Role of Vickers Ligament in the Pathogenesis of Madelung Deformity.

OBJECTIVE: The Vickers ligament is thought to hinder the growth of palmar ulnar radius by tethering the lunate to the radius, leading to Madelung deformity. The purpose of this study was to clarify the nature of the Vickers ligament and investigate its pathogenesis in Madelung deformities based on our observation of the Vickers ligament. METHODS: All 22 patients (33 wrists) with Madelung deformities treated surgically between 2018 and 2022 were included. The diagnosis was confirmed radiographically in all patients. The three-dimensional computed tomography (3D-CT) data of 16 patients (19 wrists) were available. Magnetic resonance imaging (MRI) data were available for 9 patients (14 wrists). Wrist arthroscopy was used in 4 patients. The Vickers ligament was resected and submitted for histopathological examination in 8 patients. Radiographic outcomes, 3D-CT, MRI, arthroscopy, surgical findings, and histopathology of the Vickers ligament were evaluated. RESULTS: The 3D-CT revealed that the Vickers ligament originated in the metaphysis and formed a metaphyseal defect at the palmar ulnar radius. In the sequential MR coronal images, the Vickers ligament could be divided into 3 branches, extending to the lunate, triquetrum and ulnar styloid. Arthroscopy and surgical findings revealed that the nature of the Vickers ligament was the stretched palmar ligament of the wrist. The histopathology results revealed ligamentous tissue and fibrocartilaginous metaplasia with a structure similar to that of the triangular fibrocartilage complex (TFCC). CONCLUSIONS: The Vickers ligament is not a separate aberrant ligament. The nature of the Vickers ligament is a combination of the stretched TFCC ligament (palmar radioulnar ligament, ulnotriquetral ligament and ulnolunate ligament) and radiolunate ligament. The possible pathogenesis of Madelung deformity might be focal early epiphyseal closure at the middle part of the sigmoid notch, which leads to focal growth retardation of the radius and pulls palmar ligaments proximally to form the Vickers ligament.

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.

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.

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.

Modified dome osteotomy and anterior locking plate fixation for distal radius variant of Madelung deformity: a retrospective study.

PURPOSE: Dome osteotomy of the distal radius with Vickers ligament release is an established method of treatment for Madelung deformity. Many different surgical procedures have been proposed in literature but techniques, patient inclusion and efficacy evaluations are heterogeneous. MATERIALS AND METHODS: A retrospective review of children affected by 'distal radius' Madelung deformity and treated with a standardized surgical procedure (modified reverse dome osteotomy of the distal radius and volar fixation with a small locking plate) between 2010 and 2018 at a single center was performed. The technique used in this study, reversing the shape of the classical dome osteotomy, allowed for an improved three-planar correction of the distal radial epiphysis and volar plate fixation allowed for an increased stability and reduced soft tissue morbidity. A structured follow-up including a prompt post-operative rehabilitation program (without wrist immobilization) was established. Pain relief, functional outcome and cosmetic appearance were assessed with a structured clinical assessment, DASH Score and radiographic assessment, accordingly. RESULTS: A total of 15 wrists in 13 children (12 females, mean age of 15.6 years, range 11-19) were included. The mean follow-up time was 3.8 years. Bone union and pain relief were obtained in all cases. Improvement in the range of motion was detected in extension, supination and flexion of the wrist. Radial inclination was increased by 15.3° and lunate subsidence by 4.1 mm. CONCLUSIONS: The use of volar fixation with a small locking plate and immediate post-operative rehabilitation for reverse dome osteotomy of the distal radius in pediatric patients affected by 'distal radius' Madelung's deformity is stable and effective.

The prevalence of Vickers' ligament in Madelung's deformity: a retrospective multicentre study of 75 surgical cases.

In a retrospective multicentre study, we aimed to correlate clinical factors and findings on plain radiographs and MRI with the intraoperative presence of Vickers' ligament in Madelung's deformity. We screened the records, in which the absence or presence of Vickers' ligament was affirmatively indicated, of 75 consecutive operated extremities in 58 patients. In 83% a Vickers' ligament was observed intraoperatively. The whole bone Madelung type (as opposed to the distal type) and a distal radial notch were independent, significant predictors for the presence of the ligament. The correct Vickers detection rate using MRI was 85% of the 27 cases for which MRI was available. Thus, the MRI was a good but not perfectly reliable modality. We conclude that Vickers' ligament is present in the majority but not all cases with Madelung deformity. We advise that patients with a more severe type of Madelung's deformity and a distal radial notch should be monitored closely.Level of evidence: IV.

Madelung's deformity in children: Clinical and radiological results from a French national multicentre retrospective study.

INTRODUCTION: Madelung's deformity is rare. Treatment often consists in radial or ulnar osteotomy, Vickers' ligament resection or ulnar epiphysiodesis. The present multicenter retrospective study reports clinical and radiographic results in various surgeries. The study hypothesis was that early surgery improves outcome. MATERIAL AND METHOD: Files were analyzed for 36 children (47 wrists) operated on between 1998 and 2015. Mean age at diagnosis was 12 years (range, 6-15 years). Mean follow-up was 7.2 years (range, 2-17.4 years). Two age groups were distinguished: 6-11 and 12-17 years. Endpoints were esthetic, functional (Mayo Wrist Score: MWS) and radiographic [lunate fossa angle (LFA), radial height (RH), lunatum cover rate (LCR), ulnar head translation (UHT), lunatum ascension (LA)]. Techniques mainly comprised distal radius osteotomy (DRO) with ulnar shortening osteotomy (USO), and Vickers' ligament resection (VR), either isolated or associated to osteotomy. RESULTS: Age at surgery guided choice of technique, but did not impact results. VR gave better functional results when performed in young children; it was mainly performed in the 6-11 years age group, providing very satisfactory results (7 out of 8 wrists) with excellent function (7 out of 8), without improving radiographic parameters; in the 12-17 years age group, even when esthetic results were excellent, functional results were only good-to-acceptable (3 out of 5 wrists) and 1 result was poor. DRO+USO was mainly performed in the 12-17 years age group, with very satisfactory or satisfactory results (8 out of 10 wrists) and excellent function (6 out of 10). DRO+USO appeared to be an option of choice, significantly improving radiographic parameters and correcting the deformity (24.5° improvement in LFA (p=0.0033) and 36.2% improvement in LCR (p=0.0103)). DRO+USO+VR was mainly implemented in the 12-17 years age group, with very satisfactory results (3 out of 4 wrists) and excellent-to-good MWS in most cases (3 out of 4), but without significant radiographic improvement. DISCUSSION: Esthetic and functional results were comparable regardless of age, except for VR, which gave better functional results in the 6-11 years age group, but without significant difference. CONCLUSION: The present study showed that, regardless of children's age, surgery provided esthetic, functional and radiographic benefit, although no decision-tree could be drawn up. LEVEL OF EVIDENCE: IV.

Madelung Deformity of the Wrist Managed Conservatively.

A 19-year-old female presented with pain, deformity, and slightly restricted left wrist motion for five years with gradual progression. Physical examination revealed volar subluxation of the left hand, dorsally prominent ulnar styloid, radial and dorsal bowing of the distal forearm, and mild restriction in wrist dorsiflexion. Radiographs showed a failure of ossification of the ulnar side of the distal radial epiphysis, increased radial inclination angle, dorsal subluxation of the distal ulna, V-shaped proximal carpal row due to proximal migration of the lunate, and increased interosseous space. A diagnosis of Madelung deformity of the left wrist was made. Conservative management with oral analgesics, activity restriction, and a volar splint was done as the patient was skeletally mature, had only mild pain with no functional limitation or gross deformity. At the six-month follow-up, she was doing well with decreased pain and no new complaints.

Radiotriquetral Ligament in Madelung's Deformity Associated with Leri-Weill's Dyschondrosteosis.

Madelung's deformity (MD) is frequently associated with Leri-Weill's dyschondrosteosis (LWD) even if the primary isolated form (PI-MD) is much more common. Recent studies pointed out how two abnormal ligaments, the Vickers ligament (VL) and the radiotriquetral ligament (RTL), are defining traits of MD. To date, in PI-MD, both VL and RTL have been reported. In MD associated with LWD (LWD-MD), the VL is also present, but the RTL has never been reported. We herein report the first case of MD associated with a genetically confirmed LDW with an RTL, detected on MRI. This report describes the MRI imaging features of MD-LWD, which have not been adequately characterized in previous literature.

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.

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.

Skeletal Deformity Associated with SHOX Deficiency.

SHOX haploinsufficiency due to mutations in the coding exons or microdeletions involving the coding exons and/or the enhancer regions accounts for approximately 80% and 2-16% of genetic causes of Leri-Weill dyschondrosteosis and idiopathic short stature, respectively. The most characteristic feature in patients with SHOX deficiency is Madelung deformity, a cluster of anatomical changes in the wrist that can be attributed to premature epiphyseal fusion of the distal radius. Computed tomography of SHOX-deficient patients revealed a thin bone cortex and an enlarged total bone area at the diaphysis of the radius, while histopathological analyses showed a disrupted columnar arrangement of chondrocytes and an expanded hypertrophic layer of the growth plate. Recent studies have suggested that perturbed programmed cell death of hypertrophic chondrocytes may underlie the skeletal changes related to SHOX deficiency. Furthermore, the formation of an aberrant ligament tethering the lunate and radius has been implicated in the development of Madelung deformity. Blood estrogen levels and mutation types have been proposed as phenotypic determinants of SHOX deficiency, although other unknown factors may also affect clinical severity of this entity.