Contracture Research Articles

P59 Multicentric osteolysis nodulosis and arthropathy syndrome: a case report in Erbil - Iraq

Abstract Introduction Multicentric osteolysis nodulosis and arthropathy (MONA) is inherited in an autosomal recessive manner. At conception, each sib of an affected individual has a 25% chance of being affected, a 50% chance of being an asymptomatic carrier, and a 25% chance of being unaffected and not a carrier. Once the MMP2 pathogenic variants have been identified in an affected family member, it is a skeletal dysplasia characterized by progressive osteolysis (particularly of the carpal and tarsal bones), osteoporosis, subcutaneous nodules on the palms and soles, and progressive arthropathy (joint contractures, pain, swelling, and stiffness). Case description The patient is a 15-year-old female living in Erbil, Kurdistan. Her condition started when she was 10 months old, as she began walking and experienced recurrent sore throat. At age 4 year, she had a history of falling, developed stiffness and contracture in her elbow that made it difficult to move. A few months later, she developed a painful swelling/mass in her left foot that interfered with her walking. She had delayed tooth eruption, with only 2 teeth appearing per 2 years, her fontanelle did not close until age 3 years. At age 4, she had a fall that caused stiffness in her right hand. Over the next few years, she developed masses/swelling and pain/stiffness in her feet, hands, elbows, knees, and ankles, leading to deformities. She did not have any hearing problems but missed 3 years of school due to her condition. Back pain started 3 years ago and has been decreasing Family History: Her sister, at age 4, had pain and mild swelling in her hands and feet, along with facial abnormalities. Another 4-year-old child in the family was admitted to the ICU with similar symptoms. Complete blood count, serum biochemistry, rheumatoid profile was normal. Ultrasound of abdomen and ECHO was normal. On radiological imaging done for hands, feet, spine, in which in hands has diffuse generalized osteopenia, decrease joint spaces, resorptions of the phalanges, or cortical thinning and expansion of the phalangeal, metacarpal and metatarsal bone with deformity, and in x-ray of spine shows osteopenia with scoliosis. Dexa scan done for hip and spine which reveals (-6) severe osteoporosis. The results of genetic testing on 26th of October, led to the diagnosis of MONA syndrome. The patient was started on supportive treatment (alendronate tablet 70 mg per week, Vit D tablet 5000 IU per day, NSAID for pain and physiotherapy). Discussion The existing literature on MONA syndrome has broadly characterized the skeletal manifestations in the hands and feet. However, more detailed information is needed regarding specific skeletal involvement, ray involvement (which fingers/toes are affected), type and number of joints affected, laterality (whether the involvement is symmetrical or asymmetrical), type and severity of deformities, characteristic sparing of individual joints, if any longitudinal studies could help track the chronological order of joint involvement, especially in the larger or proximal joint Research on MONA syndrome has described the skeletal manifestations in the hands and feet, but further study is needed to understand the specifics of ray involvement, joint involvement, laterality, and severity of deformity. Longitudinal studies can help track the progression of joint involvement, particularly in larger joints. The recurrence of symptoms after orthopedic surgery in one patient highlights the progressive nature of the syndrome and suggests that early surgical intervention may not be effective. Due to the rarity of MONA syndrome, it is difficult to fully understand the pattern of the syndrome’s phenotype. Furthermore, reported correlations between the syndrome’s genetic and phenotypic characteristics have been inconsistent and inconclusive The treatment for MONA syndrome is primarily supportive and includes physical therapy to slow the development of contractures and prolong mobility. Pain relief may not be achieved through medication unless the pain is caused by secondary osteoarthritis or contractures. Additionally, daily recommended supplementation of vitamin D and calcium; standard management of congenital heart defects; management of scoliosis and kyphosis per orthopedist. Key learning points • The complicated diagnostic process for MONA syndrome necessitates a re-review of the reported phenotype-genotype correlations. By expanding the understanding of the phenotype, including the detailed skeletal manifestations, the goal is to reach a consensus on the diagnostic criteria for MONA syndrome. The aim of this case report was to systematically describe the clinical manifestations, with a focus on the skeletal features, in children with molecularly confirmed MONA syndrome, and to establish relevant phenotype-genotype correlations.

Spine deformity surgery in patients with Beals syndrome can be effectively performed but does risk revision surgery.

Beals syndrome or congenital contractual arachnodactyly is a rare disorder characterized by multiple joint contractures, Marfanoid body habitus and crumpled ears and has been associated with scoliosis. This study reports a clinical series of patients with Beals syndrome who have had surgical treatment for their spinal deformity. A retrospective review of all patients at a single institution who had a genetically-confirmed diagnosis of Beals syndrome and had surgical treatment for their scoliosis were reviewed for surgical outcome and complications. There were eight patients who had surgery at an average age of 11.5years, four were female and four had cardiovascular abnormalities requiring treatment. The preoperative coronal Cobb was 82.3° which improved to 42.1°(46.8% correction), and 46.5° (43.5% correction) at final follow-up. Preoperative halo-gravity-traction was used in three patients. Three patients had a posterior instrumentation and fusion (PSFI), 2 a combined anterior/PSFI, 1 had tethering, 1 with PSFI with posteriorly-approached discectomy, and 1 with a PSFI and vertebral column resection. One of the eight patients had a critical intraoperative neuromonitoring event but was normal following appropriate responses and no patient awoke with neurologic deficits. Two had an unplanned return to the operating room for implant dislodgement and each had a successful revision. Scoliosis associated with Beals syndrome can have large curves at the time of surgery and require a variety of surgical approaches to achieve a good result. Revision surgery with return to the operating room is necessary in 25% of patients.

Motion-mimicking Robotic Finger Prosthesis for Burn-induced Partial Hand Amputee: A Case Report.

Burn injuries often result in severe hand complications, including joint contractures and nerve damage, sometimes leading to amputation. Despite early treatment, hypertrophic scarring frequently hampers hand function recovery, and the thick raised scar blocks electromyography (EMG) sensing. A promising solution involves motion-mimicking robotic finger prostheses tailored to individual patient requirements. By utilizing the versatility of motion-capturing technology on a sound finger, a robotic finger prosthesis can mimic the movement of a sound finger simultaneously with less latency than EMG-based sensory mechanisms through hypertrophic scars. This case study evaluated the clinical efficacy of a customized three-dimensional printed robotic finger prosthesis in a 24-year-old man who sustained left second finger loss due to electrical burns. Despite undergoing reconstructive surgery, the patient struggled with manual dexterity. Following the adoption of a personalized robotic finger prosthesis with a finger motion-capturing device, significant improvements in grip strength and daily task performance were observed. This innovative approach has advantages such as customization, reduced latency time for finger movements, and affordability from low-cost manufacturing, suggesting its potential for broader adoption among burn-induced amputees.

Fatigue levels and associated factors in systemic sclerosis: a cross-sectional study of 2,385 SPIN Cohort participants.

To compare fatigue in a large multinational systemic sclerosis (SSc) cohort to general population data and identify associated sociodemographic, lifestyle and SSc disease factors. Scleroderma Patient-centered Intervention Network Cohort participants completed the Patient-Reported Outcomes Measurement Information System-29 v2.0 fatigue domain. T-scores were compared with the USA general population (mean = 50; SD = 10). Multivariable linear regression was used to assess associations with sociodemographic, lifestyle, and disease-related variables. Among 2,385 participants (mean age 54.9 (SD = 12.6) years, 87% female, 38% diffuse SSc), mean fatigue T-score was 54.6 (SD = 11.0); 438 (18%) reported mild fatigue, 641 (27%) moderate, and 180 (8%) severe fatigue. Fatigue was independently associated with sociodemographic factors age (-0.10 points per year, [95% CI -0.14;-0.07]), male sex (-1.67 points, [-2.96;-0.37]), non-married status (0.97 points [0.04; 1.89]), and country (reference USA; France -2.35 points [-3.48;-1.21] and UK 2.38 points [0.80; 3.97]), and lifestyle factors smoking (4.16 points [2.52; 5.80]), alcohol consumption (-0.18 points per drink per week [-0.28;-0.07]), and body-mass index (0.34 points per unit [0.27; 0.42]). Fatigue was associated with disease-related factors gastrointestinal involvement (4.21 points [2.99; 5.43]), digital ulcers (1.51 points, [0.25; 2.77]), moderate small joints contractures (1.41 points [0.13; 2.69]), rheumatoid arthritis (4.34 points [2.37-6.31]) and Sjögren's syndrome (1.89 points [0.23; 3.55]). When pain was included in the model, its association was large (2.19 points [2.03; 2.34]) and interstitial lung disease was also associated (1.21 points [0.42; 2.00]). In people with SSc, fatigue scores were substantially higher than the general population and associated with multiple disease factors including gastrointestinal involvement, several painful disease manifestations, and lung involvement.

Surgical correction of posttraumatic triphalangeal joint flexion contractures of the fingers (systematic literature review)

Introduction Triphalangeal joint flexion contracture of the fingers is a common and challeging posttraumatic hand condition. The goal of surgical treatment is to correct finger deformity and increase interphalangeal range of motion.The objective was to systematize data on the causes of post-traumatic triphalangeal joint flexion contracture of the fingers and methods of surgical correction.Material and methods The original literature search was conducted on key resources including Scientific Electronic Library (www.elibrary.ru), the National Library of Medicine (www.pubmed.org), ScienceDirect, Google Scholar, Ovid databases according to PRISMA recommendations. Literature searches included both Russian and English studies, with one or more cases of post-traumatic triphalangeal joint flexion contracture of the fingers with the deformity surgically corrected. Cases of non-traumatic flexion contractures were excluded. Etiological factors of flexion contractures, heterogeneity of definitions and methods for recording the range of motion in the joint, anatomical features, surgical correction of flexion contractures and postoperative complications were reviewed.Results Common causes of flexion contractures included burns (32.3 %), dislocations and fracturedislocations of the finger joints (23.5 %). The median postoperative follow-up period was 13.5 months after surgical treatment. Surgical correction was produced with external fixation device (EFD) in 40 % of cases, open procedures performed in 50 % and a combined technique employed in one case (10 %). Based on calculations of the odds ratios of postoperative complications, a weak positive linear relationship was revealed between EFD and pain syndrome, and a weak negative linear relationship was observed between the open procedure and pain.Discussion There is heterogeneity of approaches regarding methods for correcting flexion contractures, surgical approaches, techniques for mobilizing joints and releasing the anatomical structures of the finger with open procedures, the distraction rate with EFD, methods for repair of soft tissue defects following the treatment of flexion contractures of interphalangeal joint of a finger.Conclusion Open procedures are commonly used for precise elimination of all components of flexion contracture of the joint and repair of soft tissue defects of the finger. A weak positive linear relationship was revealed between EF and pain syndrome. There was no significant correlation between open techniques and complications. There were no correlations between the treatment method and the contracture type; there are no treatment regimens for patients with this pathology.

The Effect of Vertebral-Pelvic Mobility on the Technology and Outcomes of Total Hip Replacement

Introduction. Total hip arthroplasty (THA) is one of the most commonly used surgical interventions among patients with diseases of the hip joint. Despite the high frequency and effectiveness of THA, instability is the main reason for repeated surgical interventions, which have a significant impact on the quality of life of patients.Purpose. Analysis of literature data on spinal-pelvic interactions, development of recommendations for eliminating pathologies of the spine and pelvis based on the analyzed data.Materials and methods. The author searched for publications in the electronic databases PubMed and eLibrary. ru using the following search terms: “spinopelvic mobility, total hip replacement, hip arthroplasty, vertebral-pelvic mobility, total hip replacement, hip arthroplasty”.Results and discussion. The orientation of the pelvic component should be planned according to hip motion assessment to position the pelvic portion of the endoprosthesis according to the new safe zone and combined sagittal index. However, the surgeon must take into account that postoperative mobility of the pelvis and spine may differ from preoperative mobility due to joint contracture or physiological aging of the spine. To date, it has not yet been determined how often these changes lead to the pelvic component of the endoprosthesis moving beyond its position and spatial location, increasing the risk of dislocation.Conclusion. A proper understanding of the relationship between the hip joint and the spine, and the impact of their interactions on the positioning of the acetabular component, is critical to reducing the risk of dislocation.

Transforming rehabilitation: A narrative review on reversible knee flexor contracture assistive devices

Abstract: Joint contracture, marked by restricted joint movement due to connective tissue and muscle shortening, is a common complication in chronic musculoskeletal conditions such as osteoarthritis, osteonecrosis, rheumatoid arthritis, healed septic joint, and postsurgical complications. This limitation adversely impacts joint mobility and flexibility, increasing the likelihood of physical constraints. Contractures elevate the risk of impaired self-care, limited physical mobility, and hindered social activities, emphasizing the critical need to manage such contractures. The study aims to find the most appropriate, effective, user-friendly mechanical device to treat reversible knee flexor contracture. Method of a literature review was conducted utilizing PubMed, Google Scholar, and the physiotherapy evidence database (PEDro) up to April 2023. The inclusion criteria comprised studies related to flexion contracture (FC), written in English languages were included. The literature searched using the terms “flexion contracture, hamstring contracture, knee flexor contracture, burn contracture, knee joint hypomobility, and devices for flexion contracture.” Result after applying the selection criteria, the initial screening of literature gives 35,400 results on Google Scholar, PubMed, and PEDro. Subsequently, 128 articles underwent screening based on abstract and full-text availability in the English language. Following this, seven articles were selected and thoroughly reviewed, which included randomized control trials, systematic reviews, and exploratory studies. The study concluded the use of conventional physiotherapy interventions, coupled with assistive devices, diminishes the burden on physiotherapists and provides effective improvements to patients with FCs.

LMNA-Cardiomyopathy in Emery-Dreifuss Muscular Dystrophy

Emery-Dreifuss muscular dystrophy is a rare disease resulting from a genetic defect in nuclear envelope proteins, most commonly in emerin and lamin A/C. The disease is characterized by slowly progressing weakness of the scapular-brachial and pelvic-peroneal muscle groups, myodystrophy, primary joint contracture and cardiomyopathy with rhythm disorders and conduction abnormalities. Cardiovascular complications and life-threatening arrhythmias are the main cause of death in such patients at a young age. Depending on the leading symptoms and family history, patients are under the care of different specialists. Unfortunately, neurologists, cardiologists, cardio surgeons and orthopedics are not well informed about this rare condition and thus the disease tends to be not diagnosed in time. This article examines the data of epidemiology, pathophysiology, features of the course, diagnosis, approaches to the management of cardiovascular pathology in progressive Emery-Dreyfus muscular dystrophy with the development of LMNA cardiomyopathy. A clinical case of this disease is also given.

Do Not Forget the Spine MRI in Children With Arthrogryposis Multiplex Congenita: High Prevalence of Tethered Spinal Cord and Preliminary Clinical Findings Following Detethering.

Arthrogryposis multiplex congenita (AMC) encompasses congenital conditions with joint contractures in 2 or more joints. Patients with AMC may have scoliosis and neural axis malformations. The study aimed to determine the prevalence of tethered spinal cord (TSC), characterized by a low-lying conus medullaris, and secondarily, present preliminary findings following surgical untethering in children with AMC. Patients 18 years of age and younger with a diagnosis of AMC and a spine MRI were identified. The presence of a TSC was defined as a low-lying conus with termination at or below the lower third of the L2 vertebral body. A pediatric neurosurgeon and a pediatric orthopaedic surgeon independently reviewed MRIs. The medical records of patients with AMC who underwent untethering were examined to evaluate preoperative and postoperative clinical findings. The prevalence of TSC in our AMC patients was compared with published normative data using χ2 analysis. Forty-two of 105 AMC patients (40%) had TSCs. There was a greater percentage of patients with AMC and a TSC compared with the unaffected population (P<0.0001). Sixteen patients underwent detethering through filum terminale sectioning. Nine patients had preoperative neurological deficits in addition to their AMC. There were no postoperative complications. All patients had improvement in their bowel and bladder symptoms within 3 months after their detethering procedure. TSC is more prevalent in the pediatric population with AMC compared with those without AMC. MRI is recommended for all patients with AMC due to its high prevalence. Although not a benign procedure, surgical detethering may have potential benefits for children with AMC. Level IV-therapeutic study.

Therapy for Dupuytren’s Disease: Collagenase Therapy—A Long-Term Follow-Up Study

Background: Dupuytren’s disease (DD) is a systemic connective tissue disorder of the palm. It particularly affects men of Northern European or Caucasian origin over the age of 55. In addition to the classical surgical therapy via limited fasciectomy, Dupuytren’s contracture can also be treated minimally invasively. A relatively new treatment method is the use of collagenase injections (Xiapex) to reduce the contracture of the fingers. The data regarding the long-term success of this therapy are currently limited. Methods: In this monocentric retrospective study, we examined 35 patients who were treated with collagenase (Xiapex) for Dupuytren’s contracture in the long fingers. Following the manufacturer’s recommendations, the injection was administered intralesionally, and the cord was ruptured through the passive extension of the finger under local anesthesia with Mepivacain the following day. The clinical follow-up examination was conducted after an average of 5.7 years. The stages of Dupuytren’s disease were documented using the Tubiana classification. Additionally, parameters of finger extension ability, differentiated by metacarpophalangeal (MCP), and proximal interphalangeal (PIP) joints, as well as patient-specific risk parameters, were evaluated Results: The long-term results of collagenase therapy after an average of 5.7 years showed a significant improvement in the contracture of the affected fingers. In the MCP joints, the flexion contracture decreased from 42° to 17° (p ≤ 0.001), and in the PIP joints, it decreased from 56° to 33° (p ≤ 0.001). The primary recurrence rate was 11% for the MCP joints and 19% for the PIP joints, respectively. The analysis of risk factors showed a significant risk for worse long-term outcomes in patients with diabetes and those with nicotine abuse. Conclusions: Collagenase therapy for Dupuytren’s disease achieved significant long-term improvements in contracture in both MCP and PIP joints. In accordance with general risk factors for DD, patients with diabetes and those with nicotine abuse are at risk of worse long-term outcomes. Overall, it is a time-saving, low-risk, and straightforward technique for treating the disabling contracture component of this disease.

Lower Limb Comprehensive Reconstructive Surgery: Experiences from an Orthopedic Teaching Unit

Abstract Introduction: The burden of open extremity fractures is well recognized in placing a burgeoning demand on an already exhausted medical facility in low- and middle-income countries. This study aims to evaluate the functional outcome following “orthoplastic management” by orthopedic surgeons trained in limb reconstruction surgery. Methodology: Adult patients with open lower limb injuries treated from January 1, 2022, to December 30, 2022, who underwent single or staged fixation and reconstruction procedures were identified. Patients with Gustilo–Anderson type IIIB fractures requiring skeletal stabilization with soft-tissue cover were included. Polytrauma patients requiring multidisciplinary surgical attention were excluded. A team of orthopedic surgeons trained in limb reconstruction was involved in the management. Inpatient and outpatient department follow-up by the treating team was done to document complications (infections, reoperations, and graft failures) and functional outcomes using the Lower Extremity Function Score. The duration of mean follow-up was 1 year since index surgery. Results: A total of 24 adult patients with open lower limb injuries with preoperative Ganga Hospital Score (GHS) were included. Decision toward fixation with primary closure or further reconstruction was made collectively. The gray zone (GHS score of 15–16) of patients was more aggressively managed with emphasis on salvage. Additional reconstructive procedures were carried out based on the assessment at consequent follow-up. Three patients had a suboptimal joint function (less than functional range of motion for the involved joint) due to the resultant loss of compartment(s) and joint contracture. Five patients were retaken to the theater for further supplementary or corrective procedures following the index, definitive surgical procedure (bone grafting, partial flap loss, and fixator readjustment). Conclusion: Good-to-excellent functional outcome was demonstrated with the “orthoplastic approach” where skeletal stabilization as well as soft-tissue reconstruction was performed by trained orthopedic surgeons. This study demonstrated that orthoplastic surgery is a safe and effective method of limb salvage.

Clinical utility of extracorporeal shock wave therapy in restoring hand function of patients with nerve injury and hypertrophic scars due to burns: A prospective, randomized, double-blinded study.

Joint contractures and nerve injuries are common after hand burns. Extracorporeal shock wave therapy (ESWT) is effective not only for the regeneration of various tissues, including scar tissues, but also for reducing pain and pruritus in patients with burns. Researchers have attempted to explore the effects of ESWT on hand dysfunction caused by nerve injury following burns. We evaluated the effects of ESWT (compared to sham stimulation) on hands with nerve injury and hypertrophic scars and thereby on hand function. The current study was a double-blind randomized controlled trial involving 120 patients. The ESWT parameters were as follows: energy flux density, 0.05-0.30mJ/mm2; frequency, 4Hz; 1000 to 3000 impulses per treatment; and 12 treatments, one/week for 12 weeks. Outcome measures were as follows: 10-point visual analog scale for pain, Jebsen-Taylor hand function test, grip strength, Purdue Pegboard test, ultrasound measurement of scar thickness, and skin characteristics before and immediately after 12 weeks of treatment. No significant inter-group difference was noted after the initial evaluation (P>0.05). More significant improvements were found in the ESWT group than in the sham group in terms of the VAS score (P=0.004), extension ROMs of hand joints (P=0.02), the JTT scores (writing, small, and light) (P<0.001, P<0.001, and P=0.002), and skin characteristics (melanin, skin distensibility, and biologic skin elasticity) (P=0.004, P<0.001, and P<0.001). Other measured outcomes did not differ between the two groups after the treatment. We identified the clinically beneficial effects of ESWT in promoting hand function, improving scarring, and alleviating scarring-related pain, thereby highlighting its advantages in improving hand function that has been impaired due to nerve injury and hypertrophic scars after burns.

Surgical Repair of Multiple Pulley Injuries with Proximal Interphalangeal Joint Contracture under WALANT

Surgical Repair of Multiple Pulley Injuries with Proximal Interphalangeal Joint Contracture under WALANT

Impact of Serial Casting on Autonomic Nervous System Responses during Virtual Reality Tasks in Children with Cerebral Palsy: A Pilot Study Comparing Orthoses and Barefoot Conditions.

Cerebral palsy (CP) is a group of movement disorders that impair posture and mobility, often leading to spasticity and joint contractures. Interventions like serial casting are commonly used to improve joint mobility and manage spasticity in children with CP. However, its effects on the autonomic nervous system (ANS) remain unclear. This study aimed to evaluate the effects of serial casting and ankle-foot orthoses (AFOs) on ANS responses during a virtual reality (VR) standing task, comparing these interventions with a barefoot condition. Thirty children with CP were randomized into three groups (n = 10 per group): serial casting, AFOs, and barefoot. Heart rate variability (HRV) was used to assess ANS responses across three phases: seated rest, VR task, and recovery. The results showed that the serial casting group exhibited higher sympathetic activity during rest compared to the other groups, but had a reduced sympathetic response during the VR task. Additionally, the serial casting group displayed a more pronounced parasympathetic rebound during recovery, similar to the orthoses and barefoot groups. While serial casting provides essential joint stability, it alters ANS response patterns, leading to heightened sympathetic activation at rest, without providing significant improvements in ANS behavior during physical activity.

Arthrodesis as Salvage for Failed Metacarpophalangeal Arthroplasty

Background: Surgical management of failed metacarpophalangeal (MCP) arthroplasties includes revision arthroplasty and arthrodesis. The purpose of this study was to review the indications and outcomes of arthrodesis as a salvage procedure for failed MCP arthroplasties. Methods: This was a retrospective cohort study of all patients undergoing salvage MCP arthrodesis at a single institution from 1990 to 2020. Patient charts were reviewed for patient demographics, indication for salvage, surgical technique, revision rate, and time to radiographic union. Patient-reported outcomes, including the Michigan Hand Outcomes Questionnaire and an MCP-specific questionnaire detailing pain, functional, appearance, and patient satisfaction, were also collected. Results: Eleven digits in 9 patients (6 women, 3 men, median age of 66) with median 36-month follow-up were included. The majority (91%) of patients also had concomitant soft tissue deformities, including joint contractures, extension lag, and collateral ligament insufficiency. The overall revision rate following arthrodesis was 45% with 3 digits requiring one revision each, and 2 digits undergoing 3 revisions. The overall union rate was 91% with median time to union of 4 months from most recent arthrodesis. Patient-reported outcomes obtained from 4 patients demonstrated improvements in pain and function. Conclusion: Despite a high revision rate of 45%, salvage MCP arthrodesis following arthroplasty has a high eventual union rate of 91% and is associated with improved pain and function based on 4 patients’ experiences. Arthrodesis as a salvage procedure for failed MCP arthroplasties should be considered in patients with persistent joint instability and functionally limiting soft tissue deformities.

Individual Joint Distractions Using Ilizarov Mini-Fixator Device for Chronic Simultaneous Dislocation of the Distal and Proximal Interphalangeal Joints in the Same Finger

Simultaneous dislocation of the distal and proximal interphalangeal joints is an uncommon injury. Treating chronic cases proves challenging due to severe contractures affecting both joints and surrounding soft tissues. Here, we report an unusual chronic contracture case in a 46-year-old man who suffered a dorsal dislocation of the distal and proximal interphalangeal joints of the little finger. The patient underwent primary joint distraction followed by open reduction, leading to stable radiographic restoration and a functional range of motion in the affected finger 6 months postoperatively. Joint distraction was a reliable approach for the treatment of chronic joint dislocation with severe soft tissue contracture, especially for multiple joint contractures. In addition, the Ilizarov mini-fixator device was effective in enabling individualized traction on both the distal and proximal interphalangeal joints.

Rheumatological manifestations of H syndrome.

H syndrome (HS) is a rare autosomal recessive genodermatosis characterised by cutaneous hyperpigmentation, hypertrichosis, sclerodermatous thickening, and multisystemic involvement. It results from mutations in the SLC29A3 gene encoding the human equilibrative nucleoside transporter 3, leading to impaired histiocyte apoptosis and unchecked proliferation. We report the case of a 24-year-old Moroccan male who had a history of insulin-dependent diabetes mellitus. He developed hyperpigmented skin patches with hypertrichosis and induration. Musculoskeletal findings included bilateral hallux valgus, pes planus, reducible flexion contractures of the proximal interphalangeal joints, and restricted ankle dorsiflexion. Additional findings consist of lymphadenopathy, hepatomegaly, hypogonadism, and ophthalmic manifestations. Investigations showed elevated sedimentation rate, anaemia, and osteopaenia. Ankle ultrasound revealed calcaneal enthesopathy and subcutaneous infiltration. In reporting this case, we aim to highlight the significant rheumatological involvement that can arise in patients with H syndrome and explore potential treatment options to improve the musculoskeletal findings.

Extracorporeal Shock Wave and Melatonin Alleviate Joint Capsule Fibrosis after Knee Trauma in Rats by Regulating Autophagy.

Joint contracture is a common clinical problem affecting joint function. Capsule fibrosis plays a pivotal role in the progression of Joint contracture. Previous studies have reported that autophagy plays a regulatory role in visceral fibrosis. This study aimed to investigate whether extracorporeal shock wave therapy (ESWT) and melatonin alleviate joint capsule fibrosis in rats with extended knee joint contracture by regulating autophagy. A rat knee joint extension contracture model was made. Then, the rats were treated with ESWT, melatonin, ESWT + melatonin, or ESWT + melatonin + mTOR agonist for 4 weeks. The range of motion (ROM) of the knee joints was measured. Joint capsules were collected and observed for pathological changes by H&E and Masson staining. LC3B protein expression was evaluated by immunofluorescence staining. TGF-β1, MMP-1, Col-Ⅰ, Col-Ⅲ, LC3, ATG7, Beclin1, p-AMPK, p-mTOR and p-ULK1 protein expressions were measured by Western blot assay. The intervention groups had significantly improved ROM of knee joint (P < 0.05), significantly improved pathological changes on HE and Masson staining, significantly decreased protein expressions of TGF-β1, MMP-1, Col-Ⅰ, Col-Ⅲ and pmTOR (P < 0.05), and significantly increased protein expressions of LC3B, LC3II/LC3I ratio, ATG7, Beclin1, p-AMPK, and p-ULK1 (P < 0.05). Among these groups, the effects demonstrated by the ESWT + melatonin group were the best. With the mTOR agonist supplement, the therapeutic effects of extracorporeal shock waves and melatonin were significantly reduced. ESWT plus melatonin alleviated knee joint capsule fibrosis in rats by regulating autophagy.

Does the timing of anterior cruciate ligament reconstruction surgery affect flexion contracture formation in rats?

BackgroundAnterior cruciate ligament injuries are commonly treated with ligament reconstruction surgery, but post-operative joint contracture is a major complication. The optimal timing for anterior cruciate ligament reconstruction remains controversial, with some clinical studies suggesting that early surgery may increase the risk of joint contractures, while others have found no such association. To clarify this, we investigated the effects of the timing of reconstruction surgery on contracture formation using a rat model. MethodsAnterior cruciate ligament-transected rats were divided into groups based on the timing of reconstruction: immediate, early, and delayed (1, 14, and 28 days after transection, respectively). Some anterior cruciate ligament-transected rats did not receive reconstruction surgery. Untreated rats served as controls. At 56 days after ligament transection, we assessed knee extension range of motion before (including both myogenic and arthrogenic factors) and after myotomy (arthrogenic factor only), as well as fibrotic changes in the joint capsule. FindingAnterior cruciate ligament transection alone significantly decreased range of motion before myotomy, but not after myotomy. In all reconstructed groups, both range of motions before and after myotomy were significantly reduced compared to the control, indicating the induction of arthrogenic contracture by reconstruction surgery. Fibrotic changes in the joint capsule were observed in all reconstructed groups, contributing to arthrogenic contracture formation. However, the timing of reconstruction had no effect on range of motions and fibrotic changes in the joint capsule. InterpretationOur findings may help guide clinical decision-making regarding the timing of anterior cruciate ligament reconstruction surgery.

Exercise for patients with neuromuscular diseases

Background: Neuromuscular diseases (NMD) vary widely with regard to their onset, progression, and symptoms, which include muscle weakness, sensory loss, pain, fatigue, and joint contractures. Owing to this diversity, creation of a unified exercise therapy approach is challenging.Current Concepts: Exercise recommendations for patients with NMD prioritize maintenance of patients’ current functional status, prevention of further decline, and provision of compensatory training. Conventionally, highintensity exercises are not recommended owing to the risks of overwork weakness and muscle injury. Recent studies indicate that tailored aerobic exercises can improve endurance, cardiovascular function, and overall health without worsening muscle injury. Flexibility exercises are essential to prevent joint contractures. Resistance training should focus on avoidance of high-intensity and eccentric exercises and on maintenance of rather than an increase in muscle strength.Discussion and Conclusion: Evidence regarding the benefits of exercise in patients with NMD is inconsistent. Aerobic exercise is usually safe and may help maintain function and increase maximal oxygen uptake; however, low-intensity exercise is preferred to avoid fatigue and muscle injury. Resistance training may help preserve muscle strength in some patients; however, high-intensity workouts are not should be avoided to mitigate the risk of muscle overload and injury. Tailored exercise programs designed according to disease characteristics are essential.. Further well-designed, large-scale studies are necessary to establish effective and safe exercise guidelines for patients with NMD.