What has changed in total hip arthroplasty in patients of juvenile idiopathic arthritis since 2000? A systematic review and pooled data analysis.

PURPOSE: The prevalence of juvenile idiopathic arthritis (JIA) is estimated to be 16-150 per 100,000 children worldwide. The hip joint may be involved in over 50% of children leading to significant morbidity which may require surgical intervention in the form of arthroplasty. The literature lacks a concise overview of the outcomes, including complication and implant survival of total hip arthroplasty (THA) in juvenile idiopathic arthritis (JIA). The aim of this study is to systematically analyze the literature and report the outcomes of THA in JIA. METHODS: Search was conducted in the online databases PubMed, Embase and Cochrane database. It included all original studies which evaluated clinical and/or radiological outcomes of THA in JIA with a minimum sample size of 5 patients and published in English. The level of evidence of the included studies was graded according to the Oxford Centre for Evidence Based Medicine. The Institute of Health Economics checklist was used to assess the quality of the studies included. RESULTS: The nine studies included were retrospective in nature with all being Level IV according to Oxford Centre for Evidence Based Medicine. 475 hips in 304 patients with majority of them being females (241/304, 79.2%) were included in this review. All the studies reported the outcome objectively using various scores. The proportion of revision surgeries (92/378), either femoral or acetabular, noted was 22% (95% CI 10-33%). The proportion of acetabular revisions (72/378) was 16% (95% CI 8-25%) as compared to 4% (95% CI 1-6%) for femoral revisions (20/378). There was no difference in survivorship when cemented and uncemented implants were compared. CONCLUSION: JIA patients with advanced hip disease represent a unique population with need for extra-long implant longevity. THA in patients of JIA leads to improved pain relief as well as mobility but the conversion of the same outcomes to functional activity is not proportionally improved. The current trend is the use of uncemented and ceramic-on-ceramic implants. Acetabular implants require earlier revision as compared to femoral implants. Age at surgery can be delayed by early institution of methotrexate which indirectly improves implant survival. LEVEL OF EVIDENCE: IV.

Good result for twelve years after bilateral hip and knee arthroplasties for ankylosis with juvenile idiopathic arthritis: a case report.

Juvenile idiopathic arthritis (JIA) can lead to joint deformity and bone destruction, which can cause gait disturbances. To the best of our knowledge, there are no case reports with over 10 years of follow-up on quadruple joint arthroplasties (QJA) for bilateral hip and knee ankylosis associated with JIA. We present the case of a 29-year-old woman with JIA. The patient suffered from bilateral ankylosis of the hips and knees and developed a swing gait requiring double crutches. We performed staged QJA with careful attention to postoperative rehabilitation and her physical features, which included excessive pelvic anteversion, poor bone quality, and short statue of bones. Twelve years after surgery, the patient was able to walk without any support and showed good clinical functional scores. In addition, no radiological loosening following QJA was observed. We hereby introduce a surgical strategy for total hip arthroplasty for excessive pelvic anteversion, which involves two methods to calculate pelvic tilt on a pelvic anteroposterior radiograph. These methods were able to approximately predict postoperative pelvic changes.

Two-Staged Total Knee Arthroplasty in Juvenile Rheumatoid Arthritis with Severe Flexion Deformity: A Case Report.

CASE: Juvenile rheumatoid arthritis (JRA) is associated with severe flexion contracture of the knees disabling a patient's ability to walk. Although various treatment modalities are available in the literature, we report a 2-stage procedure in a case of a 22-year-old female JRA patient with bilateral severe flexion deformity of the knee: stage 1-correction of the flexion contracture deformity by ring fixators and stage 2-total knee arthroplasty (TKA) for arthritis. To the best of our knowledge, no such approach has been reported previously. CONCLUSIONS: An Ilizarov ring fixator is a safe technique for gradual correction of a severe flexion deformity. Subsequent TKA can be performed as a standard procedure.

Synovectomy in juvenile idiopathic arthritis: A systematic review and meta-analysis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is an inflammatory arthropathy with onset in children younger than 16 years. Treatment is primarily medical; however, surgical interventions, such as arthroscopic or open synovectomy, can be beneficial. Many studies have investigated synovectomy in JIA, but the results of these studies have not been synthesized to our knowledge. Therefore, we performed a systematic review of the literature reporting synovectomy as a treatment for JIA to provide clinical recommendations regarding its risks and benefits. METHODS: On March 8, 2022, we searched the Cochrane Library, Embase, PubMed, Scopus, and Web of Science for studies evaluating clinical outcomes of open or arthroscopic synovectomy to treat JIA in patients younger than 18 years. We included only studies published in English and excluded studies of synovectomy to treat other arthropathies, septic arthritis, hemophilia, or foreign body arthropathy. The level of evidence for included studies was determined by using the Oxford Centre for Evidence-Based Medicine criteria. We qualitatively analyzed clinical outcomes data, including patient-reported pain relief, rates of symptom recurrence, and postoperative complications. RESULTS: Of 428 articles assessed, 14 were included in our analysis. One was a randomized trial, 1 was a case-control study, and all others were case-series. Studies consistently reported that synovectomy was associated with improved function and decreased pain postoperatively. However, comparisons with modern medical therapy were lacking. Rates of arthritis recurrence varied, with increasing symptom recurrence with longer follow-up and re-synovectomy rates up to 15%. Oligoarticular disease and early disease course were associated with better response to synovectomy, whereas systemic and polyarticular disease were associated with poor response. Stiffness requiring manipulation under anesthesia was the most common complication (4% of all included patients). CONCLUSION: Although synovectomy is associated with positive functional outcomes and pain reduction postoperatively, there was inadequate comparison thus inadequate evidence to recommend it over modern medical therapy. The current literature suggests that synovectomy should be offered only to patients for whom medical management has failed, while noting the risks of decreased range of motion and symptom recurrence over time.

Orthognathic surgery for juvenile idiopathic arthritis of the temporomandibular joint: a critical reappraisal based on surgical experience.

Juvenile idiopathic arthritis (JIA) involving the temporomandibular joint (TMJ) can result in significant dentofacial deformities that may require orthognathic surgical correction. The aim of this study was to assess the functional and aesthetic results relative to stability after bimaxillary surgery with counterclockwise rotation of the occlusal plane in patients with JIA. A retrospective chart review was conducted of all patients affected by JIA who underwent orthognathic surgery between January 2000 and December 2019 at the Face Surgery Centre (Parma, Italy). Patient records were evaluated for surgical indications, complications, and outcomes. The final study sample included 13 patients (12 female, one male). The mean age of the patients was 18.6 years (range 17-26 years) at the time of surgery; 12 patients had bilateral TMJ disease. At the 1-year follow-up, all patients except one had a stable occlusion with a natural, well-balanced morphology of the face and adequate dynamic excursion of the mandible. The 1-year postoperative cone beam computed tomography (CBCT) scan revealed complete ossification at all osteotomy sites. Bilateral sagittal split osteotomy with mandibular advancement is an effective procedure with a low rate of complications for patients with JIA with stable disease confirmed by preoperative CBCT or magnetic resonance imaging.

Does Combined Temporomandibular Joint Reconstruction With Patient Fitted Total Joint Prosthesis and Orthognathic Surgery Reduce Symptoms in Juvenile Idiopathic Arthritis Patients?

PURPOSE: Patients with juvenile idiopathic arthritis (JIA) and TMJ involvement may have major dentofacial deformities, pain, and jaw dysfunction. The aim of this study was to evaluate surgical outcomes for JIA patients relative to TMJ pain, headache, jaw function, diet, disability, and quality-of-life (QOL) after TMJ reconstruction with patient-fitted total joint prostheses (TJP) and concomitant orthognathic surgery. METHODS: A retrospective cohort study was conducted on a JIA patient group (JIAG) with significant dentofacial deformity, reconstructed with TJP and concomitant orthognathic surgery, and was compared to a control group (CG) of non-JIA patients that received the same surgical protocol with similar surgical movements. Primary predictors were the 2 groups: JIAG and CG. Data were evaluated and compared presurgery and at longest follow-up using Likert analog scales for the primary variables: TMJ pain, headache, jaw function, diet, and disability. Maximum interincisal opening (MIO) was measured in mm. QOL was rated in JIAG as improved, same, or worse. Comparative data were tested for significance (α = 0.05) using Wilcoxon signed rank and paired T-tests. RESULTS: Forty JIAG patients (8 males, 32 females) met the inclusion criteria, median age 17.5 years, median follow-up 26.5 months, and 26 CG patients (1 male, 25 females), median age 35.5 years, follow-up 24 months. Significant improvements (P ≤ .05) occurred postsurgery for TMJ pain, headache, jaw function, diet, and disability for both groups. Mean increase MIO for JIAG was 36.4 to 43.3 mm, for CG was 35.2 to 37.8 mm. Thirty-eight JIA patients (95%) improved in QOL, 2 had no change, and none were worse. CONCLUSIONS: This study suggests that patient-fitted TJP for TMJ reconstruction in conjunction with orthognathic surgery for the JIA patient provides long-term improvement relative to TMJ pain, headache, jaw function, diet, disability, MIO, and QOL.

Does Combined Temporomandibular Joint Reconstruction With Patient-Fitted Total Joint Prosthesis and Orthognathic Surgery Provide Stable Skeletal and Occlusal Outcomes in Juvenile Idiopathic Arthritis Patients?

PURPOSE: To determine skeletal and occlusal surgical changes and long-term stability outcomes for patients diagnosed with juvenile idiopathic arthritis (JIA) after TMJ reconstruction with TMJ Concepts patient-fitted total joint prostheses (TJP) and concomitant maxillary orthognathic surgery. MATERIAL/METHODS: A retrospective cohort study was conducted of all patients diagnosed with JIA, receiving TJP, and concomitant maxillary orthognathic surgery between 1991 and 2019, at Baylor University Medical Center treated by 1 surgeon. Patient evaluations presurgery (T1), immediate postsurgery (T2), and at longest follow-up (LFU) (T3) were analyzed using 20 cephalometric landmarks to compute 29 linear and angular measurements to determine surgical changes, long-term skeletal and occlusal stability, as well as oropharyngeal airway changes. Comparative data were tested for significance (α = 0.05) using paired and unpaired t tests. RESULTS: Forty-two patients met the JIA inclusion criteria, with a median age of 17.5 years and median postsurgical follow-up of 26 months. There were significant surgical changes (T1-T2) (P ≤ .05) for all parameters associated with mandibular linear and angular surgical changes, except for the horizontal position of posterior nasal spine and the vertical/horizontal position of gonion, indicating highly stable surgical outcomes. There were significant improvements in the oropharyngeal airway dimensions. CONCLUSIONS: This study suggests that TMJ Concepts patient-fitted TJP for TMJ reconstruction in conjunction with maxillary orthognathic surgery for counterclockwise rotation of the maxillo-mandibular complex for the JIA patients provides long-term skeletal and occlusal stability as well as dimensional improvement in the oropharyngeal airway.

Shoulder arthroplasty in patients with juvenile idiopathic arthritis: long-term outcomes.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease that occurs in the pediatric population. Often, JIA continues throughout life, leading to progressive polyarticular arthritis and significant joint destruction and disability, oftentimes requiring replacement surgery. This study aimed to determine the outcomes of primary shoulder arthroplasty (SA) in patients with JIA. METHODS: Over a 42-year time period (1977-2019), 67 primary SA (20 hemiarthroplasty [HA], 38 anatomic total shoulder arthroplasty [TSA], and 9 reverse shoulder arthroplasty [RSA]) with a prior diagnosis of JIA formally established in a multidisciplinary rheumatologic clinic met inclusion criteria. Further assessment was performed with inclusion of the visual analog scale pain score, active shoulder range of motion (ROM), imaging studies, complications, and implant survivorship free from reoperation and revision. RESULTS: SA led to substantial improvements in pain and ROM across the entire cohort at an average follow-up period of 12.2 years (range, 2-34 years). TSA was associated with the lowest pain scores (0.8; P = .02) and the highest American Shoulder and Elbow Surgeons Standardized Shoulder Assessment Form scores (77.4; P = .04) at the most recent follow-up when compared to HA and RSA. There were 14 (21%) complications across the cohort with rotator cuff failure (n = 4; 5.9%) as the most common complication followed by infection (n = 3; 4.5%). Revision surgery was performed in 5 shoulders (7.5%), with 5-year implant survival rates of 95.1% at 5 years, 93% at 10 years, 89.4% at 20 years, and 79.5% at 30 years. At 30 years, TSA was associated with better survival (90.1%) than HA (71.8%). CONCLUSIONS: Primary shoulder arthroplasty in the form of HA, TSA, and RSA offers a reliable surgical option for JIA patients with respect to pain reduction and ROM improvements. Unique challenges still exist in this cohort, in particular younger patients with an elevated propensity for glenoid bone erosion and a complication rate of 20.9%. As such, HA may not be ideal in this patient population. However, despite rotator cuff and glenoid concerns, TSA seems to be associated with better pain relief and patient-reported outcomes with the most durability in the long term when compared to HA.

Alloplastic Temporomandibular Joint Replacement in Patients With Systemic Inflammatory Arthritis and Connective Tissue Disorders.

PURPOSE: We present a retrospective study to report the outcomes of total temporomandibular joint (TMJ TJR) replacement with alloplastic devices in patients suffering from systemic inflammatory arthropathies. METHODS: A total of 39 patients with a diagnosis of rheumatoid arthritis (RA), ankylosing spondylitis (AS), psoriatic arthritis (PA), juvenile idiopathic rheumatoid arthritis (JIA), or systemic lupus, underwent alloplastic total joint replacement(s) (TJR) from 1999 to 2019. Maximal interincisal opening (in mm) was recorded before surgery (T0), at 1-year post-surgery (T1) and at last follow-up (T2). A visual analog scale (from 0 to 10) was used for subjective examination of pain before surgery (T0) and at last follow-up (T2). Comparisons were conducted with statistical significance set at P < .05. RESULTS: Seventy-four joints were replaced in 39 patients. Thirty-two were female. The mean age was 36 years old (range 18-61) and the mean follow-up was 45.9 months (SD 49.4). The most common diagnosis was RA (n = 21), followed by JIA (n = 5) and AS (n = 5), PA (n = 4), lupus (n = 3), and mixed connective tissue disorder (n = 1). The mean pain score had fallen from 6.8 (SD 3.2) at T0 to 1.3 (SD 2.4) (P < .001) at T2. The maximal interincisal opening had improved from a mean of 22.1 mm (SD 13.3) at T0 to 34.3 mm (SD 8.5) (P < .001) at T2. One patient got persistent dysesthesia in the V3 distribution. There were no serious late complications. CONCLUSION: Patients suffering from systemic inflammatory arthropathies involving the TMJs can be successfully treated by TJR with alloplastic devices. The long-term reduction of TMJ symptoms and functional improvement in this initial study suggest good predictability for this treatment.

Uncharted waters: mesenchymal stem cell treatment for pediatric refractory rheumatic diseases; a single center case series.

BACKGROUND: With the advent of innovative therapies including biologics and Janus kinase inhibitors, children with rheumatic diseases are more likely to have improved outcomes. Despite these advances, some children do not respond, or they, or their parents fear adverse events and seek other alternatives. Increasingly, private companies are offering mesenchymal stem cells (MSC) as an alternative, which are described as natural therapies for rheumatic diseases, often insinuating them as a cure. MSC have immunomodulatory properties, and transplantation of these stem cells have been used to successfully treat immunologic conditions like graft-versus-host disease. Lately, MSC research in adult lupus has been encouraging, but the clinical trials are still underway and in most, MSC therapy is not a standalone treatment. This retrospective case series will highlight three cases of pediatric refractory autoimmune disease whose parents sought out and received MSC therapy as a self-decision without first seeking medical advice from our specialty. The three families felt that their children were improved and in two believed that their child was cured. MSC have the potential of beneficial immunomodulation and may be a powerful tool in the therapy of rheumatic disease, but well controlled clinical trials are necessary and should be designed and monitored by experts in childhood rheumatic disease. CASE PRESENTATION: Three children with three different rheumatic diseases; systemic lupus erythematosus, mixed connective tissue disease and juvenile idiopathic arthritis were under the care of pediatric rheumatology at a large, tertiary-care, teaching institution. Multiple non-biologic and biologic disease-modifying anti-rheumatic drugs failed to significantly decrease disease activity, and as a result, the families chose to undergo MSC therapy. After transplantation, all children improved per patient and parent report and tapered off conventional immunosuppressive drugs. No serious adverse events occurred in these three patients. CONCLUSION: The three cases presented in this report reflect comparable beneficial outcomes and minimal risks published in adult studies. These were not controlled studies, however, and benefit was reported rather than documented. These cases suggest that MSC transplantation may prove a promising adjunctive treatment option; however, further research, development of standardized infusion therapy protocols, and well-designed monitored clinical trials are essential.

Sustained remission after haploidentical bone marrow transplantation in a child with refractory systemic juvenile idiopathic arthritis.

BACKGROUND: Some patients with systemic juvenile idiopathic arthritis (SJIA) and severe, refractory disease achieved remission through intensive immunosuppressive treatment followed by autologous hematopoietic stem cell transplantation (HSCT). However, disease relapsed in most cases. More recently selected SJIA patients received allogenic HSCT from a HLA-identical sibling or a HLA matched unrelated donor. While most transplanted patients achieved sustained SJIA remission off-treatment, the procedure-related morbidity was high. CASE REPORT: A girl presented SJIA with a severe disease course since the age of 15 months. She was refractory to the combination of methotrexate and steroids to anti-interleukin (IL)-1, then anti-IL-6, tumor necrosis factor alpha inhibitors, and thalidomide. Given the high disease burden and important treatment-related toxicity the indication for a haploidentical HSCT from her mother was validated, as no HLA matched donor was available. The patient received a T replete bone marrow graft at the age of 3.7 years. Conditioning regimen contained Rituximab, Alemtuzumab, Busulfan, and Fludarabine. Cyclophosphamide at D + 3 and + 4 post HSCT was used for graft-versus-host-disease prophylaxis, followed by Cyclosporin A and Mycophenolate Mofetil. Post HSCT complications included severe infections, grade 3 intestinal graft-versus-host-disease, autoimmune thyroiditis, and immune thrombocytopenia. Three years after HSCT, the child is alive and well, notwithstanding persistent hypothyroidy requiring substitution. Immune thrombocytopenia had resolved. Most importantly, SJIA was in complete remission, off immunosuppressive drugs. CONCLUSION: Allogenic HSCT may be a therapeutic option, even with a HLA haplo-identical alternative donor, in patients with inflammatory diseases such as SJIA. Despite increased experience with this treatment, the risk of life-threatening complications restrains its indication to selected patients with severe, refractory disease.

Role of alloplastic reconstruction of the temporomandibular joint in the juvenile idiopathic arthritis population.

We present outcomes following total joint replacement of the temporomandibular joint (TMJ) in adolescent and young adult patients with juvenile idiopathic arthritis (JIA), and discuss a multidisciplinary treatment model. A retrospective review was performed of patients presenting to the University of North Carolina Oral and Maxillofacial Surgery Service (Chapel Hill, NC) from 2016- 2018 who underwent unilateral or bilateral total replacement of the TMJ for a diagnosis of end-stage joint disease secondary to JIA. Inclusion criteria included diagnosis by a rheumatologist, presentation to our department in adolescence (under 18 years of age), surgical intervention in adolescence or young adulthood (under 25 years of age), and documentation of preoperative and postoperative pain, maximum incisal opening (MIO), and quality of life measures. A database was created and data were then analysed both qualitatively and quantitatively. Five patients met the inclusion criteria. All achieved MIO of more than 35mm with a mean improvement of 24mm, and were able to tolerate a regular diet. All preoperative pain had essentially been eliminated. All patients reported a considerable improvement in quality of life. To our knowledge, this is the first report to document a series of paediatric and young adult patients with JIA who required total replacement of the joint for end-stage joint disease. To our knowledge, it is also the first to describe the use of a collaborative clinic of oral and maxillofacial surgeons, neuroradiologists, dental radiologists, orofacial pain specialists, paediatric rheumatologists, and paediatric nurse practitioners, to care for these patients.

Early Joint Replacement in Juvenile Idiopathic Arthritis: Trend Over Time and Factors Influencing Implant Survival.

OBJECTIVE: To describe early prosthesis implantations in a cohort of patients with juvenile idiopathic arthritis (JIA) followed in a tertiary referral hospital and to analyze possible factors influencing implant survival. METHODS: This was a retrospective cohort study. Charts of all patients with JIA who underwent total joint replacement at Gaetano Pini Hospital, Milan, Italy from January 1992 to June 2019 were retrieved, and relevant data were analyzed. RESULTS: Eighty-five patients met the inclusion criteria for this study, with a median follow-up period of 17.2 years. The median age at first prosthesis was 22.7 years. The total number of replaced joints was 198 over a period of 27 years. The hip was the most frequently replaced joint, accounting for almost two-thirds of the total number of implants; the other one-third refers mostly to knee implants. Polyarticular JIA and systemic JIA were the most represented JIA categories in the study cohort. A significant upward trend of the age at arthroplasty and of disease duration before arthroplasty over decades was found. The rates of implant survival at 5, 10, and 15 years were comparable (from 84% to 89%); 50% of implants lasted ≥20 years. CONCLUSION: We reported retrospective data on early joint replacement in a cohort of patients with JIA. We observed a progressive and significant upward trend of both age at arthroplasty and disease duration before the first arthroplasty over time. The JIA category, year of implant, and presence of complications significantly affected implant survivorship.

Alloplastic Temporomandibular Joint Reconstruction for Patients With Juvenile Idiopathic Arthritis.

PURPOSE: The purpose of this study was to assess the efficacy of alloplastic temporomandibular joint (TMJ) total joint replacement (TJR) in patients with juvenile idiopathic arthritis (JIA). MATERIALS AND METHODS: All patients with a formal diagnosis of JIA requiring a TMJ patient-specific TJR between 2010 and 2018 at The University of Texas-Health at San Antonio were retrospectively analyzed. To be included, patients must have had a formal diagnosis of JIA, complete records, and TMJ reconstruction with the TMJ Concepts patient-specific total joint prosthesis (TMJ Concepts, Ventura, CA). Clinical data acquisition was required at a minimum of 12 months after surgery (longest follow-up [LFU]). Subjective and objective analyses were performed using a 10-point visual analog scale at the preoperative and LFU time points. Surgical data at the perioperative and LFU time points were recorded for comparison. RESULTS: Twenty patients with JIA met the inclusion criteria. The mean visual analog scale measurements for facial pain, TMJ pain, jaw function, diet, and disability were all significantly reduced at LFU. The maximal interincisal opening with pain was increased from 33.5 mm preoperatively to 44 mm at LFU, and the mean maximal interincisal opening without pain was increased from 31.1 mm preoperatively to 43 mm at LFU. None of the patients had complications from their TMJ TJR. CONCLUSIONS: Alloplastic TMJ reconstruction is a safe and efficacious treatment option for the surgical management of end-stage TMJ disease in JIA patients.

Shoulder arthroplasty for juvenile idiopathic arthritis.

There is limited literature to guide shoulder surgeons in the management of juvenile idiopathic arthritis (JIA). We aim to help clinicians to formulate an approach to the surgical management of the condition through a review of the available literature on arthroplasty in JIA, general considerations when operating on patients with inflammatory arthropathy and recommendations based on the authors' experience. Four articles report formal data on arthroplasty in JIA with favourable improvements in post-operative pain and function scores after the long-term follow-up. Significant heterogeneity in treatment and a lack of standardisation in quantitative outcomes highlights the need for further larger scale and higher quality research. The aim of this study is to review the evidence and provide information on preoperative evaluation of surgical candidates, operative techniques, choice of implant design and to evaluate functional outcomes in patients who undergo shoulder arthroplasty.