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ABSTRACTDespite the low prevalence of each rare disease, the total burden is high. Patients with rare diseases encounter numerous barriers, including delayed diagnosis and limited access to high-quality treatments. In order to tackle these challenges, the European Commission launched the European Reference Networks (ERNs), cross-border networks of healthcare providers and patients representatives. In parallel, the aims and structure of these ERNs were translated at the federal and regional levels, resulting in the creation of the Flemish Network of Rare Diseases. In line with the mission of the ERNs and to ensure equal access to care, we describe as first patient pathways for systemic sclerosis (SSc), as a pilot model for other rare connective and musculoskeletal diseases. Consensus was reached on following key messages: 1. Patients with SSc should have multidisciplinary clinical and investigational evaluations in a tertiary reference expert centre at baseline, and subsequently every three to 5 years. Intermediately, a yearly clinical evaluation should be provided in the reference centre, whilst SSc technical evaluations are permissionably executed in a centre that follows SSc-specific clinical practice guidelines. In between, monitoring can take place in secondary care units, under the condition that qualitative examinations and care including interactive multidisciplinary consultations can be provided. 2. Patients with early diffuse cutaneous SSc, (progressive) interstitial lung disease and/or pulmonary arterial hypertension should undergo regular evaluations in specialised tertiary care reference institutions. 3. Monitoring of patients with progressive interstitial lung disease and/or pulmonary (arterial) hypertension will be done in agreement with experts of ERN LUNG.

Management of recurrent Dupuytren’s disease of the little finger is challenging. Various treatment modalities have been proposed: external fixation, local skin flap, dermofasciectomy, or even amputation. An alternative surgical technique was introduced by Honecker et al. in 2016 and refined by Raimbeau et al. in 2019, consisting in resection of the middle phalanx and shortening arthrodesis. We modified the technique by combining arthrodesis with a limited fasciectomy of the abductor and/or pretendinous cord in the fifth ray to improve cosmetic and functional outcomes.

Emerging advances in immersive virtual reality incorporating optical hand-tracking present promising potential for application in orthopedic hand therapy. The system is designed to analyze hand movements, enabling users to “use” their hands virtually in any fabricated setting. This article, supplemented with videos, examines practical applications of immersive virtual reality in routine hand therapy and provides a scientific presentation of the interaction of immersive virtual reality with our physiological and neurological systems. Indications for immersive virtual reality use, critical evaluations and recommendations are comprehensively discussed. Immersive virtual reality has the potential to evolve into a standard treatment modality in orthopedic hand therapy.

Traumatic lesions around the base of the thumb have special features due to the location and structure of the joint and its inherent potential instability. This causes different fracture patterns, which are mostly isolated around the metacarpal base but can also involve just the trapezium or both. Exceptionally, there may be isolated dislocation. Fracture patterns are variable and influence the type of surgery. The most common fracture is Bennett’s fracture accounting for 4% of all hand fractures and sometimes associated with trapezium fracture, usually in male subjects. Different fracture mechanisms have been proposed. Apart from intra-articular fractures of the metacarpal base and the trapezium, proximal metaphyseal fractures can exceptionally be treated conservatively by immobilization. All other fractures require open or closed reduction combined with different types of temporary pinning or open reduction and internal fixation with screws or locking plate in case of comminution. Immobilization depends on the type of surgical treatment and can be removable or non-removable. Close follow-up is mandatory to avoid the inconveniences of secondary swelling with non-removable plaster and resin casts. Extra-articular malunion may be tolerated, but articular malunion must be corrected surgically by intra-articular osteotomy to restore the joint. In case of posttraumatic joint degeneration, treatment will focus on a case-by-case basis on the patient’s complaints. Arthrodesis or prosthetic surgery can be proposed in case of severe problems caused by osteoarthritis.

Clostridiumhistolyticumcollagenase (CHC) is rapidly becoming a mainstream treatment option for Dupuytren's disease for hand surgeons. We performed a retrospective study of the efficacy of this substance at a lower concentration, but a higher total dose than recommended by the manufacturer. Thirty-nine patients with 43 affected hands were treated and analyzed at a mean follow-up of 27 months. Subgroup analysis was done for patients who received treatment in one or two joints (group A), and for patients who received treatment in more than two joints (group B). We found that our CHC solution is safe and effective when simultaneously treating as many as four joints affected by Dupuytren's disease. Patients in group B had a lower (but non-significant) treatment failure rate compared to patients in group A. Also, higher satisfaction rates were observed in group B, again non-significant. No major complications were observed in any group. CHC use is associated with only minor complications. The results are consistent even when up to four joints are treated with one dose.

Epstein-Barr virus-associated smooth pulmonary tumor is a rare condition that mostly affects immunosuppressed patients. This case describes a young boy with a history of kidney transplantation who presented recurrent pneumonia. Multiple endobronchial soft tissue tumors affecting both right and left bronchial tree were found and partially removed by bronchoscopy. Immunohistologic analysis demonstrated Epstein-Barr virus-associated smooth pulmonary tumor. Immunosuppressive therapy was changed from tacrolimus to sirolimus. A few months later, new right upper lobe and inferior left lobe tumors were found. Recurrent left lower lobe pneumonia prompted lobectomy. In the present case, complete resection and change of immunosuppressive treatment were effective.

A case-control study was conducted to compare wound healing with or without skin suture after endoscopic carpal tunnel release. The primary endpoint was esthetics on the Manchester Scar Scale (MSS). Patients were recruited between April 2016 and April 2017. Inclusion criteria comprised carpal tunnel syndrome with clinical and electromyographic confirmation on the Canterbury NCS (nerve conduction studies) Severity Scale. Age, gender, occupation, handedness, smoking status, and operated side were noted. In the first group (64 patients) the skin was not sutured. In the second group (44 patients) the skin was closed Ethilon™ 5-0 intradermal running suture. All 108 patients had 3 months’ follow-up. Since data were not normally distributed, analysis was performed with the Mann–Whitney U test (MWU) for independent samples. There were no significant differences in age (p = 0,416), gender (p = 0.670) or occupation (p = 0.725) between groups. MSS score did not significantly differ between groups (MWU test; p = 0.529): sutureless, 6.64 ± 1.21; sutured, 6.45 + 1.09. Thus, sutureless wound closure can be a useful option in treating carpal tunnel syndrome by a single-portal endoscopic technique. That the wound remains open enables easy evacuation of any hematoma, avoiding pain due increased intra-tissular pressure.

This report emphasizes careful consideration of surgical technique for intramedullary screw fixation in middle phalanx fractures. Highlighting pitfalls, particularly with K-wire placement, it suggests the antegrade trans-articular approach as superior, urging further research for improved patient outcomes.

Since thumb base arthritis is an increasing problem in an active population, indications for prosthetic replacement may increase.

L’utilisation des systèmes de libération de canal carpien est très répandue en France. Plusieurs systèmes ont été mis sur le marché depuis qui John Agee et James Chow en 1989. Ces systèmes se composent d’éléments réutilisables (souvent divers) et d’éléments (souvent un) à usage unique. Ces derniers ne sont en principe pas réutilisables et doivent être détruit après utilisation.

Trapeziometacarpal instability is a well-known condition that could lead to trapeziometacarpal osteoarthritis. With the focus in the literature being on ligament insufficiency, bony deformity has received much less attention. Although trapezial tilt is currently used to measure trapezial dysplasia, we believe trapezial inclination is a more reliable measurement. The purpose of this study is to compare these two methods for evaluating trapezial dysplasia and to define the most reproducible measurement. Fifty patients were identified who had three consecutive radiographs of the trapeziometacarpal joint. Eaton views with little to no signs of osteoarthritis (Eaton stage I or II) were used. Both trapezial tilt and trapezial inclination were measured by two independent observers on three radiographs for every patient. Intra-observer variation, absolute agreement and consistency, and intra-observer variation and consistency were assessed for both measurements. Mean trapezial tilt was 135° and mean trapezial inclination was 10°. For both observers, intra-observer variation of trapezial inclination was significantly less than for trapezial tilt. Our study shows that mean trapezial inclination in a middle-aged adult population is approximately 10°. This measurement is more reproducible than the more frequently used trapezial tilt and may be a better parameter to define trapezial dysplasia and to guide its treatment.

Painful instability due to proximal migration of the first metacarpal remains a challenging problem in patients who have undergone multiple surgeries for trapeziometacarpal osteoarthritis. The objective of this study was to investigate whether scaphometacarpal (SMC) arthrodesis with a structural bone graft is an acceptable and definitive salvage procedure for the multioperated patient. Three patients underwent SMC arthrodesis using a structural iliac crest bone graft. All patients had symptomatic instability of the thumb base and had undergone three to four surgeries prior to the arthrodesis. All patients were satisfied with the outcomes. Mean opposition decreased from 8 to 7 and mean retropulsion remained at 1. Mean grip strength increased from 3.5 to 10.5kg and mean precision pinch strength increased from 1.5 to 2.5kg. Fusion was confirmed in all patients. SMC arthrodesis with a structural bone graft is an acceptable procedure that largely preserves thumb opposition and restores stability with increased grip and pinch strength. Although far from ideal, it can be considered as a definitive salvage procedure in patients who have already had multiple surgeries to the trapeziometacarpal joint.

Ulnar styloid fractures occur frequently concomitant with distal radial fractures. Although unstable distal radial fractures are mostly surgically treated, ulnar styloid fractures are often ignored. Unstable fractures at the base of the ulnar styloid may lead to persistent ulnar pain, due to distal radioulnar joint instability or ulnar styloid non-union.