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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.

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

Objective SHOX gene is one of the genetic causes of short stature and the clinical phenotype includes variable degrees of growth impairment such as Langer mesomelic dysplasia, Leri-Weill dyschondrosteosis or idiopathic short stature. In this study, we aimed to describe the clinical features and molecular results of SHOX deficiency in a group of Turkish patients who had skeletal findings with/without short stature. Methods Fourty-six patients with idiopathic short stature, disproportionate short stature or skeletal findings without short stature from 35 different families were included in the study. SHOX gene analysis was performed using Sanger sequencing and MLPA analysis. Results We detected three different point mutations (two nonsense, one frameshift) and one whole SHOX gene deletion in 15 patients from 4 different families. While 4 of 15 patients had Langer mesomelic dysplasia, the remaining patients had clinical features compatible with Leri-Weill dyschondrosteosis. Madelung's deformity, cubitus valgus, muscular hypertrophy and short forearm were the most common phenotypic features as well as short stature. Additionally, hearing loss was also detected in 2 patients with Langer mesomelic dysplasia. Conclusion In this study, we presented the clinical spectrum and molecular findings of 15 patients with SHOX gene mutations or deletions. SHOX deficiency should be especially considered in patients who have disproportionate short stature or forearm anomalies with/without short stature. Although most of the patients had a partial or whole gene deletions, SHOX gene sequencing should be performed in suspected cases. Furthermore, conductive hearing loss may rarely accompany these clinical manifestations.

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.

Objective This project investigates two cases of skeletal dysplasia from archaeological excavations of the New Kingdom Period (c. 1400–1050 BCE) portion of the Tombos cemetery in Sudan. Materials: Fair to well-preserved skeletal remains of two individuals, one adult and one juvenile, are examined here. Methods All available skeletal elements were analyzed macroscopically. A differential diagnosis was conducted for each individual. Results The adult individual, U36.Sh2.B10, displays bilateral mesomelic dysplasia and Madelung’s deformity. The juvenile individual, U36.Sh2.B5, also displays bilateral mesomelic dysplasia and characteristics associated with Madelung’s deformity. Conclusions A differential diagnosis of Leri–Weill dyschondrosteosis (LWD) is suggested for the adult female individual (U36.Sh2.B10). The second case (U36.Sh2.B5) is an approximately three to five-year-old individual and is difficult to diagnose given the young age; however, LWD remains the most likely diagnosis. Significance There are few cases of LWD in the paleopathological literature, and fewer still of juveniles. The cases described are useful examples in expanding research demonstrating the variability in the expression of skeletal dysplasias in juveniles and adults. Limitations Taphonomic alterations and fragmentation of the crania and portions of the postcrania limited the observation of the full suite of characteristics associated with skeletal dysplasias. U36.Sh2.B5 is difficult to diagnose given the individual’s young age and the possibility that this individual had not yet developed the more observable characteristics associated with these conditions. Suggestions for further research Researchers are encouraged to continue examining the range of expression of skeletal dysplasias in juveniles and adults.

Madelung's deformity of the wrist arises from premature closure of the medial and volar aspect of the distal radial physis. True Madelung deformities reveal the presence of a “Vickers” ligament which is a short, volar, radioulnar ligament. Clinically, patients report increasing deformity, pain, and poor range of motion. Radiological features include increased radial inclination, volar tilt of the distal radius, and a positive ulnar variance. Surgical intervention usually comprises either a “Vickers” ligament release and distal radius physiolysis or a radial dome osteotomy. In future, EOS Imaging could aid diagnosis by providing more detailed images of the deformity while minimizing radiation exposure. Furthermore, three-dimensional printing and computer-navigated deformity correction could revolutionize management by facilitating simulation training, expediting surgery, and reducing intraoperative error.

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.

Madelung’s deformity is an uncommon congenital wrist condition characterized by premature closure of the volar-ulnar aspect of the distal radius physis, volar carpal subluxation, and distal ulna prominence. It is classified as a malformation of the radio-ulnar axis involving the entire upper limb, according to the Oberg-Manske-Tonkin Classification. It accounts for less than 2% of congenital upper extremity differences. Madelung’s deformity is most commonly idiopathic, but a Madelung-like deformity may result from trauma, infection, multiple hereditary exostoses (MHE), and Ollier’s disease. Additionally, it is associated with skeletal dysplasias involving mutations of the short stature homeobox (SHOX) gene. Madelung’s deformity predominantly affects females and becomes clinically apparent during adolescence. Affected individuals may present with wrist pain, restricted range of motion of the wrist and forearm, decreased grip strength, and function difficulties, as well as aesthetic concerns. Several surgical options have been described for children with Madelung’s deformity, depending on their age and degree of deformity, and include physiolysis, soft tissue release, and osteotomies, with promising outcomes.

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 is rare, accounting for 1.7% of congenital anomalies. The treatment is essentially surgical and several techniques have been described. We report the result of a case treated in an adult patient by a double osteotomy of the radius and ulna. The patient is satisfied.

Several investigators have defined measurements for Madelung’s deformity based on the distal radius or on the longitudinal ulnar axis to avoid the distorted distal radius and its lunate fossa. However, errors may occur in severe cases because of ulnar deformity and displacement. We quantified seven established measurements for Madelung’s deformity relying on the central axis of the capitate. The inter- and intrarater reliability of the capitate-related and the ulna-related techniques were compared. We observed a higher inter- and intrarater reliability for the capitate-related method than for the ulna-related method. Better agreement was also observed for measurements of distance than for measurements of angles. However, the palmar tilt angle measurement method was neither reliable nor reproducible. The capitate-related technique can help to accurately determine the severity of Madelung’s deformity, assist in surgical planning and identify the prognosis. Level of evidence: III

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. We corrected the palmar subluxation of the carpus, restored her good function, and relieved her wrist pain.

Maria Francisca Cegarra-Navarro *, Alexandra Eleonor Castillo-Burns , Jose Antonio Diaz-Manzano , Maria Jesus Fernandez-Ferrando a Hospital General Universitario Reina Sofia de Murcia, Avda. Intendente Jorge Palacios, 1, 30003 Murcia, Spain Hospital Clinico Universitario Virgen de la Arrixaca de Murcia, Ctra. Madrid-Cartagena, s/n, 30120 El Palmar, Murcia, Spain i n d i a n j o u r n a l o f r h e u m a t o l o g y 1 1 ( 2 0 1 6 ) 5 2 – 5 4

Background Although Madelung’s deformity (MD) is believed to be a rare congenital anomaly of the wrist, the symptoms do not manifest till late childhood. It is caused by asymmetric growth at the distal radial physis secondary to premature and partial volar–ulnar-sided arrest, and a thickened radiolunate volar ligament (Vickers ligament) that creates a tether across that physis segment, restricting growth across it. Radial growth disturbance causes the distal radius to deform in the coronal (increasing radial inclination) and sagittal (increasing volar tilt) planes, and eventually lead to a progressive, three-dimensional wrist deformity including radiocarpal and distal radioulnar joint (DRUJ) malalignment. Various techniques for the surgical management of MD have been described, but clear evidence to support the use of any single approach is lacking. Patients and methods Eight wrists in six patients with MD were included in this study. All patients underwent combined radial biplane closing wedge osteotomy above Vickers’ ligament radial origin, which allowed for three-dimensional correction of the deformity and reposing of the ligament, making it easy to achieve more congruity of the DRUJ, and temporary fixation with dual percutaneous K-wires of the DRUJ in the achieved re-optimized new components’ correlation. Results At 22 months of an average follow-up, patients were satisfied with the appearance of their wrists and forearms. The range of active pain-free wrist movements improved notably in supination and extension, correction toward normal values for the mean measurements of radiographic parameters was reported, and the DRUJ was congruent with sustained improvement of its mean gap distance at the latest follow-up radiographs. Conclusion This technique seems to be safe and can reliably restore hand–forearm alignment with respect to DRUJ re-optimization through the redesigned distal radial alignment in both coronal and sagittal planes, and improve joint congruity.

We present our experience with forearm lengthening using Ilizarov external fixator in cases of length discrepancies between radius and ulna and forearm–wrist deformity that occurred duo to different causes. Twelve patients were treated by Ilizarov external fixator between 2008 and 2010 with a mean age of 10 years. There were seven males and five females. The etiology was Madelung’s deformity in seven patients, multiple cartilaginous exostosis in three patients, post-traumatic growth arrest of distal radius epiphysis in one patient, and chronic osteomyelitis of radius in one patient. The length discrepancy ranged from 1 to 4 cm (mean 2.2 cm). Lengthening of radius was done in nine patients and lengthening of ulna in three patients. The mean of follow-up period was 2 years. At follow-up, all patients were satisfied with the functional and cosmetic results. There was an improvement in pain and range of motion .The mean length gained was 2.2 cm and the mean healing index was 43.7 days/cm. Lengthening of short forearm is functionally, cosmetically, and psychologically beneficial. The Ilizarov method is a reliable, successful, and safe method, and it is the gold standard to treat forearm length discrepancy and deformity problems preserving a satisfactory function of upper limb during treatment.

Madelung’s deformity is a premature growth arrest of the volar ulna aspect of the radial growth plate leading to ulna overgrowth and a progressive wrist deformity. Treatment depends on the skeletal maturity of the patient and can be targeted at either the ulna or the radius. We describe the rationale and evidence behind the treatments available and propose a classification to guide management.

La maladie de Madelung est une déformation du poignet due à une atrophie de la partie médiale du cartilage de croissance distal du radius. Il en résulte une subluxation antéro-médiale du carpe,limitant les amplitudes articulaires. Cette dysplasie osseuse est rare et représente 1,7% des anomalies congénitales. Les auteurs rapportent un cas bilatéral chez une fille de 21 ans. L’étude de cette observation nous a permis d’étudier la fréquence, les signes et les moyens de son diagnostic ainsi que les mesures thérapeutiques adéquates afin de pouvoir répondre aux attentes des patientes.Pan African Medical Journal 2016; 23

A 10-year-old girl presented to the emergency department after her younger brother jumped and sat on her left forearm. She had moderate pain over her left wrist. On examination, the wrist looked deformed with volar shift of the hand relative to the long axis of the forearm and marked prominence of the ulna dorsally (figure 1). Figure 1 The lateral view of the left forearm shows marked dinner-fork deformity of wrist with volar shift of hand. The dorsal view is unremarkable apart from a rounded wrist area. The wrist was minimally swollen with mild tenderness over …

Madelung’s deformity was first described by Malgaigne in 1855 however carries Madelung’s name from his description in 1878 [1]. The deformity is caused by an abnormal growth arrest of the palmar-ulnar distal radius physis. In addition to the abnormal growth, there is an abnormal palmar ligament tethering the lunate to the palmar-ulnar radius epiphysis and metaphysis (Vickers’ ligament) that is thought to contribute to the deformity [11]. The deformity is characterized by a prominent dorsal subluxation of the ulnar head and palmar sag of the hand and wrist with a shortened forearm. Patients present with wrist pain as well as concerns about the aesthetic deformity.

Surgery may be indicated in treating Madelung’s deformity and numerous techniques have been described. This study reports the early clinical and radiological results of a radial biplanar opening wedge osteotomy and modified Darrach procedure, using the excised ulnar head as a trapezoidal bone graft. Between 2000 and 2008, five adult wrists with symptomatic Madelung’s deformity underwent surgery. All patients were female, with an average age at surgery of 34 years. Assessment included range of movement, grip strength, DASH scores and radiological imaging. All patients improved both subjectively and objectively with regards to pain, functional range of movement, and appearance at mean follow-up of 55 months (range 14—113). All osteotomies united. One patient required removal of hardware for restricted rotation. This technique provided satisfactory results that are comparable to other studies, and avoids the use of iliac crest bone graft.

Human growth is a multifactorial trait influenced by environmental, hormonal, and genetic factors. Although it is clear that multiple factors contribute to an individual's final height and limb development, genetic factors play a crucial role. One such gene is the short stature homeobox ( SHOX) containing gene. Knowledge about the SHOX gene has rapidly increased since its discovery in 1997, and we now know that SHOX haploinsufficiency affects the development of the extremities and is an important cause of short stature. Currently, SHOX mutations occur with an estimated incidence of roughly 1 in 1000 newborns, making mutations of this gene one of the most common genetic defects associated with growth failure and skeletal deformities. Heterozygous mutations of SHOX have been implicated in patients with Madelung's deformity, Leri-Weill dyschondrosteosis (77%), Turner's syndrome (66%), and idiopathic short stature (3%), and homozygous mutations of SHOX gene have been identified in patients with Langer's mesomelic dysplasia (100%). Recognition of the early radiographic features encountered in SHOX haploinsufficiency maybe pivotal for the diagnosis. In this article, we summarize the genetic and clinical features of the various SHOX haploinsufficiency-associated disorders. We present the characteristic imaging features of these disorders and the results of growth hormone treatment trials.

Madelung deformity (MD) is a rare, normally painful abnormality of the wrist and forearm which characteristically begins in adolescence. Usually the deformity appears between the age of 8 and 14 years, often progressing from initially mild functional pain to fatigue and loss of strength and finally, reduced mobility. We present the MR-findings in three patients with bilateral MD, using a high-resolution imaging protocol adapted for 3.0 Tesla (3.0 T) examinations.Wrist images of three patients were acquired at a 3.0 T Scanner (Gyroscan Intera, Philips Medical Systems, Best, The Netherlands), using a dedicated phased array coil. The imaging protocol consisted of coronal T1-weighted Turbo-spin-echo (T1w-TSE) and coronal and sagittal T2-weighted TSE sequences (T2w-TSE).MR-images of these three girls demonstrated severe volar bayonet configuration of the forearms with a dorsal prominence of the ulnar head, also a curved distal radial articular surface with increased ulnar angulation, due to a deceleration of growth in the ulnar portion of the distal epiphysis. The proximal carpal row showed pyramidal configuration. Also visible was a prominent short radiolunate ligament, the so called Vickers ligament, which originates from the ulnar border of the radius, inserts into the volar pole of the lunate and likely contributes to carpal pyramidalization. Furthermore, the images demonstrated an anomalous hypertrophied and elongated volar radiotriquetral ligament which, to our knowledge, has been described elsewhere only in another case.High resolution imaging at 3.0 T permitted a detailed analysis of the complex pathomorphology in patients with MD. Investing the better signal-to-noise ratio at higher field strengths into spatial resolution an excellent image quality could be obtained, depicting the Vickers ligament and the anomalous volar radiotriquetral ligament in this rare disease.

We report on a girl presenting Léri-Weill dyschondrosteosis (LWD) due to deletion of the SHOX gene. Her family included individuals with short stature alone or with both short stature and mesomelia or Madelung's deformity. The deletion was demonstrated through detection of hemizygosity for microsatellite markers SHOX-CA repeat, DXYS10092, DXYS10093 and DXYS10091 localized around the SHOX gene, with retention of paternal alleles in the proband and three of her sisters who had short stature as the only clinical feature. Hemizygosity for these loci was also observed in their mother, who had short stature too. The deletion in the proband was however larger, including locus DXY10083. The proband's only sister with normal height did not carry the deletion. Family history suggests transmission of the deletion from the proband's maternal great-grandfather to her grandfather via the Y chromosome, and from the grandfather to the proband's mother via the X chromosome after crossing-over in the pseudoautosomal region proximal to the SHOX gene.

Four measurements, ulnar tilt, lunate subsidence, lunate fossa angle and palmar carpal displacement, on wrist radiographs of 26 patients with Madelung’s deformity and 48 normal subjects were compared. The range of measurements on wrists with Madelung’s deformity was wider than on normal wrists, with severe deformities having very abnormal values. Some Madelung’s patients had values that were within the normal range for each of the four measurements. Measurement of the lunate fossa angle demonstrated the least overlap between normal wrists and wrists of patients with Madelung’s deformity with only two in the normal range. A lunate fossa angle over 29° may help identify early Madelung’s deformity.

Osteological examination of a 9th–11th century skeleton from Black Gate Cemetery, Newcastle-upon-Tyne, reveals Madelung's deformity of both forearms and shortened stature due to reduced tibial length. These are indicative of dyschondrosteosis, a genetically transmitted mild form of mesomelia. This case adds to a small but growing presence of this condition in pre-modern societies. Copyright © 2007 John Wiley & Sons, Ltd.

Purpose To establish whether Madelung's deformity demonstrates a radiographic continuum of severity and whether a forme fruste does exist. Methods Ulnar tilt, lunate subsidence, palmar carpal displacement, and lunate fossa angle were measured in 81 wrist radiographs with obvious or suspected Madelung's deformity. Statistical analyses based on these measurements were performed to ascertain if there is a deformity continuum. Results Ranges of 15° to 51° (mean, 28°) for ulnar tilt, –7 to +11 mm (mean, –0.8 mm) for lunate subsidence, 9 to 25 mm (mean, 15.3 mm) for palmar carpal displacement, and 20° to 56° (mean, 33°) for lunate fossa angle were obtained. Significant correlations were observed between all measurements. Conclusions Madelung's deformity encompasses a spectrum of radiographic abnormality. Type of study/level of evidence Diagnostic I.

This chapter is divided into four sections. 1. Shoulder: (a) Obstetric brachial plexus injuries are usually secondary to traction, about 90 % recover. The function of biceps brachii is the best indicator of recovery. Differential diagnoses should always be considered. (b) Pseudoarthrosis of the clavicle occurs due to a failure of fusion of the secondary ossification centres. Patients present with a palpable lump for which surgery is rarely indicated. 2. Elbow: The pneumonic CRITOL for secondary ossification centres around the elbow is a useful aide when diagnosing elbow injuries. (a) Congenital radial head dislocation is the most common congenital anomaly of the elbow. 60 % of patients have other anormalities. Patients present with restricted forearm rotation and elbow extension. Non operative treatment is recommended. Surgery in the form of radial head excision is an option if symptomatic after skeletal maturity. 3. Wrist: (a) Madelung’s deformity occurs due to premature fusion of the volar and ulna aspects of the distal radius, which leads to volar and ulna tilt of the distal radial surface. 4. Hand: Swanson’s classification is the most widely used for congenital hand abnormalities. It is broken down into seven types. The most common abnormalities are syndactyly and polydactyly. The most common acquired hand deformity is trigger thumb, spontaneous resolution occurs in 50 % cases, resolution is unlikely if it has not occurred before the age of 3 and surgery in the form of releasing the A1 pulley may be indicated.

Purpose To evaluate the hypotheses that all Madelung's deformity subjects have dyschondrosteosis (defined as short stature and mesomelia, in addition to Madelung's deformity) and to evaluate the concept that Madelung's deformity may affect the entire radius. Methods A radiographic and medical records review was performed for 26 subjects (46 extremities) with Madelung's deformity. The radiographs were assessed for radius and ulna length, sagittal radial bow, severity of the Madelung deformity, and radiocapitellar joint space. The sagittal radial bow and the radiocapitellar joint space were used to classify subjects according to whether the Madelung deformity was limited to the distal radius or involved the entire radius. Results Thirty-one extremities in 18 subjects were classified as having a distal radius Madelung deformity and 15 extremities in 8 subjects were classified as having an entire radius Madelung deformity. The radius and ulna length and subject height were significantly decreased compared with age- and height-matched normal values in both groups; the entire radius group was more severely affected. In addition, the entire radius group had more severe deformities with respect to lunate subsidence and ulnar tilt. All of the entire radius subjects and 9 of 14 of the distal radius subjects had dyschondrosteosis. Conclusions Madelung's deformity presents as a spectrum. It may affect the entire radius or it may affect only the distal radius. Extremities with involvement of the entire radius have a shorter radius and ulna, decreased height, and a more severe deformity than extremities with involvement of only the distal radius. Additionally, most subjects with Madelung's deformity have dyschondrosteosis. Type of study/level of evidence Diagnostic III.

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. Level of Evidence Therapeutic, Level IV.

This article describes a modification of the double-osteotomy radial technique that has been described previously and summarizes our clinical experience with this method in 4 female patients. All patients had improvement in functional motion and were satisfied with the improved appearance of their wrist after surgery.

Madelung's deformity is an uncommon congenital condition of the wrist usually seen in adolescent girls. It first was documented in the 1800s and is characterized by a shortened radius that curves ulnarly and volarly, a prominent ulna head that projects dorsally from the wrist, and a triangular arrangement of the carpal bones. It is mostly an aesthetic deformity although functional problems and pain may prompt surgeons to undertake a variety of corrective surgical procedures with varying degrees of success. This challenging condition is encountered rarely in a hand surgeon's practice. Even more obscure than the condition is the physician it is named after: Otto Wilhelm Madelung, a distinguished and successful German surgeon who lived at the turn of the century. This article provides a historical perspective on the person and the condition that is still an enigma a century later.

We present our experience with correction of Madelung's deformity by the Ilizarov technique. Seven patients (eight deformities) were treated by osteotomy of the radius with subsequent lengthening and angular correction. They were reviewed at a mean of 30 months (1.5 to 5.5 years). At the time of operation their mean age was 19 years (9 to 44). At follow-up all were free from pain and supination had improved by a mean of 34 degrees and pronation by 9 degrees. Flexion had increased in most cases with a median increase of 15 degrees, but only one patient gained further extension. Radial and ulnar deviation were increased by a mean of 6 degrees and 9 degrees, respectively. Radiographic measurements showed that the mean volar angulation had been reduced from 25 degrees to 11 degrees, ulnar inclination from 45 degrees to 30 degrees and carpal malalignment (volar translation) from 7 to 2 mm. The mean lengthening of the radius was 12 mm (6 to 25). All the patients were satisfied with the functional and cosmetic results.

Objective/Hypothesis: Madelung’s deformity results from a growth defect in the ventral and medial part of the growth plate of the distal extremity of the radius, causing a disorientation of the ulnar part of radial glenoid. Surgical procedures to improve aesthetics and function for Madelung’s deformity are numerous and difficult to assess because the disease is uncommon. We propose and evaluate an original technique in a retrospective study of 19 wrists: the reverse wedge osteotomy (RWO) of the distal radius. Materials and Methods: Twelve women and 1 man Madelung’s deformity were treated from 1992 to 2016. The wrists (6 bilateral, 7 unilateral) were reviewed by an independent examiner at an average follow-up of 6 years (6 months-22 years). Surgery was motivated by aesthetic and functional discomfort at the average age of 23 years, before any complication. RWO was developed to reorient the radial joint surface while reducing overall radius length as little as possible. Osteotomy was performed through an anteroradial or radial approach with an average time under 2 h. The bone wedge was harvested from the excess cortical on the dorsal and radial aspect of the radius. The circumferential wedge was then removed, reversed, and put back into the osteotomy to ensure closing on cortical excess and lengthening on the opposite side. Fixation was achieved by an anterior locking plate in all cases. An associated osteotomy of the ulna was necessary to avoid an ulnocarpal conflict for 4 cases with severe deformity. Objective (morphology of the wrist, range of motion, and grip strength) and subjective (Quick Disabilities of the Arm, Shoulder, and Hand and Patient-Rated Wrist Evaluation scores) data were analyzed. Radiological settings were taken from McCarroll’s criteria. A vector model of the procedure was established to estimate osteotomy angles from 2 indexes from McCarroll. The nonparametric Wilcoxon test ( P < .05) was used for statistical analysis. Results: All cases achieved fusion at 3 months. Eight of the 19 wrists had the plate removed. There was no complication except for hypoesthesia on the radial side of the thenar eminence in 2 cases. Aesthetics and range of motion improved. Improvement was significant for flexion, pronation, and supination with preserving the grip strength, as well as the radiological parameters of McCarroll: significant correction of the palmar and ulnar deviation of the radial epiphysis, as well as rising of the lunate and palmar displacement of the carpus. Average Quick Disabilities of the Arm, Shoulder, and Hand and Patient-Rated Wrist Evaluation scores were less than 30 out of 100 at review. All patients were satisfied aesthetically and functionally. Conclusions: The corrective power of RWO is well adapted to the severe radial epiphyseal dystrophy of Madelung’s deformity. Clinical and radiological results are convincing and meet patients’ expectations. RWO has a special place among the techniques proposed so far. The flattening of the dorsal aspect of the wrist is hoped to provide long-term protection against extensor tendon tear. Vector model allows preoperative planning and optimizes the realization.

The case reported here refers to the skeletal remains of a mature adult male found in a collective grave known as ‘Giant's tomb’ located near Donori (Sardinia) and dating to the end of the Bronze Age. The skeleton showed bilateral shortening of the forearm associated to radial bowing, marked deformations at the radio-ulnar distal joints and subsequent posterior dislocation of both ulnae. The whole alterations fit well with a diagnosis of Madelung's deformity, a rare form of mesomelic dysplasia. At present, this case is the most ancient evidence of Madelung's deformity. Copyright © 2002 John Wiley & Sons, Ltd.

Treatment of Madelung's deformity is still controversial. We reviewed retrospectively 19 patients with Madelung's deformity (two bilateral, 21 cases) who underwent surgery to the radius and ulna to improve range of motion, decrease pain and improve appearance of the wrist. Nineteen patients underwent 21 distal radial osteotomy procedures using three different techniques: subtraction, addition or dome osteotomy. Ulnar shortening and redirection of the distal ulna was performed in 12 cases; a long oblique osteotomy was used in 10 of these cases. The Sauve-Kapandji technique was performed in five cases, an ulnar distal epiphysiodesis in two cases and a combination of osteotomy and epiphysiodesis in one case. The aim was to reduce the distal radial slope and to restore the orientation and congruity of the distal radio-ulnar joint and to improve its function. Pain was reduced as a result of the procedure: more than 75% of the cases had no or intermittent pain at the review. Pronation improved from 63° to 68° (P=0.467, not significant) and supination improved from 48° to 72° on average (P=0.034, significant). Grip strength increased from 11 to 18 kgf (P=0.013, significant). Madelung's deformity is not always a benign condition and it responds well to corrective osteotomies.

Two individuals with bilateral Madelung's deformity were identified in a Late Intermediate period comingled tomb at the northern highland site of Marcajirca, Ancash, Peru (ca. AD 1250). Comparisons of the size and robusticity of the radii and ulnae suggest the individuals represent a male and a female. The difference in the severity of the changes is thought to represent variability in the expression of the deformity seen in males and females in clinical cases. Three comparatively short, thick tibiae were also recovered from this tomb, which may suggest that the individuals demonstrate Leri-Weill dyschondrosteosis, a type of dwarfism characterized by mesomelic shortening. These are the first examples of Madelung's deformity to be described from an archaeological context in South America and offer an insight into the use of tombs (chullpas) in Late Intermediate period Ancash.

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 stabilized with temporary pin fixation and a long arm cast until the bone has healed.

The paper presents a European family showing bilateral Madelung's deformity within five consecutive generations. Females as well as males are affected alternately indicating autosomal dominant inheritance. Despite of a body height within the lower normal range in two patients the diagnosis of dyschondrosteosis could not be clearly established as further hints for dwarfism are missing. The etiology of Madelung's deformity may be difficult to establish. There seems to be the possibility of an inheritance independent from the complete syndroma of dyschondrosteosis. Therefore the genetic counselling will always be difficult and has to regard the wide variety of symptoms ranging from little pain and cosmetic disturbance to moderate pain and functional impairment.

We report a case of Madelung's Deformity in a 23-year-old female. When she was 11 years old, she was aware of the deformity of her bilateral wrist and forearm. Wrist pain started when she was 13 and the symptoms worsened. We performed wedge osteotomy of radius with the Sauve-Kapandji Method at the bilateral sides when she was 22 and 23. After the operation, wrist pain and range of motion improved.

Introduction Madelung's deformity is a bone dysplasia that occurs predominantly in adolescent females, characterized by early epiphyseal growth arrest in the medial part of the distal radius. This leads to an upward and medial displacement of the radial joint surface, restricting range of motion. Objectives The objective of this study was to determine whether there was a link between clinical and radiological data in children with Madelung's deformity and to test the hypothesis of a relation between the deformity and a genetic mutation. Methods A retrospective study recruited 13 patients with Madelung's deformity, with a mean age of 13.2 years (range, 8–18 years). Assessment comprised level of pain, range of motion and grip force, with standard AP and lateral wrist X-rays. Every patient except one underwent molecular genetic screening, adhering to current recommendations. Results Pronation-supination, radial inclination and grip force were significantly impaired compared to normal results. All X-ray measurements were significantly abnormal, except for the lunate-covering ratio. Genetic mutation (SHOX) was systematic in the 12 patients screened. Discussion Radiological deformity did not correlate with functional disturbance or pain. Non-acquired Madelung's deformity requires molecular screening for SHOX or XO mutation, which definitively diagnoses Leri-Weill dyschondrosteosis or Turner syndrome. Conclusion A larger series is necessary to confirm these preliminary results, which nevertheless suggest that non-acquired Madelung's deformity is not isolated but syndromic. Early detection of Leri-Weill or Turner syndrome is essential, due to their therapeutic specificities. Level IV.

Madelung deformitesi el bileğinin gelişimsel anomalisidir. Distal radius büyüme plağının medial tarafının gecikmiş büyüme hızına ve eklem yüzeyinin rotasyonu sonucunda radiusun medial bölümünün göreceli kısalması ve eklem yüzeyinin volar rotasyonuna bağlı olarak oluştuğu düşünülmektedir. Klinik ve radyolojik bulgular deformite için tanı koydurucudur. Hasta semptomlarının şiddetine bağlı olarak konservatif ya da cerrahi yöntemler ile tedavi edilir. Yazımızda bilateral Madelung deformitesi tanısı ile ameliyat edilen olguyu literatür eşliğinde sunduk Madelung's deformity is the developmental anomaly of the wrist. Delayed growth rate of the medial side of the distal radius growth plate and articular surface rotation causing relatively shortening of the medial side of the radius are considered to be the main reasons. The diagnosis of the condition is based on clinical and radiological findings. Patients with this deformity are treated by surgical or conservative methods according to the severity of the symptoms. In this paper, we present a patient with bilateral Madelung's deformity who underwent corrective osteotomy

This section deals with the inflammatory and degenerative problems affecting the hand and wrist, with osteoarthritis and rheumatoid disease given special consideration. The urgent condition of hand infection is considered, with up-to-date references on the management of these problems. The soft tissue problems of De Quervain’s and carpal tunnel syndrome are covered in detail, with treatment options and outcomes. The pathology underlying Keinbocks and avascular necrosis in the wrist, and the treatment strategies, are dealt with.

The patient is a 39-year-old woman who presented nocturnal paresthesia lasting for 6 months on both hands, but predominantly right (dominant hand). She presented mechanical pain of both wrists, with a visual analog score (VAS) of 90/100. Upon examination, there was a solid tumor on the posterior side of both wrists (Fig. 1), painful limitation of extension of the wrist and supination, mainly on the left side. The hemogram and blood chemistry were normal, as well as the neurophysiological study of the median and ulnar nerves. A karyotype demonstrated mosaicism (86% XX, 5% XXX, 9% X). The posteroanterior wrist X-rays (Fig. 2) showed typical Madelung deformity changes on the right wrist1: shortening of the radius compared to the ulna, dorsally and radially curved radius, dorsal and radial convexity, an angle similar to the distal radial joint surface, a mismatch of the distal radioulnar joint and carpal dislocation. The lateral projection (Fig. 3) demonstrated anterior arching of the radius and dorsal dislocation on the ulnar head; the carpus was also dislocated on the ulnar side and anteriorly in the distal radioulnar joint, in a way that made the wrist bones appear to follow the arch of the radius.2 The patient was diagnosed with a carpal tunnel syndrome due to Madelung’s deformity and was programmed for an evaluation by the orthopedics department but, due to a delay, medical treatment with local steroid infiltrations was carried out. 1 ml of triamcinolone was injected into the right wrist using a standard technique.3 The pain improved in the next 2 days (VAS: 50/100), disappearing completely after one week (VAS: 0/100).

For bilateral Madelung's deformity in a 14-year-old girl we did the Sauve and Kapandji operation without additional radius osteotomy. The operation was justified by the protrusion of the head of the ulna, the limitation of the rotation and diminution of the strength of the wrist, and inability to do sporting activity. The clinical findings were referred to the instability of the inferior radio ulnar joint There was no pain. The post operative review showed no pain, with recovery of grasp and of sporting activity (gymnastic and dance). The esthetic result satisfied the surgeon and the patient The operation permitted repositioning of the inferior radio ulnar joint. We have not seen synostosis of the osteotomy and the width of it increased progressively without radiological and physical instability of the inferior radio ulnar joint. The literature study allows us to discuss the different treatment possibilities and to explain our choice.The simplicity of this operation is interesting, because it was possible to stabilize the carpal joint, with abolition of the physical problems of this youg girl, without the needing osteotomy of the radius, which takes longer to recover, in those cases without pain before the operation.

International audience; Madelung's deformity results from a growth defect in the palmar and ulnar region of the distal radius. It presents as an excessively inclined radial joint surface, inducing "spontaneous progressive palmar subluxation of the wrist". The principle of reverse wedge osteotomy (RWO) consists in the reorientation of the radial joint surface by taking a circumferential bone wedge, the base of which is harvested from the excess of the radial and dorsal cortical bone of the distal radius, then turning it over and putting back this reverse wedge into the osteotomy so as to obtain closure on the excess and opening on the deficient cortical bone. RWO corrects the palmar subluxation of the carpus and improves distal radio-ulnar alignment. All five bilaterally operated patients were satisfied, esthetically and functionally. Its corrective power gives RWO a place apart among the surgical techniques currently available in Madelung's deformity.

An elderly male medieval skeleton excavated from St Gregory's Priory presented with unilateral Madelung's deformity. The right radius and ulna were shortened due to premature fusion of the distal growth plate. The radius displayed lateral and dorsal bowing and the ulna was dislocated posteriorly. Pronation and supination was not possible and the available hand bones presented with disuse atrophy. Degenerative changes had reduced the mobility of the elbow joint and marked cubitus valgus was demonstrated. The unilateral presentation, shortening of the ulna and atrophy of the hand bones and the sex of the skeleton suggest a post-traumatic rather than an idiopathic form of Madelung's deformity. As far as we are aware this is the first archaeological example of this condition.

Congenital upper limb malformations represent complex pathologies because of their varied clinical presentations, imaging features, and etiologies. They can be divided into (1) failure of formation with transverse, intercalary, and longitudinal (preaxial, postaxial, and mesoaxial) deficiencies, (2) failure of differentiation with synostoses, carpal coalitions, syndactylies, and symphalangism, (3) duplication with ulnar dimelia and polydactylies, and (4) brachydactylies. Congenital Madelung's deformity, clinodactyly, camptodactyly, and Kirner's deformity are usually included in these malformations. Despite advances in molecular diagnosis, a good knowledge of clinical and imaging features as well as special consideration of other skeletal or nonskeletal abnormalities are essential to eventually diagnose an embryo fetopathy (maternal valproate treatment, constriction band syndrome), a genetic disorder (trisomy 21 or Down syndrome, Turner's syndrome, Holt-Oram syndrome), or a nongenetic syndrome (vertebral, anal, cardiac, tracheal, esophageal, renal, limb association, Poland's syndrome). Genetic counseling for a child presenting with a congenital upper limb malformation is of great value, both for the treating team and the parents, and imaging is often required. The latter is still largely supported by conventional radiography, both for diagnosis and functional prognosis, but ultrasound and magnetic resonance imaging will be great tools in the near future to better evaluate these conditions.