Looking back and beyond the 2017 diagnostic criteria for hypermobile Ehlers-Danlos syndrome: A retrospective cross-sectional study from an Italian reference center.

The most common conditions with symptomatic joint hypermobility are hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD). Diagnosing these overlapping connective tissue disorders remains challenging due to the lack of established causes and reliable diagnostic tests. hEDS is diagnosed applying the 2017 diagnostic criteria, and patients with symptomatic joint hypermobility but not fulfilling these criteria are labeled as HSD, which is not officially recognized by all healthcare systems. The 2017 criteria were introduced to improve diagnostic specificity but have faced criticism for being too stringent and failing to adequately capture the multisystemic involvement of hEDS. Herein, we retrospectively evaluated 327 patients from 213 families with a prior diagnosis of hypermobility type EDS or joint hypermobility syndrome based on Villefranche and Brighton criteria, to assess the effectiveness of the 2017 criteria in distinguishing between hEDS and HSD and document the frequencies of extra-articular manifestations. Based on our findings, we propose that the 2017 criteria should be made less stringent to include a greater number of patients who are currently encompassed within the HSD category. This will lead to improved diagnostic accuracy and enhanced patient care by properly capturing the diverse range of symptoms and manifestations present within the hEDS/HSD spectrum.

Adults with paternal UPD14 causing Kagami-Ogata syndrome: Case report and review of the literature.

Kagami-Ogata syndrome (KOS) is a clinically recognizable syndrome in the neonatal period. It is characterized by specific skeletal anomalies and facial dysmorphisms. It is typically caused by paternal uniparental disomy of chromosome 14, while epimutations and microdeletions are less commonly reported causes. In the pediatric setting, KOS is a well delineated syndrome. However, there is a dearth of literature describing the natural history of the condition in adults. Herein, we describe a 35-year-old man, the first adult with KOS reported due to paternal uniparental disomy 14, and review reports of KOS in other affected adults. This highlights the variability in neurocognitive phenotypes, the presence of connective tissue abnormalities, and the uncertainties around long-term cancer risk.

High prevalence of gastrointestinal disorders in a large cohort of patients with joint hypermobility.

The gastrointestinal (GI) manifestations in children with hypermobile Ehlers-Danlos syndrome/joint hypermobility syndrome (hEDS/JHS) are not well described. We investigated the prevalence of GI disorders in children and young adults with hEDS/JHS through a single-center retrospective review. Demographic data, clinical history, symptoms, and diagnostic studies were reviewed. Of 435 patients with hEDS/JHS, 66% were females (age 5-28 years). We noted a high prevalence of constipation (61%), dysphagia (32%), dyspepsia and/or gastroparesis (25%), eosinophilic esophagitis (EoE) (21%), and celiac disease (4%) in our cohort. Upper endoscopy and gastric emptying scans had the highest yield to detect abnormalities. Motility studies were abnormal in 31% of the 80 patients who underwent them. Dysphagia symptoms are significantly associated with EoE. Thirty-three percent of dysphagia patients had EoE, versus 16% of non-dysphagia patients (p < 0.001). Screening hEDS/JHS patients for GI issues should be routine, with further investigations and referrals guided by identified symptoms.

A new conceptual model for anorexia nervosa: A role for connective tissue?

The etiology of anorexia nervosa (AN) remains to be fully elucidated, and current theories also fail to account for the direct effect of starvation on the health of the organs and tissues, specifically the connective tissue present in most organs of the body. Individuals with hereditary disorders of connective tissue manifest with clinical symptoms that overlap with AN, as the abnormal connective tissue also contributes to many of the other extra-articular manifestations of these hereditary disorders. This article hypothesizes that a similar pathophysiology may also contribute to the clinical presentation of AN. Therefore, a better understanding is needed to elucidate: (1) the relationship between abnormal connective tissue and AN, (2) the impact of starvation toward the development of abnormal connective tissue and how this manifests clinically, (3) the etiology of autonomic nervous system changes contributing to the dysautonomia in AN, and (4) how the sensory signals sent from potentially abnormal connective tissue to the central nervous system impact interoception in AN. A conceptual model incorporating abnormal connective tissue is provided. PUBLIC SIGNIFICANCE: The etiology of AN remains poorly understood and current theories fail to account for the direct impact of starvation on the health of the organs and tissues of the body. There is significant clinical overlap between AN and hereditary connective tissue disorders. This paper attempts to provide a new conceptual model for AN in which abnormal connective tissue contributes to the underlying pathogenesis.

Variability of joint hypermobility in children: a meta-analytic approach to set cut-off scores.

Current international consensus of the appropriate Beighton score cut-off to define if a child has generalised joint hypermobile or not is based upon expert opinion. Our aim was to determine the prevalence of Beighton scores of children worldwide to provide a recommendation for establishing the Beighton score cut-off to identify generalised joint hypermobility in children. We used AMED, OVID Medline, Embase and CINAHL to find published articles from inception to April 2024 describing Beighton scores of children up to and including 18 years from the general population. We extracted study demographics including country of publication, total number of participants, summary data about the age and sex of participant, Beighton scores and any cut-off used where authors deemed children hypermobile and how many children were rated at the corresponding Beighton scores. There were 37 articles reporting on the prevalence or incidence of hypermobility at cut-off scores from 28,868 participants. Using the cut-off of ≥ 6 resulted in a prevalence of 6% for studies reporting male data and 13% for studies reporting female data. Limited data reporting availability precluded further sub-analysis at a Beighton score of ≥ 7, age, pubertal status and ethnicity.    Conclusion: The working threshold for identifying generalised joint hypermobility in children should be a Beighton score of 6 or more. Our analysis also suggests a Beighton score of 7 or greater may be appropriate in childhood, particularly for females. What is Known: • The working threshold for identifying generalised joint hypermobility in children previously was set based on expert opinion. What is New: • The threshold to identify hypermobility in children should be at a minimum of ≥ 6 on the Beighton score.

Psychological interventions to improve pain, fatigue, anxiety, depression, and quality of life in children and adults with hypermobility spectrum disorders and Ehlers-Danlos syndrome: a systematic review.

Hypermobility spectrum disorders (HSD) affect individuals across physical, psychological and social domains, making assessment and management difficult. Management for this condition primarily focuses on addressing the musculoskeletal complaints using physiotherapy rather than the additional manifestations such as fatigue, anxiety and depression. This systematic review aims to identify psychological interventions and assess whether they improve the lived experiences of individuals with HSD. It also aims to assess which psychological interventions were most effective, which symptoms were most effectively managed by a psychological intervention, and whether there were differences between children and adults. Studies were included if they were a randomised controlled trial or pre/post-test design, a sample of any age and clinical diagnosis of HSD (including Ehlers-Danlos syndrome), used a psychological intervention and assessed the effect of the intervention on lived experiences using appropriate outcome measures. Risk of bias was assessed using the Mixed Methods Appraisal Tool. The results were narratively synthesised. Six studies were included in the review, one isolated psychological intervention and five incorporated a psychological intervention within a multidisciplinary programme. The interventions predominantly aimed to reduce pain including intensity, interference, pain-related fear and catastrophising, with anxiety and depression, affect, daily living, fatigue also being evaluated. The most beneficial psychological interventions were those delivered alongside physiotherapy in an outpatient or community setting, improving both the physical and psychological aspects of pain, subsequently improving quality of life. However, there lacks randomised controlled trials with larger samples to definitively confirm the significant findings discussed in this review.

Correlation between benign joint hypermobility syndrome and headache in children and adolescents.

BACKGROUND: Benign Joint Hypermobility Syndrome (BJHS) is a most common hereditary connective tissue disorders in children and adolescents. This study aimed to investigate the prevalence and subtypes of headache in children with BJHS. METHODS: This observational-analytical study was conducted in a case-control setting on school children aged 7 to 16 years in 2021-2023 in Isfahan, Iran. Students were examined for BJHS using Beighton criteria by a pediatric rheumatologist. Headache disorder was diagnosed according to the Child Headache-Attributed Restriction, Disability, and Social Handicap and Impaired Participation (HARDSHIP) questionnaires for child and adolescent and International Classification of Headache Disorders (ICHD-III). RESULTS: A total of 4,832 student (mean age 10.3 ± 3.1 years), 798 patients with BJHS and 912 healthy children were evaluated. The probability of headache in children aged 7-11 with hypermobility was 3.7 times lower than in children aged 12-16 with hypermobility (P = 0.001). The occurrence of headache in children with BJHS was more than the control group (P = 0.001), and the probability of headache in children with BJHS was 3.7 times higher than in healthy children (P = 0.001). Migraine was the most common headache type reported of total cases. The probability of migraine in children with BJHS was 4.5 times higher than healthy children ( P = 0.001). CONCLUSION: This study showed a significant correlation between BJHS and headache (especially migraine) in children and adolescents.

Anterior knee laxity is greater in athletic females who attain menarche at a younger age.

PURPOSE: Females with above-average anterior knee laxity values are at increased risk of anterior cruciate ligament (ACL) injury. The purpose of this study was to examine the effects of menarche age (MA) and menarche offset on anterior knee laxity in young, physically active women. METHODS: Anterior knee laxity (KT-2000) and menstrual characteristics (per self-report) were recorded in 686 Slovenian sportswomen from team handball, volleyball and basketball club sports (average years sport participation: 7.3 ± 3.6 years). Females were stratified into four groups based on their self-reported age at menarche: 9-11, 12, 13 and 14+ years. Anterior knee laxity was compared across MA groups using a univariate analysis of variance (ANOVA) with Bonferroni correction, with and without controlling for factors that could potentially differ between groups and influence anterior knee laxity. Females were then stratified into four groups based on the number of years they were away from their age at onset of menarche. Groups were compared using a univariate ANOVA with Bonferroni correction, with and without controlling for factors that differed between groups and could influence anterior knee laxity. RESULTS: Anterior knee laxity was greater in females who attained menarche at 12 years of age (6.4 ± 1.5 mm) or younger (6.6 ± 1.6 mm) compared to 14 years of age or older (5.8 ± 1.2 mm) (p < 0.001; partial η2 = 0.032). Anterior knee laxity was 0.7-1.4 mm greater in females who were 5 or more years away from menarche compared to those who were within 2 years of menarche (5.8 ± 1.3 mm; p < 0.001). CONCLUSION: Anterior knee laxity is greater in females who attained menarche at a younger age and in females who are 5 or more years postmenarche. Age of menarche represents a critical pubertal event that is easy for women to recall and may provide important insights into factors that moderate anterior knee laxity, a risk factor for ACL injury in women. LEVEL OF EVIDENCE: Level IV.

Prevalence of bilateral ulnar nerve subluxation among professional baseball pitchers.

BACKGROUND: Radiographic and physical examination findings of ulnar nerve instability have been recognized in overhead throwing athletes, despite the fact that some of these abnormalities may be asymptomatic and represent adaptive changes. While recommendations for screening and early detection have been made that can adversely impact an athletes' career, the presence of bilateral ulnar nerve subluxation and its relationship with medial elbow symptoms has not been characterized in professional overhead throwing athletes. PURPOSE: To characterize the prevalence of bilateral ulnar nerve subluxation among professional baseball pitchers. METHODS: A cross-sectional observational analysis was conducted utilizing standardized ultrasonographic examinations of bilateral elbows in 91 consecutive professional baseball pitchers (median age, 22 years; range, 17-30 years). The relationship between ulnar nerve subluxation and ulnar nerve signs, symptoms, and provocative physical examination maneuvers was also investigated. RESULTS: The prevalence of bilateral ulnar nerve subluxation was 26.4% (95% CI, 17.7%-36.7%; 24 of the 91 athletes). Thirty-five athletes (38.5%; 95% CI, 28.4%-49.2%) had subluxation in at least 1 elbow. No athletes with subluxation had positive ulnar nerve signs, symptoms, or provocative tests. CONCLUSION: Ulnar nerve subluxation is common among professional pitchers, and is more often than not bilateral. In this population of athletes, ulnar nerve subluxation does not appear to be associated with pathological findings.

First ray mobility in hallux rigidus, hallux valgus, and normal feet based on weightbearing computed tomography and three-dimensional analysis: A case-control study.

BACKGROUND: Hallux valgus and hallux rigidus are disorders affecting the first ray and are associated with hypermobility of this structure. This study aimed to investigate the three-dimensional mobility of each joint of the first ray between feet with hallux valgus or hallux rigidus and healthy feet using weightbearing and nonweightbearing computed tomography (CT). METHODS: This case-control study analyzed 17 feet of 11 healthy volunteers (control group), 16 feet of 16 patients with hallux valgus (HV group), and 16 feet of 11 patients with hallux rigidus (HR group). First, nonweightbearing foot CT imaging was performed in the supine position on a loading device with no load applied, with the legs extended and the ankle in the neutral position. Next, a load equivalent to body weight was applied for weightbearing CT imaging. Distal bone displacement relative to the proximal bone was quantified three-dimensionally under both conditions. RESULTS: In the HV group, the talonavicular joint showed significantly greater eversion (P = 00.011) compared with the control group and significantly greater dorsiflexion (P = 00.027) and eversion (P < 00.01) compared with the HR group. In the medial cuneiform joint, the HV group showed significantly greater eversion (P < 00.01) and abduction (P = 00.011) than the control group. For the first tarsometatarsal joint, the HV group showed significantly greater dorsiflexion (P = 00.014), inversion (P = 00.028), and adduction (P < 00.01) than the control group, and greater inversion (P < 00.01) and adduction (P < 00.01) than the HR group. Dorsiflexion of the first tarsometatarsal joint was significantly greater in the HR group compared with the control group (P = 00.026). CONCLUSION: Hypermobility of the first ray appears to be three-dimensional: in hallux valgus, it is centered at the first tarsometatarsal joint, while in hallux rigidus it is mainly in the sagittal plane at the first tarsometatarsal joint only. This difference may explain the different deformities ultimately observed in each condition.

Risk factors associated with symptoms of temporomandibular disorders among women with hypermobile Ehlers-Danlos syndrome: Questionnaire-based study in Finland and Sweden.

BACKGROUND: Generalized joint hypermobility as a characteristic feature of Ehlers-Danlos syndromes (EDS) is among the factors contributing to temporomandibular disorders (TMD). OBJECTIVE: To evaluate the prevalence of TMD symptoms and their risk factors among women born in Sweden or Finland who were 27- to 78-year-olds with diagnosed hypermobile EDS (hEDS). METHODS: A cohort of women with confirmed hEDS (n = 185) was constructed from the members of the National EDS Associations in both countries. Based on questionnaire data, frequency of independent variables in terms of socio-demographic, general health and oral health-related factors, comorbid symptoms and psychological distress for self-reported TMD symptoms as the dependent variables, were calculated first. Prevalence ratios (PR) and their 95% confidence interval (95% CI) were estimated for the association between independent and dependent variables. RESULTS: Nearly all participants reported TMD symptoms (98%) with TMD pain (95%), TMJ clicking (90%) and jaw fatigue (80%) as the most common symptoms and TMJ crepitation (63%) and luxation (44%) as the least common symptoms. Risk factors for TMD among 27- to 50-year-olds participants were Finland as a country of birth, living alone and self-reported worst pain in the body (not the joints). The respective risk factors among the 51- to 78-year-olds were Finland as a country of birth, family history of EDS, tinnitus and regularly taking contraceptives. CONCLUSIONS: Among adult women with confirmed hEDS, socio-demographic and health-related factors and comorbid symptoms were significantly associated with TMD but with differences regarding age group. Therefore, management of TMD requires a multidisciplinary approach among the affected.

Treating pain in patients with Ehlers-Danlos syndrome : Multidisciplinary management of a multisystemic disease.

BACKGROUND: The clinical picture of people with Ehlers-Danlos syndromes (EDS) is complex and involves a variety of potential causes of pain. This poses major challenges to patients and healthcare professionals alike in terms of diagnosis and management of the condition. OBJECTIVES: The aim of the article was to provide an overview of the specific pain management needs of patients with EDS and address their background. MATERIAL AND METHODS: A selective literature search was performed to highlight the current state of research on pain management in EDS patients. RESULTS: Affected patients require multimodal pain management considering their individual needs, disease-specific features, and comorbidities. CONCLUSION: Medical awareness and evidence need to be further improved to enhance the medical care situation of these patients with complex needs.

Digital resources for surgical and restorative treatment of excessive gingival display in one session.

OBJECTIVE: This article aims to showcase the implementation of a digital workflow in addressing a case of multifactorial excessive gingival display in a patient with high esthetic demands, incorporating both surgical and restorative interventions in a single session. CLINICAL CONSIDERATIONS: A 28-year-old female patient presented with excessive gingival display, attributed to a combination of short teeth due to altered passive eruption, lip hyperactivity, and a sub-nasal depression that lodged the upper lip during spontaneous smiling. The multidisciplinary treatment strategy encompassed surgical crown lengthening, the placement of a biovolume in the maxillary concavity, and the rehabilitation of the six anterior teeth with direct composite resin, all done in a single session. Smilecloud Biometrics was used to digitally plan the smile, and the final wax-up/mock-up was approved by the patient prior to any irreversible procedure. A digital planning center (GuiderLab) enabled the materialization of the virtual planning and the printing of the periodontal surgical guide, the biovolume, and the resin layering guides for the restorative technique. CONCLUSIONS: Adopting a digital workflow in multidisciplinary cases with excessive gingival display leads to predictable and more expedited outcomes, ensuring a favorable result between soft and hard tissues. CLINICAL SIGNIFICANCE: Excessive gingival display is a condition with multifactorial etiologies, including dentoalveolar, periodontal, skeletal, or muscular origins, or a combination of these factors. The diagnoses of altered passive eruption and a hypermobile upper lip are common in daily clinical practice and can be successfully managed through surgical crown lengthening and filling of the maxillary concavity, respectively. To achieve the desired outcome, restorative procedures often complement these surgical interventions.

Impact of chronic wrist hypermobility on proprioception, strength, and functional performance in young adults.

BACKGROUND: Chronic joint hypermobility has been attributed to repetitive ligamentous microtrauma, benign joint hypermobility syndrome (BJHS), or genetic connective tissue disorders that lead to pain and functional impairment, especially among females. Chronic wrist hypermobility (CWH) prevalence, etiology, and effects on proprioception, strength, and function have yet to be established. PURPOSE: This pilot study aimed to determine the CWH prevalence among adults; its effects on proprioception, strength, and function; and whether these effects are gender based. STUDY DESIGN: This was a quasi-experimental cross-sectional study. METHODS: Ninety wrists (55 participants, mean age 27.46 years) with no wrist range of motion (ROM) restrictions or previous trauma for ≥6 months were screened for CWH based on an exploratory set of diagnostic criteria. Fifty-eight wrists (34 adults) were allocated to a CWH group, and 32 wrists (21 adults) were allocated to a healthy control group. Twenty-five CWH and 25 healthy control matched (gender, age, and handedness) participants were compared. Assessment included the active wrist joint position sense test, hand-held dynamometry for wrist extension and grip strength, and the patient-rated wrist evaluation for function. Testers were blinded to group allocation. RESULTS: A 64.4% CWH prevalence existed among CWH participants, who were mostly asymptomatic females (74%). Frequent etiologic factors were midcarpal (96.5%) and scapholunate (39%) instabilities and BJHS (37%), which was higher among females (30%) than males (8%). Independent t-tests showed statistically significant (p < 0.05) group differences in wrist proprioception, wrist isometric extension, grip strength, and function with moderate-high (0.41-0.75) effect size. No significant gender differences existed in proprioception and function. CONCLUSIONS: CWH is very prevalent among functional independent young adults with atraumatic midcarpal and intercarpal ligamentous laxities and BJHS. CWH prevails among women and adversely affects wrist proprioception, strength, and function. The study's specific CWH diagnostic criteria may be useful for clinicians to identify and timely manage impacted individuals by CWH.

Development, validation, and psychometric analysis of Foot and Ankle Flexibility Index (FAFI).

AIM: To develop a new tool for identifying joint hypermobility of the paediatric foot and ankle, based on a dichotomous scoring system utilising the Lower Limb Assessment Score (LLAS), to separate the foot and ankle items. MATERIAL AND METHODS: A total of 205 children, aged between 5 and 10 years, participated in a cross-sectional study. The new tool Foot and Ankle Flexibility Index (FAFI) was predicated upon the last 7 items of LLAS, which are specific to the foot and ankle. The internal consistency was measured with Cronbach's test. Kappa statistics with 95% CI were calculated to verify the level of inter-rater and intra-rater agreement for the FAFI. RESULTS: Cronbach's alpha returned 0.82. The correlations between items returned a mean of 0.59 (range: 0.43-0.74). The discrimination score on the ROC curve (4 points) showed that the model can be used to identify children with joint hypermobility of the foot and ankle. Inter-rater reliability was largely good (ICC = 0.89). Excellent intra-rater reliability was found (ICC = 0.96) CONCLUSIONS: This study identified high reliability between evaluators, and high sensitivity and specificity, for a new reliable and valid tool for the identification of foot and ankle joint hypermobility.

Conservative management of hand impairment in children and adolescents with heritable disorders of connective tissue: A scoping review.

AIMS: To synthesize and critically appraise available interventions in the conservative management of hand impairment for children and adolescents with heritable disorders of connective tissue (HDCT). METHODS: A search of peer-reviewed literature and online platforms were included with data regarding hand impairment and function, conservative management and outcome measures extracted and appraised. Levels of evidence were applied to published literature. RESULTS: Ten peer-reviewed papers, eleven webpages and YouTube videos met the inclusion criteria. Reported interventions included: strengthening, orthoses, assistive equipment, education and pacing. Evidence of intervention effectiveness and evidence-based guidance on dosage were absent, with no consistency of outcome measures monitoring intervention effectiveness. Online platforms posted by health professionals predominantly provided advice for families without clinical detail of interventions. CONCLUSIONS: There is a consistent suite of interventions identified in both peer-reviewed literature and online platforms used by clinicians and families to manage hand impairment for children and adolescents with HDCT. Clear dosage parameters and outcome measures are needed in future intervention studies to determine the effectiveness of interventions and guide clinicians in how best to treat hand impairment. Increasing accountability and quality of online resources posted by health professionals for families is warranted to ensure dosage details and precautions are provided.

Chronic Pain and Joint Hypermobility: A Brief Diagnostic Review for Clinicians and the Potential Application of Infrared Thermography in Screening Hypermobile Inflamed Joints.

Joint hypermobility syndromes, particularly chronic pain associated with this condition, including Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSD), present diagnostic challenges due to their multifactorial origins and remain poorly understood from biomechanical and genomic-molecular perspectives. Recent diagnostic guidelines have differentiated hEDS, HSD, and benign joint hypermobility, providing a more objective diagnostic framework. However, incorrect diagnoses and underdiagnoses persist, leading to prolonged journeys for affected individuals. Musculoskeletal manifestations, chronic pain, dysautonomia, and gastrointestinal symptoms illustrate the multifactorial impact of these conditions, affecting both the physical and emotional well-being of affected individuals. Infrared thermography (IRT) emerges as a promising tool for joint assessment, especially in detecting inflammatory processes. Thermal distribution patterns offer valuable insights into joint dysfunctions, although the direct correlation between pain and inflammation remains challenging. The prevalence of neuropathies among hypermobile individuals accentuates the discordance between pain perception and thermographic findings, further complicating diagnosis and management. Despite its potential, the clinical integration of IRT faces challenges, with conflicting evidence hindering its adoption. However, studies demonstrate objective temperature disparities between healthy and diseased joints, especially under dynamic thermography, suggesting its potential utility in clinical practice. Future research focused on refining diagnostic criteria and elucidating the underlying mechanisms of hypermobility syndromes will be essential to improve diagnostic accuracy and enhance patient care in this complex and multidimensional context.

Surgical management of severe planovalgus foot deformity in children with generalised joint hypermobility.

BACKGROUND: This study aimed to evaluate the outcomes of calcaneal lengthening osteotomy (CLO) and double arthrodesis of the talonavicular and calcaneocuboid joints (DA) for correcting planovalgus foot deformity exclusively in patients with generalised joint hypermobility. METHODS: We retrospectively reviewed 29 feet in 17 consecutive patients who underwent either CLO or DA. The mean age at surgery was 11.3 ± 2.3 years, and the mean follow-up duration was 7.7 ± 3.2 years. Preoperative and final follow-up radiographs and dynamic foot-pressure measurements were analysed. RESULTS: Both operations significantly improved the radiographic parameters, except for the lateral talocalcaneal angle in the CLO group. Pedobarographic study demonstrated an elevation of the medial longitudinal arch and an improved foot-pressure distribution after both surgeries. The plantar pressure in the lateral forefoot significantly increased only in the DA group, while the pressures exerted on the medial forefoot and hindfoot and the arch index improved only in the CLO group. CONCLUSIONS: Both CLO and DA effectively improve the foot alignments of the deformity in patients with generalised joint hypermobility. However, differences were observed in the changes in the lateral talocalcaneal angle and plantar pressure distribution between the two procedures. LEVEL OF EVIDENCE: Therapeutic Level III.

Ultrasound in Female Urinary Incontinence.

Urinary incontinence (UI) is a common health condition that may interfere with the quality of life. A comprehensive evaluation of female UI helps with effective and safe treatments. Ultrasound has gained popularity to explore UI recently because it can collect crucial information for treatment planning and counseling. Translabial and introital approaches are commonly and reliably applied to ultrasound. The images can be obtained using two-dimensional and three-dimensional ultrasounds. Ultrasound is the only modality capable of confirming the presence or absence of a mid-urethral sling (MUS) and is able to demonstrate bulking agents as well. Although some of the ultrasound findings may only be incidental or supplementary to the patient's symptoms, ultrasound benefits for investigating the pathophysiology of UI and surgical outcomes of MUS procedures. It is anticipated that standardization in terminology, measurement techniques, and reporting can be established in the near future.

Placing joint hypermobility in context: traits, disorders and syndromes.

BACKGROUND: Joint hypermobility (JHM) is a common physical trait. It may occur alone or in combination with musculoskeletal (MSK) pain, outside or within more complex phenotypes. Hypermobility spectrum disorders (HSD) are diagnosed in individuals with JHM and related MSK pain, when an alternative diagnosis cannot be identified. Conversely, the Ehlers-Danlos syndrome (EDS) encompasses a group of rare hereditary connective tissue disorders featuring JHM along with other pleiotropic manifestations. The 2017 EDS Classification identifies 13 different subtypes. Hypermobile EDS (HEDS) is the only EDS variant still lacking a confirmatory test. SOURCES OF DATA: Literature was reviewed searching for the most relevant papers related to key arguments. Particular attention was focused on papers published after the 2017 Classification. AREAS OF AGREEMENT: Definition, epidemiology, assessment tools and patterns of JHM are presented. The morbid nature of the 2017 EDS Classification and of the 'spectrum' is also illustrated. AREAS OF CONTROVERSY: We discuss current limitations and disagreements concerning the 'spectrum', HSD and HEDS. GROWING POINTS: In the clinical context, elucidation of the pathophysiology of pain related to JHM should develop in parallel with the analysis of pleiotropic manifestations of syndromes with JHM. AREAS TIMELY FOR DEVELOPING RESEARCH: Future challenges concerning classification, nosology, diagnosis and management of JHM, EDS and related disorders are discussed.