9; and peer relationships, 42.2 ± 10.3. CONCLUSIONS: Madelung deformity has significant effects on patients' physical, mental, and social well-being, even after surgical treatment. Using social media, we were able to compensate for Madelung deformity's rarity by engaging an international audience, demonstrating the feasibility to conduct research through it, and providing a global perspective of the disease entity.

Madelung Deformity: Radioscapholunate Arthrodesis With a Neo-DRUJ.

BACKGROUND: Madelung deformity is a rare wrist anomaly that causes considerable pain while restricting function. In this study, we describe a radioscapholunate (RSL) arthrodesis with a neo-distal radioulnar joint (DRUJ) in Madelung deformity patients with an abnormal sigmoid notch and compare results to patients after a reverse wedge osteotomy. METHODS: Six wrists underwent RSL arthrodesis with a neo-DRUJ in a two-phase approach: (1) modified RSL arthrodesis with triquetrectomy; and (2) distal scaphoidectomy. Seven wrists underwent a reverse wedge osteotomy procedure. RESULTS: There were no differences found in postoperative pain, grip strength, or range of motion (ROM), apart from extension, which was decreased after RSL arthrodesis with a neo-DRUJ. Quality of life and Michigan Hand Outcomes Questionnaire scores were similar. CONCLUSIONS: Although clinical outcome parameters are not different among the two groups, the RSL arthrodesis with construction of a neo-DRUJ could prove a valid treatment option for a subset of patients with a severely affected sigmoid notch.

Radial Osteotomy With Callus Distraction for the Correction of Severe Madelung Deformity.

We report a case of an 11-year-old girl with bilateral severe Madelung deformity who underwent radial osteotomy with callus distraction. The distal radial articulation was corrected at surgery, and a unilateral fixator was subsequently used for callus distraction. No postoperative complication was noted. The patient did not need any additional surgeries for correction of the deformity. Forty-three months after surgery, the patient had a nearly full range of motion without any pain. Forearm deformity was not noticeable, except for surgical scar on both wrists.

Results of primary radial corrective osteotomy in Madelung's deformity.

INTRODUCTION: The purpose of this study was the evaluation of surgical outcomes in a series of wrists with Madelung's deformity treated with radial corrective osteotomy. We hypothesize that this surgical technique is a suitable and safe way of treatment. MATERIALS AND METHODS: A retrospective review of patients with Madelung's deformity treated with radial corrective osteotomy between January 2001 and June 2017 at a single large department of hand surgery in Germany was performed. Patients who met the inclusion criteria were invited for follow-up and outcome variables including pain, range of motion, patient-rated outcome measures, and radiographic measurements were obtained for comparison with preoperative data collected from the patients' medical records. RESULTS: 14 wrists were included. The average age at the time of surgery was 21.9 years, and the average follow-up was 7.2 years. The average visual analog pain scale at rest decreased from preoperative 2.6 points to postoperative 0.7 points. Under strain, the average VAS declined from 7.4 to 4.9 points. The mean DASH Score decreased from 42.9 before surgery to 22.0 points after surgery. Range of motion improved slightly in five out of six directions of motion, with the greatest increase seen in average supination from preoperative 68.5° to postoperative 82.0°. Averages of all five measured McCarroll's parameters and ulnar variance decreased, as expected from corrective surgery. Four wrists (26.8%) needed subsequent procedures. CONCLUSION: Radial corrective osteotomy was a suitable treatment of Madelung's deformity in our collective, although surgical outcomes are not yet fully satisfying.

Conservative Management of Madelung Deformity of the Wrist in a 15-Year-Old Male: A Case Report.

A 15-year-old boy presented to an outpatient sports medicine clinic with mysterious wrist pain for three months. Imaging diagnosed this mysterious wrist pain as a Madelung deformity, a rare condition characterized primarily by the early cessation of growth in the volar-ulnar distal physis of the radius. Patients may present with restricted wrist range of motion, pain, and ulnar tenderness, but they may also be asymptomatic. Traditionally, treatment is surgical, but this case was managed with conservative measures primarily, with improvements in both function and outcomes. This case report should serve as a reminder to include rare diagnoses in the differential when working up joint pain and to show the role of conservative management in Madelung deformity.

Analysis of the effectiveness of sequential plate internal fixation in correction of Madelung deformity after ulnar osteotomy and shortening / 中国修复重建外科杂志

OBJECTIVE@#To investigate the effectiveness of sequential plate internal fixation in the correction of Madelung deformity after ulnar osteotomy and shortening.@*METHODS@#The clinical data of 13 patients with Madelung deformity admitted between September 2015 and July 2021 were retrospectively analyzed. There were 5 males and 8 females with an average age of 18.3 years ranging from 17 to 23 years. The disease duration ranged from 12 to 24 months, with an average of 17 months. Three cases had a clear history of trauma. All patients had external radial deviation deformity and limited movement of the ulnar deviation, and the ulnar impact pain was significant during ulnar deviation movement; 9 patients had limited wrist joint supination movement, and the supination movement was normal. In the first stage, ulnar osteotomy and shortening combined with external fixator were used to correct wrist deformity in 13 patients. After operation, bone transfer was performed 6 times per day, with adjustments made every 4 hours, which was 1 mm per day. After the osteotomy was in place, the ulnar plate internal fixation was performed to reconstruct the ulnar stability in the second stage. The Cooney wrist joint score was used to assess the pain, function, range of motion, flexion and extension range of motion, and grip strength of the wrist joint before operation and before the removal of internal fixator. The subjective feeling and appearance satisfaction of patients were recorded.@*RESULTS@#After the second-stage operation, all the 13 patients were followed up 10-22 months, with an average of 15 months. The deformity of wrist joint disappeared after operation, and the flexion, extension, and ulnar deviation were basically normal. There was no complication such as ulnar impingement sign, nonunion or infection. Wrist function, pain, and range of motion were significantly improved after operation, except for 1 patient who had no significant improvement in rotation and pain. The ulnar internal fixator was removed at 10-18 months after the second-stage operation. The scores of pain, function, range of motion, flexion and extension range of motion, and grip strength in the Cooney wrist score before removal of internal fixator significantly improved when compared with those before operation ( P<0.05). Subjective and appearance satisfaction of patients were excellent in 9 cases, good in 3 cases, and fair in 1 case.@*CONCLUSION@#Ulnar osteotomy and shortening with sequential plate internal fixation for correction of Madelung deformity, with mild postoperative pain, can effectively avoid bone nonunion, improve wrist joint function, and have significant effectiveness.

Shelf Osteotomy for Madelung's Deformity in Adults: A Novel Technique.

Background Madelung's deformity (MD) comprises increased volar and ulnar tilt of the distal radius joint facet, secondary to an idiopathic physeal dysplasia. Such change causes radial shortening and a consequent distal ulnar prominence, along with wrist pain and loss of motion. Classic surgical techniques are problematic for adults, as they are specific for children and adolescents whose distal radial physis is still open. Description of Technique It is suggested a new treatment method for MD in skeletally mature patients: a distal radius osteotomy and joint realignment are performed through a volar approach to increase the support area of the lunate bone. The rotation and lengthening of the distal epiphysis of the radius generate support and cover to the lunate bone, with improvement of both radiocarpal and distal radioulnar joints. Patients and Methods We describe the technique in details and report the treatment of a 25-year-old female patient. Results Early clinical and radiographic outcomes are encouraging for the treatment of symptomatic patients. Conclusion There is a plethora on the literature about conflicting opinions on the best treatment options and surgical techniques are quite variable, although usually with good results. Besides, the technique here described is indicated during a specific stage of disease presentation, consisting of young adults without any wrist-degenerative changes. Having said that, it is possible to claim that MD treatment with shelf osteotomy is a concept change. Our main goal is to reconstruct the diseased segment and improve wrist stability.

Medium to long term functional and radiographic outcomes in patients with Madelung's deformity treated by isolated or combined radioulnar osteotomy.

INTRODUCTION: Madelung's deformity is a rare congenital condition of the wrist that can cause pain, aesthetic concerns, reduced range of motion and grip strength. Currently, there is no consensus on the optimal age for surgery or whether operative procedures can be isolated or combined depending on the extent of the deformity. The main objective of our study was to analyze the postoperative functional clinical results at a minimum of 3-years follow-up in patients operated on for Madelung's deformity. The secondary objectives were (1) comparison of preoperative and postoperative radiographic parameters, and (2) to assess whether certain preoperative radiographic parameters influence the choice of surgical procedure. HYPOTHESIS: The surgery offered at our center achieves clinical and radiological result necessary for long-term activities of daily living, and varies according to the severity of the distal radial deformity. PATIENTS AND METHODS: We carried out a retrospective observational monocentric study including patients operated on between 2004 and 2016. Clinical (VAS, mobility), functional (PRWE score), and radiographic assessments were performed before and after the last follow-up. RESULTS: The study included 11 patients (12 wrists) with a mean age of 17±7.3 years and a mean follow-up of 8.1 years (4-12.3). The mean VAS was 2.3 (0-6) and the mean PRWE score was 37 (0-108). The mean flexion-extension arc was 134° and that of pronation and supination was 142°. The mean grip strength was 25.8±11.8kg. Four out of 6 radiographic criteria were significantly improved. An isolated radial osteotomy or combined radioulnar osteotomy was performed when the sagittal radial tilt was greater than 30° and protrusion of the lunate greater than 5mm, otherwise below these values, an isolated ulnar osteotomy was performed. DISCUSSION: Our center offers surgical management of Madelung's deformity by osteotomy which improves the majority of postoperative radiographic parameters and gives satisfactory clinical and radiographic results after a mean follow-up of 8.1 years. The surgery is influenced by the severity of the distal radial deformity, including protrusion of the lunate and sagittal radial tilt. LEVEL OF EVIDENCE: IV, Retrospective study.

Simultaneous three-dimensional correction of percutaneous radial osteotomy with circular external fixator in Madelung's deformity.

Partial deficiency of distal radial and volar cartilage plate growth is the main pathology in Madelung's deformity. Surgical treatment can use a Taylor Spatial Frame (TSF) external fixator, which provides simultaneous multiplanar correction and lengthening after radial osteotomy, sparing Vickers' ligament and the ulna. We sought to evaluate the radiological and functional results of Madelung's deformity correction by TSF, by retrospectively analyzing eight Madelung's deformities in seven patients between August 2011 and May 2015. Mean age was 14.8 years (range, 11-26), and mean follow-up 35.1 months (24-78). We evaluated demographic data and pre- and post-operative radiological and functional results. Mean preoperative visual analog pain scale score improved from 7.7 to 1.6. Mean preoperative DASH score improved from 19.1 (9.5-46.6) to 3.7 (2.3-6.8). Mean preoperative radial length improved from 184 mm (138-209) to 196 mm (142-213). We think that software-based correction using a TSF is a safe solution in Madelung's deformity, with minimal margin of error and risk of postoperative stiffness and infection. LEVEL OF EVIDENCE: IV.

Retrospective study of radial dome osteotomy with volar plate fixation versus K-wires in Madelung's deformity: long-term follow-up.

PURPOSE: The emerging role of the locking plate improved the technique also in corrective osteotomies in Madelung's deformity, but there is a lack of analyses between the fixation techniques, as well as little information in functional outcomes and long-term follow-up. The current study compared the outcomes, pitfalls, and advantages of volar plate and screws fixation versus K-wires fixation in a long-term follow-up. METHODS: Twenty-eight children presenting the "distal radius" variant of Madelung's deformity underwent Vickers ligament release and distal radial dome osteotomy between 2009 and 2015. Twenty-three children (20 females and 3 males, mean age 15 years and 7 months at surgery and 24 years and 8 moths at follow-up), with 26 operatively treated wrists, were available for follow-up. A retrospective two-cohort study, evaluating clinically and radiologically results, has been conducted with a mean eight years and seven months follow-up. RESULTS: Bone union and pain relief were obtained in all cases, as well as improvements in wrist motion and radiographic indices. A statistically significant correlation was identified between the volar plate fixation and an improved lunate subsidence on X-ray, and a trend towards an improved DASH score in the cohort with plate and screws, as well as significant improvement in wrist extension and supination always in the plate fixation cohort. CONCLUSIONS: The long-term follow-up enabled the patients to report on more definitive outcomes in terms of functional and cosmetic improvements. Volar plate fixation is an effective technique allowing for simple post-operative management and earlier rehabilitation program with improved clinical and radiographic outcomes.