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.

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.

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.

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.

Beyond the guidelines management of juvenile idiopathic arthritis: a case report of a girl with polyarticular disease refractory to multiple treatment options and Leri Weill syndrome.

BACKGROUND: The last two decades brought new treatment options and high quality guidelines into the paediatric rheumatologic practice. Nevertheless, a number of patients still present a diagnostic and therapeutic challenge due to combination of vague symptoms and unresponsiveness to available treatment modalities. CASE PRESENTATION: We report a case of sixteen years old girl suffering from polyarticular type of juvenile idiopathic arthritis refractory to multiple treatment options. She first presented at the age of 4 with swelling and contractures of both knees. Her symptoms were initially unresponsive to nonsteroidal anti-inflammatory drugs and progressed despite treatment with intraarticular and systemic glucocorticoids and methotrexate. Throughout the years, she received several biologics together with continuous administration of nonsteroidal anti-inflammatory drugs and disease modifying anti-rheumatic drugs as well as intraarticular and systemic glucocorticoids in disease flares. However, none of this options  provided a permanent remission, so various other modalities, as well as other possible diagnoses were constantly being considered. Eventually she became dependent on a daily dose of systemic glucocorticoids. In 2018, the treatment with Janus kinase inhibitor tofacitinib was initiated, which led to gradual amelioration of musculoskeletal symptoms, improvement of inflammatory markers and overall well-being, as well as to the weaning of systemic glucocorticoids. As the swelling of the wrists subsided for the first time in many years, Madelung's deformity was noticed, first clinically, and later radiographically as well. Genetic analysis revealed short-stature homeobox gene deficiency and confirmed the diagnosis of Leri Weill syndrome. CONCLUSIONS: This case report emphasizes the need for reporting refractory, complicated cases from everyday clinical practice in order to build-up the overall knowledge and share experience which is complementary to available guidelines. Individual reports of difficult to treat cases, especially when additional diagnoses are involved, can be helpful for physicians treating patients with common rheumatological diseases such as juvenile idiopathic arthritis.

Carpal Kinematics in Madelung Deformity.

PURPOSE: Various skeletal and soft tissue abnormalities have been identified in Madelung deformity and have been hypothesized to play a causal role in its progressive symptomatology; however, our pathological understanding of these changes remains limited. In this study, we biomechanically assessed the Madelung deformity wrist, using 4-dimensional computed tomography imaging. METHODS: Nine Madelung deformity wrists (5 patients; age, 24 ± 5 y) and 18 healthy wrists (9 volunteers; age, 28 ± 3 y) underwent 4-dimensional imaging during flexion-extension motion and radioulnar deviation. Carpal kinematics and radiocarpal joint parameters were quantified and compared. RESULTS: In Madelung deformity wrists, significantly decreased rotation was seen in the lunate (-4.6°) and the triquetrum (-4.8°) during flexion-extension motion. During radioulnar deviation, significant decreases were visible in lunate bone translation (-0.7 mm), triquetrum bone translation (-0.6 mm), and triquetrum bone rotation (-1.9°). Patients had significantly decreased articulating surface areas of the scaphoid (1.4 ± 0.2 cm2 versus 1.6 ± 0.2 cm2) and lunate (1.2 ± 0.4 cm2 versus 1.5 ± 0.3 cm2) fossa, and significantly increased radioscaphoid (1.3 ± 0.1 mm versus 1.2 ± 0.1 mm) and radiolunate (1.6 ± 0.2 mm versus 1.3 ± 0.3 mm) joint space thicknesses. CONCLUSIONS: There is a decreased mobility of the lunate and triquetrum bones in Madelung deformity. CLINICAL RELEVANCE: Four-dimensional imaging could be used in future studies that investigate the effect of surgical ligament release on carpal kinematics and subsequent wrist mobility.

[Osseous variations in radiological diagnostics of the wrist]. / Ossäre Normvarianten in der radiologischen Diagnostik des Handgelenks.

CLINICAL/METHODICAL ISSUE: The carpal joint is one of the most complex joints in the body comprising multiple bones that allow flexibility while simultaneously providing stability. Variations in osseous structures that may be either cause or result of pathological changes may make radiological reporting challenging. Only the knowledge of important osseous variations allows a reliable assessment of carpal imaging studies. STANDARD RADIOLOGICAL METHODS: The standard imaging technique for evaluation of osseous carpal structures is conventional radiography, which is followed by computed tomography (CT) and-under special circumstances-magnetic resonance imaging (MRI). Other imaging methods such as sonography or nuclear medicine studies do not play a significant role in clinical routine. METHODICAL INNOVATIONS: Apart from continuous reduction in effective radiation dose, there have been no significant methodical improvements in the past decade regarding imaging of osseous carpal structures in clinical routine. PRACTICAL RECOMMENDATIONS: As the initial diagnostic procedure, conventional radiography usually allows a safe and reliable diagnosis of osseous structures. Unclear or discrepant imaging findings between clinical and imaging assessment should initiate further imaging, preferably with CT. Only for certain questions or to reduce effective radiation dose in children MRI studies should be performed in clinical routine.

Treatment of Madelung Deformity With Vicker Ligament Release and Radial Physiolyses: A Case Series.

PURPOSE: To evaluate the surgical outcomes in a series of Madelung wrists treated with a Vicker ligament release at a young age. We hypothesize that early treatment of Madelung deformity with Vicker ligament release is safe and may minimize progression of deformity. METHODS: A retrospective review was performed at a single large pediatric institution from 2013 to 2016 of patients with a diagnosis of Madelung deformity treated with Vicker ligament release and radial physiolysis. Exclusion criteria included patients who were skeletally mature, who underwent osteotomy procedures, or who had incomplete follow-up. Patient demographics were collected, concomitant surgeries were recorded, and outcomes including range of motion and pain were documented. Measurements of standard anteroposterior and lateral radiographs were calculated before and after surgery to monitor radiographic deformity and progression. RESULTS: Six girls with bilateral Madelung deformity who underwent bilateral Vicker ligament resection and radial physiolysis (12 total wrists) were included. The average age at presentation was 7.5 years (range, 7-9 years), with an average follow-up of 30 months. Reasons for presentation included sports injuries (2), ulnar-sided wrist pain (2), and mild deformity (2). Additional radial and ulnar epiphysiodeses were performed in 2 wrists each. There were no intraoperative complications. Although pain resolved within the first month after surgery for all patients, 2 patients had intermittent unilateral ulnar-sided wrist pain at final follow-up. All patients returned to their presurgery activities. There was no loss of range of motion, and 4 wrists with preoperative supination deficits improved by an average of 17°. Radiographic measurements demonstrated improvement in the radial physeal angle in 10 out of 12 wrists (83%). No patients displayed worsening deformity after surgery. Two patients underwent subsequent procedures. CONCLUSIONS: In patients with early Madelung deformity, Vicker ligament release with radial physiolysis is a safe treatment option that theoretically has the potential to minimize the progression of radiographic deformity. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Lunate Grafting Into the Radius for Lunate Fossa Reconstruction in Madelung Deformity.

A 40-year-old woman presented with Madelung deformity and severe arthritic changes at both the radiocarpal and the distal radioulnar joints. She was treated by using her lunate as an osteochondral graft into the radius allowing reconstruction of the lunate fossa. The scaphoid and triquetrum were removed concomitantly and a Sauve-Kapandji procedure was performed. Complete bone healing was achieved. Ten years later, an excellent functional result was maintained, with a pain-free wrist, an acceptable wrist joint range of motion, as well as a favorable aesthetic appearance. This procedure may be indicated for patients with severe Madelung deformity with painful radiocarpal and distal radioulnar joints associated with severe arthritis changes.

Madelung deformity and Madelung-type deformities: a review of the clinical and radiological characteristics.

Madelung deformity of the distal radius results from premature closure of the medial volar aspect of the distal radial physis, leading to increased volar tilt and increased inclination of the radial articular surface, triangulation of the carpus with proximal migration of the lunate and dorsal displacement of the distal ulna. The deformity is particularly common in Leri-Weill dyschondrosteosis, but it may also occur in isolation. True Madelung deformity can be differentiated from Madelung-type deformities by the presence of an anomalous radiolunate ligament (Vickers ligament). In this article, we will review the imaging characteristics of true Madelung deformity, including the common "distal radius" variant, the less common "entire radius" variant and "reverse" Madelung deformity. We will discuss the role of the Vickers ligament in disease pathogenesis and its use in differentiating true Madelung deformity from Madelung-type deformities arising from trauma or multiple hereditary exostoses. Surgical management of these patients will also be addressed.

Madelung Deformity.

Madelung deformity of the wrist is more common in females and is often associated with Leri Weill dyschondrosteosis, a mesomelic form of dwarfism. Patients with Madelung deformity often report wrist deformity resulting from the prominence of the relatively long ulna. The typical Madelung deformity is associated with a Vickers ligament that creates a tether across the volar-ulnar radial physis that restricts growth across this segment. The distal radius deforms in the coronal (increasing radial inclination) and the sagittal (increasing volar tilt) planes. There is lunate subsidence and the proximal carpal row adapts to the deformity by forming an upside-down pyramid shape or triangle. Treatment depends on the age at presentation, degree of deformity, and magnitude of symptoms. Mild asymptomatic deformity warrants a period of nonsurgical management with serial x-ray examinations because the natural history is unpredictable. Many patients never require surgical intervention. Progressive deformity in the young child with considerable growth potential remaining requires release of Vickers ligament and radial physiolysis to prevent ongoing deterioration Concomitant ulnar epiphysiodesis may be necessary. Advanced asymptomatic deformity in older children with an unacceptable-appearing wrist or symptomatic deformity are indications for surgery. A dome osteotomy of the radius allows 3-dimensional correction of the deformity. Positive radiographic and clinical results after dome osteotomy have been reported.

Inter- and intrarater reliability of ulna variance versus lunate subsidence measurements in Madelung deformity.

PURPOSE: To assess inter- and intrarater reliability of both ulna variance and lunate subsidence measurement methods in a large consecutive series of children with Madelung deformity. METHODS: Ulnar variance and lunate subsidence were measured on 41 standard anteroposterior wrist radiographs from 31 patients with Madelung deformity. The patients had a mean age of 13 years (range, 5-25) at the time of presentation. Two pediatric orthopedic hand/upper limb surgeons evaluated all radiographs twice in a 4-week interval using standard digital imaging software. Intraclass correlation coefficients (ICCs) were calculated for inter- and intrarater reliability, and results were reported using the Landis and Koch criteria. RESULTS: The interrater ICC for the ulna variance measurements was substantial, and for the lunate subsidence almost perfect. The intrarater ICC for ulna variance was substantial for both raters. In contrast, the intrarater ICC for lunate subsidence was almost perfect for both raters. CONCLUSIONS: Measurement of lunate subsidence showed both superior interrater and intrarater reliability compared with the ulnar variance method. Whenever relative ulna length is assessed in children and adolescents with Madelung deformity, the lunate subsidence should be the preferred method to characterize deformity.

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.

Cylindrical corrective osteotomy for Madelung deformity using a computer simulation: case report.

We report an adolescent patient with Madelung deformity that we successfully treated by cylindrical corrective osteotomy of the distal radius. We used customized surgical guides, which were designed based on preoperative 3-dimensional computer simulation.

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.

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.

#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.

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.

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.

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.