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

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

Madelung Deformity: Diagnosis and Treatment Options

Madelung deformity (MD) comprises an increased volar and ulnar tilt of the joint facet of the distal radius, secondary to an idiopathic physeal dysplasia. Such change causes radial shortening and consequent distal ulnar prominence, along with wrist pain and loss of motion. Surgery becomes an option in patients with severe deformity that do not respond to conservative treatment.The classic surgical techniques are problematic for adults, as they are specific for children and adolescents, whose radial physis is still open. Very few papers discuss the treatment of adult patients; furthermore, most are focused on the distal radioulnar joint, and thus do not approach the origin of the pathology.When analyzing computed tomography scans with tridimensional reconstruction, a feature of MD, growth arrest of the volar and ulnar portions of the distal radius, is noted, causing the typical distal radius deformity that leads to lack of coverage of the lunate bone. That leads to palmar subluxation of the lunate bone and consequent radiocarpal instability.We herein describe the possibilities of treatment in different stages of evolution, summarizing the authors' view on MD.

Una mano da SHOX

A gene SHOX haploinsufficiency was diagnosed in a 13-year-old girl with short stature. Madelung deformity was absent but some dysmorphism of the fingers and toes were present.

Madelung Deformity: Radioscapholunate Arthrodesis With a Neo-DRUJ

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

Madelung Deformity: Surgical Correction with Radial Dome Osteotomy

Madelung deformity is a rare wrist malformation caused by a growth disturbance of the palmar and ulnar part of the distal radial physis. The aim of this study is to evaluate the outcome of radial dome osteotomy in patients affected by Madelung deformity. The endpoint of this operation is to improve the orientation of the articular surface of the radius, so as to support to the carpal bones. Between 2017 and 2019, in our clinic, 4patients were treated using this technique. Post-operative pain was evaluated using the NRS. Functional outcomes were assessed through evaluation of ROM, grip strength via Jamar dynamometer and using DASH questionnaire. The aesthetic defects were estimated using a section of the Michigan Hand Outcome Questionnaire. Correction of deformities was evaluated on post-operative RX using McCarrol Criteria. Were also analyzed the accuracy and tolerance of the plates used.

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