Radioscapholunate Arthrodesis for Management of Madelung Deformity: A Case Report.

CASE: A 32-year-old woman with bilateral Madelung deformity presented with severe pain and arthritis of the radiocarpal and distal radioulnar joints. At final follow-up, 17 months for the left and 12 months for the right wrist, she had excellent functional results with no pain. Range of motion was 30° of flexion and 30° of extension with full pronosupination. CONCLUSION: There is paucity in the literature regarding salvage procedures in adults with this deformity. We demonstrate treatment with distal ulna excision and an radioscapholunate arthrodesis. This procedure may be indicated in Madelung deformity and proximal radiocarpal and distal radioulnar joint arthritis.

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.

Guided Growth With Minifragment Plates for Angular Deformities in the Distal Radius in Skeletally Immature Patients. Preliminary Results.

INTRODUCTION: Growth modulation allows correction of progressive ulnar deviation of the distal radius in skeletally immature patients, which may occur as a consequence of various pathologies. The aim of this study is to evaluate the radiographic results and complication rate in a series of patients treated with minifragment plates. METHODS: The medical records of 12 patients who underwent guided growth with a minifragment plate on the radial aspect of the distal radius as a consequence of angular deformities in the distal radius were retrospectively reviewed. Demographic data, radiographic changes, and complication rate were analyzed. RESULTS: A total of 14 wrists and forearms were evaluated. The mean age at which surgery was performed was 10.5 years (interquartile range: 9.0 to 11.3). The average time between placement and removal of the material was 28.7 months (SD: 8,89). In each case, a general improvement of the radiographic parameters was obtained. There were 3 postoperative complications, but only 1 of them required reintervention (broken metaphyseal screw). CONCLUSIONS: Hemiepiphysiodesis using a minifragment plate is a treatment that respects the surgical anatomy and offers an alternative surgical option for angular deformities of the distal radius in children. LEVEL OF EVIDENCE: Level IV.

Shi's Yi-Qi Bu-Shen Tong-Luo decoction combined with manipulation in the management of wrist stiffness after distal radius fracture: Study protocol clinical trial (SPIRIT Compliant).

INTRODUCTION: Wrist stiffness is a common sequela of distal radial fractures. Manipulation is generally used and effective, but problems exist, such as intense pain, swelling during the process of manipulation and long treatment period. Therefore, a combinative therapeutic strategy is necessary to benefit rehabilitation after distal radius fracture. Shi's Yi-Qi Bu-Shen Tong-Luo decoction (BTD) combined with wrist manipulation has been used in the Shanghai Ninth People's Hospital Huangpu Branch in last few decades. BTD has potential therapeutic effects on rehabilitation after distal radius fracture, which should be evaluated by rigorous clinical trial. METHODS/DESIGN: A randomized, double-blind, placebo-controlled clinical trial will be conducted to determine the efficiency of BTD in relief of wrist stiffness and pain and function rehabilitation. A total of 80 wrist stiffness patients with or without pain and edema will be enrolled, and treated with wrist manipulation plus BTD or placebo for 4 weeks. The primary outcome measure is the Cooney wrist score. The second outcome measures include pain numerical rating scale, patient rated wrist evaluation, 36-item short form health survey questionnaire, and side effects. DISCUSSION: Although BTD has shown effects on rehabilitation after distal radius fracture in the Shanghai Ninth People's Hospital Huangpu Branch for decades, the universality of this efficacy needs evaluated. The results of this trial will provide a convincing evidence. TRIAL REGISTRATION: ChiCTR2000029260, January 19, 2020.

H2AFY promoter deletion causes PITX1 endoactivation and Liebenberg syndrome.

BACKGROUND: Structural variants (SVs) affecting non-coding cis-regulatory elements are a common cause of congenital limb malformation. Yet, the functional interpretation of these non-coding variants remains challenging. The human Liebenberg syndrome is characterised by a partial transformation of the arms into legs and has been shown to be caused by SVs at the PITX1 locus leading to its misregulation in the forelimb by its native enhancer element Pen. This study aims to elucidate the genetic cause of an unsolved family with a mild form of Liebenberg syndrome and investigate the role of promoters in long-range gene regulation. METHODS: Here, we identify SVs by whole genome sequencing (WGS) and use CRISPR-Cas9 genome editing in transgenic mice to assign pathogenicity to the SVs. RESULTS: In this study, we used WGS in a family with three mildly affected individuals with Liebenberg syndrome and identified the smallest deletion described so far including the first non-coding exon of H2AFY. To functionally characterise the variant, we re-engineered the 8.5 kb deletion using CRISPR-Cas9 technology in the mouse and showed that the promoter of the housekeeping gene H2afy insulates the Pen enhancer from Pitx1 in forelimbs; its loss leads to misexpression of Pitx1 by the pan-limb activity of the Pen enhancer causing Liebenberg syndrome. CONCLUSION: Our data indicate that housekeeping promoters may titrate promiscuous enhancer activity to ensure normal morphogenesis. The deletion of the H2AFY promoter as a cause of Liebenberg syndrome highlights this new mutational mechanism and its role in congenital disease.

Flexor carpi radialis brevis: a rare accessory muscle presenting as an intersection syndrome of the wrist.

The flexor carpi radialis brevis (FCRB) is a rare accessory muscle of the forearm and wrist. It is typically asymptomatic, but has been discovered either incidentally during cadaveric studies or at the time of surgery in patients with distal forearm injury. Rarely, the FCRB muscle is associated with pain. We report a patient with wrist pain related to intersection between the tendon of the FCRB muscle and the tendon of the flexor carpi radialis (FCR) muscle, with an associated longitudinal split tear of the FCR tendon, documented by magnetic resonance imaging (MRI). To our knowledge, this is only the second report in the English literature of this intersection syndrome.

Surgical Management of Spasticity of the Thumb and Fingers.

Spasticity of the hand profoundly limits an individual's independent ability to accomplish self-care and activities of daily living. Surgical procedures should be tailored to patients' needs and functional ability, and even patients with severe cognitive injuries and poor upper extremity function may benefit from surgery to improve appearance and hygiene. Careful preoperative examination and planning are needed, and consideration is given to the potential unintended detrimental effect of a surgical procedure on hand function.

Ulna Growth Patterns After Soft Tissue Release With Bilobed Flap in Radial Longitudinal Deficiency.

BACKGROUND: Centralization is commonly utilized for treating the severely deviated wrist in radial longitudinal deficiency (RLD). Individuals with RLD have congenital shortening of the ulna and previous studies have shown that traditional centralization, in particular with notching of the carpus, results in additional ulnar growth retardation. At our institution, we use a technique of soft tissue release with bilobed flap. We examined if this technique preserves the growth potential of the distal ulna, therefore, avoiding an additionally shortened forearm. METHODS: We retrospectively reviewed serial radiographs of 16 patients with 18 wrists who had at least 3 years of follow-up after a soft tissue release with bilobed flap. Radiographic lengths were measured using the method described by Heikel. Percentage of normal growth was calculated using normative data published by Maresh. Comparisons were made with preoperative, postoperative, and final follow-up studies. RESULTS: The average length of follow-up was 9.2 years (range, 3 to 16.3 y) with an average age of 11.6 years (range, 5.2 to 17.5 y). The average age at the time of surgery was 27 months (range, 14 to 48 mo). A minimum of 3 radiographic studies were available for each subject. The average ulna length preoperatively was 63.9% of age-matched normal length (51.4% to 75.3%). The average ulna length at final follow-up was 61.9% of age-matched normal length (48.5% to 70.3%). The difference was not statistically significant. In addition, there were no distal ulnar physeal arrests. CONCLUSIONS: Soft tissue release with bilobed flap does not affect ulna growth like traditional centralization procedures can. This procedure has previously been shown to retain motion and have similar recurrence rates to formal centralizations. Therefore, we advocate that it be considered more widely for use in patients with RLD and significant wrist deviation to maximize growth and improve hand position. Soft tissue release with bilobed flap can be safely used on young children and preserve ulnar growth. LEVEL OF EVIDENCE: Level IV-therapeutic.

[Os epilunatum is a rare cause of atraumatic impaired motility and wrist pain].

In this case report a 28-year-old man had a six-year history of atraumatic wrist pain. X-ray and MRI showed an accessory ossicle dorsal to the lunate, near the scapholunate ligament: an os epilunatum. Os epilunatum is a rare ossicle of the hand, and only one clinical case and few cadaveric studies have previously been reported. Resection was performed through a dorsal approach, without damaging the scapholunate ligament. Three months post-operatively the patient had increased motility of the hand and no pain.

Osteotomy as a treatment option in dysplasia epiphyseal hemimelica of the distal radius: a case report.

Dysplasia epiphysialis hemimelica (DEH), also known as Trevor disease, is a very rare disease characterized by an overgrowth of the osteocartilaginous epiphyseal caused by unknown factors. The medial side of the epiphysis is affected twice as often as the lateral side and more than one epiphysis was affected in two-thirds of the cases. Only a few cases of DEH in the upper extremities have been published, as the lower limb is usually the most affected, especially in the bones of the ankle joint. The symptoms vary from little significant deformities to symptomatic cases, pain, and decreased function depending on the location and the size of the lesion. When surgery is indicated, the complete excision of the lesion is the procedure of choice. If this is not possible, because of the location or size of the lesion, a realignment osteotomy may be necessary. We report the case of a 12-year-old patient with a distal epiphysis DEH on the left radius, treated by wedge osteotomy realignment at the level of the lesion with Kirschner wires. The clinical outcome of realignment osteotomy of the radial epiphysis has been the correction of the deformity with a normal and painless function of the wrist and hand. The case reported was an extremely rare location in the distal radial epiphysis of DEH. We are aware of only one case published previously with involvement of the distal radius in children. The surgical treatment of realignment osteotomy without complete removal of the lesion has enabled anatomical and functional improvement of the joint as well as a reduction in lesion size. LEVEL OF EVIDENCE: Level IV case report.

Correction of "Wrist" Deformity in Radial Dysplasia: A Systematic Review and Meta-Analysis.

BACKGROUND: Radial dysplasia affects 1 in 6,000 to 8,000 births, classically presenting with a shortened, bowed ulna and radially deviated hand. The optimal treatment remains unclear, with several opposing approaches advocated. This review aims to clarify the long-term outcomes of nonsurgical and surgical treatment of the "wrist" deformity. METHODS: The Embase, MEDLINE, PubMed, Cochrane Central, ClinicalTrials.gov, and World Health Organization International Clinical Trials Registry Platform (ICTRP) databases were searched for published and unpublished studies reporting long-term outcomes of surgical or nonsurgical treatment of children with radial dysplasia. Results were not restricted by date or language. Primary outcomes were hand-forearm angle, ulnar length, and "wrist" active range of motion (ROM). Studies were assessed using the Grades of Recommendation, Assessment, Development and Evaluation (GRADE) criteria. Data for the change in hand-forearm angle were pooled using random-effects meta-analysis, and mean differences and 95% confidence intervals were obtained. Primary outcome data at last follow-up were pooled, and means and standard deviations were obtained. The PROSPERO registration of this study was CRD42016036665. RESULTS: Of 104 studies identified, 12 were included in this review. Five were retrospective cohort studies and 7 were case series. No randomized studies were found. Study quality was low or very low according to the GRADE criteria. The hand-forearm angle of nonsurgically treated patients worsened during childhood, from 66° to 84°, whereas "wrist" active ROM, at 61°, was better than that for most surgically treated patients. Ulnar length with nonsurgical treatment was predicted to be 64% of normal, but was not directly reported. Isolated soft-tissue release provided a modest reduction in hand-forearm angle compared with nonsurgical treatment. Soft-tissue distraction with centralization or radialization achieved the best hand-forearm angle correction (16° radial deviation). Radialization maintained better "wrist" active ROM (46°) and ulnar length than centralization. Microvascular second metatarsophalangeal joint transfer yielded better reported "wrist" active ROM (83°) and good ulnar length compared with other surgical techniques, but a slightly worse hand-forearm angle (28°). CONCLUSIONS: There was low-quality evidence that soft-tissue distraction plus centralization or radialization achieved the best correction of the hand-forearm angle for children with radial dysplasia. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Surgical Treatment of a Rare "Reverse" Madelung Deformity in 11 Years Female Patient.

Madelung deformity is an abnormality of the distal part of the forearm due to a growth arrest in the distal radial physis creating an increase of the radial tilt angle associated with a dorsal subluxation of the distal ulna in most cases. It is a rare condition which represents only 1.7% of hand deformities being characterized by the presence of an abnormal structure, Vickers ligament, that tethers the distal radius to the lunate bone. Although it is believed to be a congenital disorder, the symptoms are absent till late childhood. We present a case of a 11 years old girl patient, who came to our clinic for deformity of both forearms, which consisted of an anteriorly curved radius, volar proeminence of the distal ulna, partial limitation of supination and pain in the last 6 months, with and insidious onsed and aggravated lately. The mother of the patient, at the age of 13, was diagnosed with the same deformity which was surgically treated at that time. Furthermore, the patient has an older sister with no deformity of the forearms. X-rays revealed an increased radial tilt and anterior luxation of the distal ulna. Considering the deformity and the presence of pain we decided to excise the Vickers ligament and make an opening and derotation wedge osteotomy of the distal radius.

Metacarpal Bone Plane Examination by Ultrasonography for the Diagnosis of Fetal Forearm and Hand Deformity.

We explored the value of the metacarpal bone plane in screening for serious fetal forearm and hand deformities, excluding simple polydactyly and dactylion deformity, by ultrasonographic examination. Observed the second to fifth metacarpal bone plane of fetuses in 20,139 pregnant women at a gestational age of 16 to 30 weeks in The International Peace Maternity &Child Health Hospital of China Welfare Institute (IPMCH). There was a total 138 cases of fetal forearm and/or hand deformity among the 20,139 pregnant women. Of these, 134 cases were diagnosed, 4 cases were not diagnosed, and 1 case was misdiagnosed. Among the 134 diagnosed cases, there were 19 cases of hand absence, 5 cases of cleft hand, 13 cases of ectrodactyly, 26 cases of radius absence, 9 cases of forearm and hand dysplasia, 55 cases of thanatophoric dysplasia, 6 cases of wrist joint dysplasia, and 1 case of forearm amputation deformity. The deformity rate was 0.76%, the diagnostic coincidence rate was 99.97%, the sensitivity was 97.10%, the specificity was 99.99%, and the false negative rate was 2.9%. As such, careful observation of the metacarpal bone plane can be used increase the diagnosis rate of fetal forearm and hand deformity.

That's my STYLEoideum - Symptomatic os styloideum in an adolescent male.

We present a 15-year-old male patient with persistent localized pain on the dorsal side of the left wrist between the base of the 2nd and 3rd metacarpal bones and over the third carpometacarpal joint. It was diagnosed as an accessory metacarpal bone -Os styloideum. This entity may be detected on plain radiographs and in ultrasound examination and is often asymptomatic. Symptomatic os styloideum occurs more frequently in the dominant hand and may be treated conservatively with corticosteroid infiltration. A palpable prominence on the dorsal side of the wrist and focal pain evoked anxiety of the adolescent patient who searched medical consultation. In the clinical examination, a bony protrusion was confirmed and different possible diagnoses have been considered. After treatment with a corticosteroid infiltration of the third carpometacarpal joint under fluoroscopy the pain resolved completely. We would like to draw attention of clinicians and radiologists to this rare anatomical variant that normally is asymptomatic, and therefore not immediately recognized. Acquaintance with this entity and its early detection may lead to conservative treatment instead of surgical excision. A comprehensive literature search, review and discussion about os styloideum are provided in the article.

Unilateral fusion of the scaphoid and the trapezium.

Fusion of the carpal bones is a rare anatomical abnormality, caused by a defect in the separation and cavitation during embryonic development. It has a prevalence of about 0.1% and most of the time, patients do not present with symptoms. Symptoms are usually caused by wear and tear of the ligaments attached to the fused carpal fragments. We present a case in which a woman aged 36 years experiences pain caused by a lunato-triquetral instability which in turn is caused by a unilateral complete fusion of the scaphoid and the trapezium. A unilateral complete fusion of the scaphoid and trapezium has not been reported before.

Anatomic Anomalies Encountered in 467 Open Carpal Tunnel Surgeries.

Carpal tunnel syndrome is the most common compression neuropathy and carpal tunnel surgery is the most frequently performed hand surgery. Anatomic anomalies may predispose the median nerve to compression. The aim of the current study was to search for anatomic anomalies in open carpal tunnel surgeries through a cross-sectional study. During a cross-sectional study in a one-year period, 436 consecutive patients (307 females and 129 males) with the average age of 50.3 ± 2.4 years underwent 467 classic open carpal tunnel surgeries. Thirty-one patients had bilateral surgeries. A thorough inspection of the incisions was conducted to search for vascular, neural, tendon and muscular anomalies. Forty-two (8.9%) hands (14 males and 28 females) had anomalies. The average age of the patients with discovered anomalies was 48.6 ± 7.6 years. Ten anomalies were seen on the left hands and 32 anomalies were seen on the right hands. Among the 42 anomalies, there were 16 persistent median arteries, 14 anomalies of the median nerve, 7 intratunnel intrusion of the flexor and lumbrical muscle bellies and 5 anomalies of the origin of the thenar muscles. There was no correlation between the discovered anomalies and the age, gender or hand sides. Anatomical anomalies are not uncommon in carpal tunnel surgeries. However, the frequencies of the reported anomalies vary among different studies. Familiarity with these anomalies increases the safety of the operation.

Ultrasonographic Evaluation of the Prevalence of an Intracompartmental Septum in Patients With de Quervain's Disease.

It has been reported that more patients with de Quervain's disease who had undergone surgical treatment had a septated dorsal compartment than did normal cadavers. The purpose of this study was to sonographically evaluate the prevalence of an intracompartmental septum in patients with de Quervain's disease and to compare the prevalence between groups categorized by sex, age, and peripartum status. The authors performed an ultrasonographic examination of 112 wrists from 103 patients with de Quervain's disease. The prevalence of a septum-like structure in the first compartment was compared between men and women, between older (≥40 years) and younger (≤39 years) patients, and between pregnant or lactating women and other patients. The prevalence of intracompartmental septum in patients with de Quervain's disease was 61.6% (69 of 112). Of the 69 wrists with an intracompartmental septum-like structure, 53 showed this structure completely through the level of the radial styloid, and 16 showed it partially on the level of the distal radial styloid. There was no significant difference between any 2 groups categorized by the patients' demographics. The prevalence of intracompartmental septation in the patients with de Quervain's disease was higher than the previously reported prevalence in cadavers and lower than that of patients who underwent surgery. This result was consistent with a previous report that patients with a septated dorsal compartment may be more at risk of contracting de Quervain's disease and more prone to failure of nonoperative treatment.