Flemish network on rare connective tissue diseases (CTD): patient pathways in systemic sclerosis. First steps taken.

Despite 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.

Traumatic lesions at the thumb base: Treatment options.

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.

Spontaneous index finger extensor tendon rupture in a SNAC wrist.

We present two recent cases of spontaneous rupture of both index finger extensor digitorum communis and extensor indices proprius tendons caused by a dorsal carpus osteophyte. Both patients had a history of scaphoid fracture non-union with evolution to scaphoid non-union advanced collapse (SNAC) of the wrist. These two cases were treated surgically with a 3-corner arthrodesis, and an interposition of a fragment of one of both ruptured tendons together with a tendon transfer of a supernumerary extensor digitorum communis of the third finger. The interposed tendon fragment was sutured with a Pulvertaft weave proximally and end-to-end distally. The supernumerary tendon was added as reinforcement to this construction. At 6-month and 14-month follow-up, the patients had a slight decrease in range of motion and functionality without hindering the activities of daily living and a great improvement in strength. Isolated extension of the index finger was possible minimum 6 months postoperatively. Spontaneous tendon ruptures of the finger extensors are not common but were described earlier in literature. Recent literature described that underlying cause of a spontaneous tendon rupture should always be corrected in order to prevent or at least delay future ruptures. To our knowledge, this is a rare type of complication and this kind of treatment has never been reported in literature.

Scaphotrapeziotrapezoid osteoarthritis: From the joint to the patient.

Scaphotrapeziotrapezoid osteoarthritis (STT OA) is common and often associated with thumb basal joint arthritis. Pain at the base of the thumb on the volar aspect and during resisted extension is characteristic of symptomatic STT OA. If conservative treatment fails, surgical treatment may be offered. In case of STT OA, treatment may range from arthrodesis to trapeziectomy (isolated or associated with ligament reconstruction and/or interposition). Any preoperative intracarpal instability (DISI) can be exacerbated by resecting more than 3 or 4 mm of the distal pole of scaphoid. For peritrapezial osteoarthritis, trapeziectomy is the logical solution, but it exposes the patient to known complications: loss of strength, long recovery, trapeziometacarpal impingement. Initial treatment of thumb basal joint arthritis by arthroplasty is also an option. Treatment of both sites is also possible by interposition of pyrocarbon implants. In all cases (isolated or associated STT OA) and no matter the technique chosen, maintaining the scaphoid height (arthrodesis, resection < 3 mm and/or associated interposition) and performing oblique trapezoidal osteotomy (to prevent scaphoid-metacarpal impingement) are the two crucial elements of surgical treatment.

Osteotomies around the thumb base in CMC 1 arthritis.

The trapeziometacarpal joint (TMC) is a saddle joint that is subjected to tremendous repetitive loads through our lifetime. This joint is apparently congruent, but only a small part of the articular surface is loaded during pinch grips. This design implies a perfect bony anatomy, high quality articular cartilage and no ligament laxity. Under certain circumstances, where these different anatomical variables are imperfect, symptoms and pain can start at a very early stage in life. They are mainly acquired, but can be posttraumatic in origin. High quality radiographic views are needed: these radiographs must be done methodically by well-trained radiologists. The symptoms and radiographic changes may not match, such as when radiographic changes are minimal but functional impairment is significant. The primary goal of treatment is conservative. This cannot be stressed enough since conservative treatment can be successful with good follow-up by the hand surgeon: resting splint, good postures at work and if necessary, anti-inflammatory drugs and paracetamol. If this fails after a minimum of 6 months, different osteotomies can be proposed, combined with ligament augmentation in some cases. These osteotomies are mainly extra-articular, can be at the level of the base of the first metacarpal and the trapezium, or can be solely at the base of the first metacarpal. Isolated osteotomies of the trapezium should be avoided since they tend to close the first web space. In certain posttraumatic cases, intra-articular osteotomy of the malunion can be done to restore congruency and provide pain relief.

Pronator quadratus repair after volar plate fixation in distal radial fractures: evaluation of the clinical and functional outcome and of the protective role on the flexor tendons-a randomized controlled study.

The objective was to evaluate pronator quadratus (PQ) repair following volar plating of distal radial fractures on clinical and functional outcome, as well as flexor tendon friction, during the first 12 months in ideally positioned plates (Soong 0). Confounding factors like variation in plate positioning, anatomical consolidation of the fracture and associated lesions were excluded. Eighty patients were included with distal radius fractures treated by volar locking plate in Soong grade 0 position, who were randomized to group PQ repair and group no repair. Sixty-five patients completed the 1-year follow-up: group PQ repair (n = 35) and group no repair (n = 30). Primary outcomes included range of motion, grip strength, pain level and QuickDASH. Secondary outcomes included distance between flexor pollicis longus (FPL) and volar rim and the largest distance between FPL and plate (soft tissue thickness occupied by PQ), assessed by ultrasonography. Mobility, grip strength and QuickDASH revealed no significant differences, except extension was significantly higher only in first 3 months and radial deviation was significantly higher only at 6 weeks after PQ repair. Pain level was significantly lower after PQ repair only in the first 3 months. Distance between FPL and volar rim and the largest distance between FPL and plate were significantly higher after PQ repair. No friction contact between FPL and volar rim was measured in both groups at all measurement moments. Consequently, protective flexor tendon effect of PQ repair could consequently not be concluded. In conclusion, clinical and functional short term benefits, except improved wrist extension and reduced pain in the first 3 months, were not proven in this study. In Soong grade 0, PQ repair is probably not necessary to prevent flexor tendon pathology. In Soong grade 1 or 2, this is still to be investigated. LEVEL OF EVIDENCE: 2.

Radiological measurement of trapezial dysplasia - variation of trapezial tilt and trapezial inclination.

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.

Scaphometacarpal arthrodesis with autologous structural bone graft as a salvage procedure after failed trapeziectomy - Surgical technique and initial results.

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.

Treatment of Dupuytren's disease using one full dose of Clostridiumhistolyticum collagenase at a lower concentration: Retrospective analysis of clinical outcomes.

Clostridiumhistolyticum collagenase (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. LEVEL OF EVIDENCE: III.

Ultrasonographic evaluation of the distance between the flexor pollicis longus tendon and volar prominence of the plate as a function of volar plate positioning and pronator quadratus repair - A cadaver study.

Flexor tendon injury after volar plating of distal radius fractures is due to friction against the plate. To assess this risk, the distance between the flexor pollicis longus (FPL) and the volar prominence of the plate was measured with ultrasonography under various conditions: a standard plate fixed proximal or distal to the watershed line and a low-profile volar rim plate, with and without transection of the pronator quadratus (PQ). Distance from the FPL to the volar prominence of the plate decreased significantly when the PQ was cut and when a standard plate was placed distal to the watershed line, with the tendon often bulging over the plate. No statistical difference was measured between a volar rim plate and a standard plate distal to the watershed line. Our results confirm the importance of positioning the volar plate proximal to the watershed line and of repairing the PQ.

Evaluation of current treatment techniques for distal radius fractures amongst Belgian orthopaedic surgeons.

This online questionnaire was initiated to investigate if a consensus on the treatment of distal radius fractures amongst orthopaedic surgeons in Belgium exist. Two cases were presented: an extra-articular fracture, with dorsal displacement (Frykman type I) and a displaced intra-articular fracture (Frykman type VII). Treatment of choice and rationale of choice were investigated. 158 responses were collected. In case of a Frykman type I, the majority of surgeons would have performed a closed reduction and intrafocal K-wiring (37.3%) or volar plating (34.8%). In case of a Frykman type VII, volar plating was the primary treatment of choice (66.5%), closed reduction and K-wiring was chosen in 24.7% and external fixation in 4.4%. We cannot conclude on a consensus in treatment choice amongst Belgian orthopaedic surgeons. Compared to previous research, an increase in the use of volar plating in intra- as well as in extra-articular distal radius fractures was noticed.

Surgical treatment after a failed trapeziectomy: A case report.

Several treatment options exist to surgically treat end-stage trapeziometacarpal osteoarthritis. Trapeziectomy is recognized as leading to a mostly acceptable functional outcome. Although rarely encountered, persisting failure of the trapeziectomy is difficult to address. We present a case where several procedures were subsequently performed to treat a failed trapeziectomy condition. First, a trapeziometacarpal prosthesis (Arpe(®), Biomet(®)) was inserted with the two components reversed: the cup in the first metacarpal and the stem in the scaphoid. This prevented normal mobility and resulted in a non-functional, chronically painful joint. Revision arthroplasty was performed with a dual-mobility component (Ebony(®), Stryker(®)), replacing the trapezium to articulate with the first metacarpal and the scaphoid. This restored the first column's length but led to prosthesis instability only 4 months after implantation. Finally, first column arthrodesis with iliac bone graft interposed between the first metacarpal and scaphoid achieved an acceptable outcome.

The wrist hyperflexion and abduction of the thumb (WHAT) test: a more specific and sensitive test to diagnose de Quervain tenosynovitis than the Eichhoff's Test.

De Quervain's disease has different clinical features. Different tests have been described in the past, the most popular test being the Eichhoff's test, often wrongly named as the Finkelstein's test. Over the years, a misinterpretation has occurred between these two tests, the latter being confused with the first. To compare the Eichhoff's test with a new test, the wrist hyperflexion and abduction of the thumb test, we set up a prospective study over a period of three years for a cohort of 100 patients (88 women, 12 men) presenting spontaneous pain over the radial side of the styloid of the radius (de Quervain tendinopathy). The purpose of the study was to compare the accuracy of the Eichhoff's test and wrist hyperflexion and abduction of the thumb test to diagnose correctly de Quervain's disease by comparing clinical findings using those tests with the results on ultrasound. The wrist hyperflexion and abduction of the thumb test revealed greater sensitivity (0.99) and an improved specificity (0.29) together with a slightly better positive predictive value (0.95) and an improved negative predictive value (0.67). Moreover, the study showed us that the wrist hyperflexion and abduction of the thumb test is very valuable in diagnosing dynamic instability after successful decompression of the first extensor compartment. Our results support that the wrist hyperflexion and abduction of the thumb test is a more precise tool for the diagnosis of de Quervain's disease than the Eichhoff's test and thus could be adopted to guide clinical diagnosis in the early stages of de Quervain's tendinopathy.

Treatment of a symptomatic trapezial dysplasia with metacarpal instability following thumb metacarpal lengthening with an addition-subtraction osteotomy.

The most important problem of trapezial dysplasia with thumb metacarpal instability is of bony origin. Together with the progressive capsuloligamentous decompensation it evolves in a progressive adduction deformity of the thumb metacarpal secondary to the dysplasia of the trapezium with its increased articular slope. The addition-subtraction osteotomy restores the anatomy combining two techniques: an abduction-extension osteotomy of the first metacarpal to correct the axis of the first metacarpal and an opening wedge osteotomy of the trapezium to reorientate the trapezial saddle. We present a case of an addition-subtraction osteotomy in a case of symptomatic trapezial dysplasia with metacarpal instability following a thumb metacarpal lengthening in a severely mutilated hand. This technique was especially effective in reducing the instability and pain but mainly in maintaining mobile the only remaining joint of the thumb.