Assessing hand grasp in patients with systemic sclerosis using the 16-grasp test: Preliminary results from a multidisciplinary study group.

BACKGROUND: Reports on hand dysfunction and rehabilitation in SSc are quite scarce in the literature and mainly focus on functional assessment tools, such as the Duruoz Hand Index and the HAMIS test for evaluating hand mobility by simulating specific grasps with nine different objects. PURPOSE OF THE STUDY: This study aimed to provide an adequate assessment methodology for hand grasp dysfunctions in patients suffering from systemic sclerosis (SSc) through the 16-grasp test. STUDY DESIGN: Case-control study. METHODS: Ninety-seven consecutive SSc patients were recruited at our Scleroderma Unit, where a 16-grasp test was performed by all patients and supervised by an experienced hand therapist. Sixteen different patterns of grasp have been divided into power grasps and precision pinch and two more modalities: static and dynamic prehension evaluation on scale from 0 to 4. We also compared previous evaluations on 19 of patients recruited. RESULTS: The majority of SSc patients (84 females and 13 males; mean age 56.0±12.0 years; mean disease duration 8.0±6.0 years) displayed grasp dysfunctions; in particular 48% and 54% reported slight difficulty in the right and left grasps respectively, 6% medium difficulty in both hands, and only 3% and 1% experienced severe difficulty respectively, while 31.5% had no issues in either hand. Our results showed that the limited cutaneous subset (lcSSc) scored a lower deficit for either grasp compared to diffuse form (dcSSc). No statistically significant differences in total grasp deficit had been noticed when comparing patients having a disease duration < 5 years or longer. In the retrospective study on 19 of these patients, 8 out of 10 lcSSc patients showed no significant changes, while in 2 out of 10, slight improvements were observed in both hands. However, in the dcSSc group, 4 out of 9 worsened bilaterally while the grasp scores for 5 of them remained unchanged. CONCLUSION: Our study reported hand involvement in both lcSSc and dcSSc forms, more significantly in dcSSc patients. This test is intended to be a more objective means of assessing grasp alterations linked to scleroderma hand deformities. Furthermore, thanks to its intuitiveness, the test may be useful for engineers designing personalized ergonomic assistive devices.

Modified dome osteotomy and anterior locking plate fixation for distal radius variant of Madelung deformity: a retrospective study.

PURPOSE: Dome osteotomy of the distal radius with Vickers ligament release is an established method of treatment for Madelung deformity. Many different surgical procedures have been proposed in literature but techniques, patient inclusion and efficacy evaluations are heterogeneous. MATERIALS AND METHODS: A retrospective review of children affected by 'distal radius' Madelung deformity and treated with a standardized surgical procedure (modified reverse dome osteotomy of the distal radius and volar fixation with a small locking plate) between 2010 and 2018 at a single center was performed. The technique used in this study, reversing the shape of the classical dome osteotomy, allowed for an improved three-planar correction of the distal radial epiphysis and volar plate fixation allowed for an increased stability and reduced soft tissue morbidity. A structured follow-up including a prompt post-operative rehabilitation program (without wrist immobilization) was established. Pain relief, functional outcome and cosmetic appearance were assessed with a structured clinical assessment, DASH Score and radiographic assessment, accordingly. RESULTS: A total of 15 wrists in 13 children (12 females, mean age of 15.6 years, range 11-19) were included. The mean follow-up time was 3.8 years. Bone union and pain relief were obtained in all cases. Improvement in the range of motion was detected in extension, supination and flexion of the wrist. Radial inclination was increased by 15.3° and lunate subsidence by 4.1 mm. CONCLUSIONS: The use of volar fixation with a small locking plate and immediate post-operative rehabilitation for reverse dome osteotomy of the distal radius in pediatric patients affected by 'distal radius' Madelung's deformity is stable and effective.

Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence).

BACKGROUND: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. MAIN BODY: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. CONCLUSION: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.

Genetic basis of congenital upper limb anomalies: analysis of 487 cases of a specialized clinic.

BACKGROUND: Specific data regarding the frequencies of the congenital upper limb anomalies (CULA) according to their etiology are hardly available due to the heterogeneity across classification systems. In this study, we aim at defining the CULA etiology of patients that have been evaluated at the Modena University Hospital's Congenital Hand Malformations multidisciplinary clinic in the years 2004 to 2012. METHODS: Medical records of 487 patients were retrospectively reviewed. On the basis of clinical, anamnestic, and genetic data, the CULA were distributed into two main groups: (1) non-Mendelian etiology, including prenatal exposure, somatic mutations and amniotic bands; and (2) Mendelian etiology, including single gene and genomic/chromosomal diseases. CULA were further grouped according to the embryological damage (formation, separation and growth defects) and to the involved axis (radial, ulnar, central). RESULTS: A Mendelian etiology was diagnosed in 199 patients (40.9%), whereas the remaining 288 cases (59.1%) were described as non-Mendelian. The involvement of the lower limbs, the presence of malformations in other organs and facial dysmorphisms were significantly more represented in the Mendelian cases. The formation defects were significantly more frequent in the non-Mendelian group (p < 0.001), whereas the frequency of separation defects was higher in the Mendelian cases (p = 0.0025). Patients with non-Mendelian etiologies showed a significantly higher frequency of central defects (p = 0.0031). CONCLUSION: The two etiologies differ in terms of patient's clinical features, morphology defect and axis involvement. This data may be helpful to the clinician during the patient's diagnostic workup by indicating the necessity for genetic testing and for determining the anomaly's recurrence risk.

Effectiveness of cast immobilization in comparison to the gold-standard self-removal orthotic intervention for closed mallet fingers: a randomized clinical trial.

STUDY DESIGN: Randomized clinical trial. INTRODUCTION: Although orthotic immobilization has become the preferable treatment choice for closed mallet injuries, it is unclear whether orthosis self-removal has an impact on the final outcome. PURPOSE: To evaluate the treatment efficacy of cast immobilization of closed mallet fingers using Quickcast(®) (QC) compared to a removable, lever-type thermoplastic orthosis (LTTP). METHODS: 57 subjects were randomized in 2 groups. DIPj extensor lag and the Gaberman success scale were used as primary outcomes. RESULTS: LTTP subjects resulted in greater extensor lag than QC subjects (x = 5°; p = 0.05) at 12 weeks from baseline, and high edema and older age negatively affected DIPj extensor lag. No other differences were found between groups. CONCLUSION: Cast immobilization seems to be slightly more effective than the traditional approach probably for its greater capacity to reduce edema. LEVEL OF EVIDENCE: 1B.

Open experience with a new myorelaxant agent for low back pain.

INTRODUCTION: Eperisone hydrochloride has been recently proposed as a muscle relaxant for the treatment of muscle contracture and chronic low back pain (LBP) as it is devoid of clinically relevant sedative effects on the central nervous system (CNS). We tested this hypothesis by performing a study of patients with LBP and muscle contracture who were treated with full-dose eperisone. METHODS: Patients with moderate to severe, acute, or relapsing LBP received eperisone 100 mg three times daily for 10 consecutive days. Assessments included: spontaneous pain, pain on movement, resistance to passive movement, antalgic rigidity, and tolerability. RESULTS: In total, 100 patients were enrolled into the study. The treatment achieved a consistent analgesic and muscle relaxant activity across all patients. Both spontaneous pain and pain on movement were significantly decreased, as was resistance encountered by the investigator to passive movements, antalgic rigidity, and muscle contracture. As a consequence, treatment with eperisone resulted in a lower rigidity of the lower back and an improved motility for patients. Only seven adverse reactions were reported, including light-headedness (1), occasional vertigo and/or loss of equilibrium (3), mild somnolence (2), and epigastric pain (1). In almost all cases, there was no need to interrupt the treatment and the adverse reaction resolved spontaneously. CONCLUSIONS: Eperisone had an analgesic and muscle relaxant effect in patients with LBP. It should be noted that while it is common practice in rheumatology to combine a pain killer with a muscle relaxant in order to achieve a satisfactory result on both symptoms, the present results with eperisone were achieved with a single drug. With an improved tolerability profile compared with nonsteroidal anti-inflammatory drugs, and a lack of significant adverse effects on the CNS, eperisone hydrochloride represents a valuable alternative to traditional analgesics and muscle relaxants for the treatment of LBP.