Madelung deformity

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...

No abstract text is available yet for this article.

CASE: The aim of the article is to report on a case of a teenager affected by Madelung deformity treated with a double osteotomy, planned by means of a 3D model. Using a custom-made cutting guide, the radial osteotomy was performed, and after the reorientation, a shortening ulnar osteotomy completed the procedure. Postoperative clinical assessment showed a normal alignment of the ulna with increased range of motion wrist motion. CONCLUSIONS: Using a 3D model when planning a multidirectional correction of a Madelung deformity may be advantageous to achieve a more accurate and precise realignment of the carpus and distal radioulnar joint...

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...

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...

The short stature homeobox-containing (SHOX) is the most frequently analysed gene in patients classified as short stature patients (ISS) or diagnosed with Leri-Weill dyschondrosteosis (LWD), Langer mesomelic dysplasia (LMD), or Madelung deformity (MD). However, clinical testing of this gene focuses primarily on single nucleotide variants (SNV) in its coding sequences and copy number variants (CNV) overlapping SHOX gene. This review summarizes the clinical impact of variants in noncoding regions of SHOX. RECENT FINDINGS: CNV extending exclusively into the regulatory elements (i...

BACKGROUND: Madelung's disease (MD) is a chronic alcoholism-associated metabolic syndrome characterized by symmetrical subcutaneous deposition of adipose tissue in the head, neck, shoulders, back, trunk, and nerve roots of the upper and lower limbs. It is relatively rare in Asian individuals and is prone to misdiagnosis. Herein, we report a case of a patient with MD who had undergone surgical management at our hospital, and we discuss the pathogenesis, diagnosis, and treatment of MD. CASE SUMMARY: We report a case of MD in a 65-year-old man of Han descent...

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...

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...

Madelung deformity is a congenital disorder with the malformation of anterior ulnar bowing of radius and a dorsally protruding ulnar head caused by premature growth disturbance at the medial volar aspect of the distal radius. This report describes a bilateral idiopathic Madelung deformity in a 17-year-old woman treated successfully using reverse wedge osteotomy of the distal radius in a symptomatic left wrist. Reverse wedge osteotomy can orient the radial joint surface while correcting the whole radius length by reversely putting the wedge bone removed from the distal metaphysis of radius, the base of which is cut from the surplus of the radial and dorsal cortical bone in the hypotrophic portion...

Background: Since the Sauvé-Kapandji procedure was introduced in 1936, many modifications were created using dynamic stabilizer, such as the ECU (extensor carpi ulnaris), the FCU (flexor carpi ulnaris), pronator teres to solve proximal ulnar stump pain. We believe that this modification is also another option for distal ulnar stump instability. Methods: From January 1998 to February 2017, there were 13 patients received the Sauvé-Kapandji (S-K) procedure with tenodesis of the ECU to the carpus and interosseous membrane...

No abstract text is available yet for this article.

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...

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...

OBJECTIVE: This paper presents a probable case of Madelung-type deformity of the right lower arm in an individual from the Merovingian burial ground (7th and 8 th century CE) from Gotha-Boilstädt (Germany). MATERIALS: A female individual with an age-at-death of 40-50 years was investigated. METHODS: Macroscopic, osteometric and radiographic analyses were performed using standard methods. RESULTS: The individual exhibits an unusual case of dysmelia of the right upper extremity...

Reproductive potential is the most important conditional indicator reflecting the ability of individuals in a population to reproduce, survive and develop under optimal environmental conditions. As for humans, the concept of reproductive potential can include the level of the individual's mental and physical state, which allows them to reproduce healthy offspring when they reach social and physical maturity. Female reproductive potential has been investigated in great detail, whereas the male reproductive potential (MRP) has not received the equal amount of attention as yet...

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...

INTRODUCTION: Three-dimensional (3D) planning and patient-specific surgical guides are increasingly used in the treatment of skeletal deformities. The present study hypothesis was that they are reliable in forearm osteotomy in children, with low morbidity. MATERIAL AND METHODS: Twenty-there children with one or several osteotomies to correct forearm deformities were retrospectively included: 9 (20 osteotomies) with surgical guide (G+), and 14 (28 osteotomies) without (G-)...

The purpose of this study is to reveal the effect on the clinical phenotype of variants detected at family examination of a case of combined pogo transposable element derived with zinc finger domain (POGZ) gene, tubulin folding cofactor E (TBCE) gene, and short stature homeobox (SHOX) gene variation. A Turkish non-consanguineous family consisting of five members was investigated. Whole exome sequence analysis and chromosomal microarray analysis (CMA) were performed for a 2-year-old male patient (the proband) with global developmental delay, hypotonia, dysmorphia, and hot water epilepsy...

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)...