Joint hypermobility can increase complications in surgical management of musculoskeletal conditions. The purpose of this review is to 1) summarize clinical outcomes in patients undergoing various Orthopedic sports medicine surgical procedures by joint, and 2) compare outcomes in patients with and without hypermobility.

A total of 38 studies consisting of 144,609 patients and 144,860 joints were included (140,625 shoulders, 2,043 hips, 1,499 knees, 689 ankles, and four elbows). Notable heterogeneity exists within the literature regarding outcomes in hypermobile patients undergoing Orthopedic sports medicine procedures. In general, outcomes for hypermobile patients undergoing procedures of the shoulder, elbow, hip, and knee demonstrated similar or slightly worse outcomes across various patient reported outcomes measures (PROMs) and in terms of recurrent instability or dislocation. There were consistently worse outcomes (e.g. higher rates of post-operative recurrent instability, PROMs) in studies evaluating hypermobile patients undergoing surgery for chronic lateral ankle instability (CLAI) compared to non-hypermobile controls. Patients with joint hypermobility tend to have similar or worse outcomes following Orthopedic sports medicine procedures related to the shoulder, knee, hip, and elbow, and consistently worse outcomes for the ankle. Future studies are encouraged to focus on the effect of hypermobility on elbow and wrist sports pathologies.

Individuals living with Hypermobility Spectrum Disorders and the hypermobile Ehlers-Danlos syndrome (HSD/hEDS) often experience recurrent joint injury, chronic pain, and fatigue. Although generalized anxiety has been recognized as a common comorbidity with HSD/hEDS, minimal research has examined depressive symptoms in this population. The purpose of this investigation was to describe the prevalence, nature, and severity of depressive symptoms in the HSD/hEDS population, and to explore associations with other potential confounding factors. All individuals with HSD/hEDS referred to a specialized connective tissue disorder Physical Medicine & Rehabilitation clinic were asked to self-report demographic data and complete the 9-item Patient Health Questionnaire (PHQ-9), 7-item Generalized Anxiety Disorder Questionnaire (GAD-7), Pain Catastrophizing Symptoms (PCS) questionnaire, and Fatigue Severity Scale (FSS) at their initial clinic visit. Data was prospectively collected between January 2019 and December 2024. Descriptive statistics were performed. A Spearman correlation matrix was used to identify relevant factors associated with depressive symptoms. Relationships emerging as significant (p < 0.001) were further analyzed using independent sample Mann-Whitney U-tests. Fifty-nine individuals (53 female, mean ± SD age: 34.4 ± 11 years) were included, with a mean ± SD PHQ-9 score of 11.2 ± 5.9, indicating moderate depressive symptom severity. 53% of participants (n = 31) met criteria for major depressive disorder (PHQ-9 ≥ 10). Higher pain catastrophizing (ρ = 0.611, p < 0.001) and higher fatigue scores (ρ = 0.593, p < 0.001) were both associated with significantly higher depressive symptoms, but there were no associations with respect to age, working status, and number of alcoholic drinks consumed per week. This research suggests that depression is highly prevalent in patients experiencing HSD/hEDS. There is also a strong association between pain catastrophizing and fatigue in those experiencing depressive symptoms. The interaction between depressive symptoms, pain catastrophizing, and fatigue should be considered in the holistic management of HSD/hEDS.

The close association of syndromes of orthostatic intolerance with and without postural orthostatic tachycardia syndrome (POTS) with Joint Hypermobility Disorders (JHD) including Hypermobility Spectrum Disorder (HSD) and hypermobile Ehlers Danlos Syndrome (hEDS) and with Mast Cell Activation Syndrome (MCAS) is now firmly established. However, the prevalence of each entity relative to the other is not well established and is affected greatly by the various definitions used for each syndrome. Use of restricting definitions for each syndrome can be problematic in the clinical setting as it under-estimates the presence of disease, thereby preventing clinicians from considering potentially helpful therapeutic options.

A retrospective review of the clinical records of 100 young patients meeting POTS criteria was undertaken to determine the frequency of HSD, near-hEDS, and hEDS as well as the frequency of MCAS using consensus-1, conservative consensus-2, and clinical criteria regardless of lab support. Effectiveness of MCAS therapies was assessed in relation to the method of MCAS diagnosis.

From records of 392 patients with orthostatic intolerance syndromes, 100 patients met POTS criteria. The frequency of JHD ranged from 13% using strict criteria of hEDS to 34% using HSD Criteria. The frequency of MCAS ranged from 2% using consensus-1 criteria, to 37% using conservative consensus-2 criteria, to 87% using clinical criteria. Patients diagnosed by clinical criteria with or without the aid of labs responded to therapy similarly to those diagnosed with stricter criteria.

Using overly strict criteria to diagnose conditions which have a high prevalence of co-occurrence misses opportunities for potential therapeutic strategies.

Previous research has demonstrated impaired proprioception and poorer responses to tactile deep pressure, visual-tactile integration, and vestibular stimuli in individuals with generalized hypermobility, potentially leading to sensory processing issues. Therefore, we aimed to explore the influence of hypermobility on somatognosia and stereognosia.

Forty-six participants were assessed using the Beighton score and categorized into three groups: non-hypermobile (n = 20), symptomatic hypermobile (n = 13), and asymptomatic hypermobile (n = 13). Somatognosia was evaluated using the shoulder width test in the vertical plane and pelvic width test in the vertical and horizontal planes. Stereognosia was assessed with Petrie's test. Spearman's rank correlation coefficient was examined the relationship between the Beighton score and measures of somatognosia and stereognosia. An unpaired t-test was used to compare variables between hypermobile (both symptomatic and asymptomatic) and non-hypermobile individuals, while a one-way ANOVA was used to compare data between the three groups.

No significant relationship was observed between Beighton scores and measures of somatognosia and stereognosia. The t-test revealed no statistically significant differences between hypermobile and non-hypermobile groups in the shoulder width, two pelvic widths, and Petrie's tests (all p ≥ 0.105). Similarly, one-way ANOVA showed no statistically significant differences between the three groups across these tests (all p ≥ 0.177).

The results indicate that somatognosia and stereognosia are not significantly related to the Beighton score and do not significantly differ between the groups studied. These sensory processing functions are unlikely to contribute to the common complaints reported by hypermobile individuals.

Not applicable.

Increasing research suggests a link between autism spectrum disorders and joint hypermobility, hypermobility spectrum disorders, and Ehlers-Danlos syndromes. However, no study systematically examined the available literature about the relationship between these conditions. A systematic literature search was conducted to identify studies (a) examining the link between autism, joint hypermobility, hypermobility spectrum disorders or Ehlers-Danlos syndrome, and (b) reporting the frequency of autism spectrum disorders in people with joint hypermobility, hypermobility spectrum disorders or Ehlers-Danlos syndrome, or vice versa. Prevalence meta-analyses were performed. Twenty articles met the inclusion criteria. Twelve studies explored joint hypermobility/hypermobility spectrum disorders/Ehlers-Danlos syndrome in autistic people. Six explored autism spectrum disorders/autistic traits in people with hypermobility spectrum disorders/Ehlers-Danlos syndrome. Two studies examined autism spectrum disorders in relatives of patients with hypermobility spectrum disorders/Ehlers-Danlos syndrome, and two explored autistic traits and joint hypermobility in non-clinical samples. Out of 15 studies examining the association between autism spectrum disorders/autistic traits and joint hypermobility/hypermobility spectrum disorders/Ehlers-Danlos syndrome, 12 reported significant results. The overall prevalence of joint hypermobility in autistic individuals was 22.3%, but it was higher (31%) when only studies assessing joint hypermobility clinically (excluding self-reports) were considered. The overall prevalence of hypermobility spectrum disorders/Ehlers-Danlos syndrome in autistic samples was 27.9%, but 39% if hypermobility spectrum disorders/Ehlers-Danlos syndrome were assessed clinically. Despite the heterogeneity of studies, these results suggest an association between autism and joint hypermobility/hypermobility spectrum disorders/Ehlers-Danlos syndrome that should be confirmed in further research.Lay abstractIncreasing research suggests a link between autism spectrum disorders (ASD) and joint hypermobility (JH), hypermobility spectrum disorders (HSD), and Ehlers-Danlos syndromes (EDS). However, no study systematically examined the available literature about the relationship between these conditions. To fill this gap, we conducted a systematic literature search to identify studies: (a) examining the link between autism, JH, HSD, or EDS, and (b) reporting the frequency of ASD in people with JH, HSD, or EDS, or vice versa. Prevalence meta-analyses were performed. Twenty articles met the inclusion criteria. Twelve studies explored JH/HSD/EDS in autistic people. Six explored ASD/autistic traits in people with HSD/EDS. Two studies examined ASD in relatives of patients with HSD/EDS, and two explored autistic traits and JH in non-clinical samples. Out of 15 studies examining the association between ASD/autistic traits and JH/HSD/EDS, 12 reported significant results. The overall prevalence of JH in autistic individuals was 22.3%, but it was higher (31%) when only studies assessing JH clinically (excluding self-reports) were considered. The overall prevalence of HSD/EDS in autistic samples was 27.9%, but 39% if HSD/EDS were assessed clinically. Despite the heterogeneity of studies, these results suggest an association between autism and JH/HSD/EDS that should be confirmed in further research.

This case highlights the intersection of psychiatric symptoms and hypermobility spectrum disorder (HSD) in a woman in her 20s with multiple comorbidities, including postural orthostatic tachycardia syndrome, chronic pain and obstructive sleep apnoea. The patient had been unresponsive to conventional psychiatric treatments for anxiety and panic attacks. On recognition of HSD, a multidisciplinary approach, including propranolol for autonomic dysfunction, pregabalin for pain and anxiety, and lisdexamfetamine for cognitive impairment, significantly improved her condition. Early identification and tailored management of HSD in psychiatric settings can optimise treatment outcomes by addressing both physical and psychiatric symptoms. This case underscores the need for psychiatrists to incorporate physical health assessments into their diagnostic processes when treating complex psychiatric presentations.

Symptomatic joint hypermobility, as found in conditions like hypermobile Ehlers-Danlos syndrome (hEDS), presents unique challenges in pain management due to associated symptoms such as chronic pain, joint instability, and dysautonomia. Despite the high prevalence of hypermobility and associated healthcare costs, there is a lack of research on effective treatments for these patients, particularly in the context of multidisciplinary pain rehabilitation programs.

This study aims to compare the baseline characteristics, attendance, graduation rates, and patient satisfaction of hypermobile and non-hypermobile adult outpatients participating in a multidisciplinary pain rehabilitation program (PRP).

This retrospective cohort study analyzed clinical data from 335 patients at the Indiana University Health Pain Navigation Service between January 1, 2023, and December 31, 2023. Baseline characteristics were assessed using patient-reported outcome measures, and attendance and graduation rates were tracked. Hypermobile and non-hypermobile groups were compared with independent samples t-tests and chi-squared tests. A multiple linear regression model was used to assess the impact of hypermobility diagnosis on PRP attendance, with pertinent demographic and baseline clinical scores entered as covariates.

Hypermobile patients differed significantly from non-hypermobile patients in demographics, including age, gender, race, education, and employment status. Despite these differences, hypermobile patients did not differ from non-hypermobile patients in PRP attendance or graduation rates. Baseline pain, depression, and pain catastrophizing scores were lower in the hypermobile group. Exit surveys indicated similar levels of overall satisfaction with the program, though hypermobile patients were less likely to report that their needs were fully met than were non-hypermobile patients.

Despite the potential for joint hypermobility to pose a barrier to participation in multidisciplinary pain rehabilitation programs, we found no evidence that patients with a hypermobile diagnosis had less participation in an intensive outpatient pain rehabilitation program. After accounting for group differences in key demographic and clinical variables, there were no significant differences in PRP attendance between hypermobile and non-hypermobile patients. Our results are encouraging regarding the potential for multidisciplinary pain rehabilitation programs to serve the needs of these patients.

Revision surgery after anterior cruciate ligament reconstruction (ACL-R) is hypothesized to be the result of an interplay between factors associated with the anatomy, physiological characteristics and environment of the patient. The multifactorial nature of revision ACL-R risk is difficult to quantify, and evidence regarding the independent roles of potentially important variables is inconsistent throughout the literature. Young patient age is often cited as one of the most prominent risk factors for reinjury after ACL-R. However, the association between a non-modifiable variable such as patient age and revision ACL-R risk is likely to be a spurious correlation due to the confounding effect of more important variables. From the perspective of healthcare professionals aiming to mitigate revision ACL-R risk through targeted interventions, awareness of factors like generalized joint hypermobility, bone morphology, muscle strength imbalances, and genetic factors is critical for the individualized risk assessment of patients with ACL injury. The aim of this current concepts article is to raise awareness of the essential anatomical, physiological, and activity-related risk factors associated with ACL injury and reinjury risk that are likely captured and confounded by patient age. LEVEL OF EVIDENCE: Level V.

The study aims to evaluate the clinical outcomes in patients with hypermobility spectrum disorder (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) with chronic pain following treatment with cannabis-based medicinal products (CBMPs).

This was a case series conducted with the UK Medical Cannabis Registry. The primary outcomes were changes in the following validated patient-reported outcome measures at 1, 3, 6, 12, and 18 months compared with baseline: Short-Form McGill Pain Questionnaire 2 (SF-MPQ-2), pain visual analog scale score (Pain-VAS), Brief Pain Inventory (BPI), five-level EQ-5D (EQ-5D-5L), Single-Item Sleep Quality Scale (SQS), General Anxiety Disorder Seven-Item Scale (GAD-7), and Patient Global Impression of Change. The incidence of adverse events was analyzed as secondary outcomes. Statistical significance was defined as P <0.050.

A total of 161 patients met inclusion criteria. Improvements were observed in BPI severity and interference subscales, SF-MPQ-2, and Pain-VAS (P < 0.001). Changes were also seen in the EQ-5D-5L index value, SQS, and GAD-7 (P < 0.001). A total of 50 patients (31.06%) reported one or more adverse event with a total incidence of 601 (373.29%). The most frequent rating for adverse events was moderate (n = 258; 160.25%), with headache being the most common (n = 44; 27.33%).

An association was identified between patients with HSD/hEDS with chronic pain and improvements in pain-specific and general health-related quality of life following the commencement of CBMPs. CBMPs were also well tolerated at 18 months. These findings must be interpreted within the context of the limitations of study design but add further weight to calls for randomized controlled trials.

This study describes aspects of pain and how pain affects everyday life and examines the relation between chronic pain and activity limitations in people with hypermobility spectrum disorders (HSD) or hypermobility Ehlers Danlos syndrome (hEDS).

This cross-sectional study used data from 2016 to 2021 obtained from the Swedish quality registry for pain rehabilitation (SQRP), comparing those with HSD/hEDS with the larger group of people with mixed chronic pain conditions as a reference group (RG).

Of the 43,801 people registered in the SQRP, 1211 (2.8%) were diagnosed with HSD/hEDS (88.9% women). The mean age of the HSD/hEDS group was younger (36.3 ± 11.8) than the RG (45.7 ± 12.8). The HSD/hEDS group had a statistically significant (p < 0.001) earlier onset of pain (calculated in years) in contrast to the RG. In the HSD/hEDS group, 80.1% had persistent pain; in the RG, 74.2% had persistent pain. The HSD/hEDS group reported more pain locations (20.0 ± 7.9) than the RG (14.8 ± 8.8). The HSD/hEDS group reported more problems performing leisure, social, and household activities than the RG; however, pain intensity was statistically significantly lower (p < 0.001) in the HSD/hEDS related to the RG.

There were indications that pain affected daily activities for people with HSD/hEDS, who had earlier onset of pain, marked more pain locations, and had more persistent pain, but pain intensity was not as decisive in contrast to the RG.

In a comparison yielding statistically significant results (p < 0.001), persons with hypermobility spectrum disorder (HSD) or hypermobility Ehlers-Danlos syndrome (hEDS) reported earlier pain onset, longer pain durations, and a greater number of pain locations but surprisingly, lower pain intensity than the reference group which consisted of a mixed group of pain conditions. These pain characteristics affected daily activities, indicating a substantial impact on daily life for those with HSD/hEDS.

Hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos syndrome (h-EDS) are connective tissue disorders associated with joint hypermobility, pain, fatigue and autonomic dysfunction. We sought to assess sexual function in women with h-EDS/HSD.

In this cross-sectional community-based case-control study, women with h-EDS/HSD completed the following online questionnaires: Female Sexual Function Index (FSFI), 31-item Composite Autonomic Symptom Score (COMPASS-31), Beck Depression Inventory-II (BDI-II) and an additional short form with questions pertaining to comorbidities and sexual activity. Scores were compared with those of healthy female controls.

A total of 84 women with h-EDS/HSD [mean age 37.1 years (s.d. 8.4)] and 75 healthy women [mean age 29.79 years (s.d. 5.38)] completed the questionnaires. Of these, 75% were diagnosed with h-EDS, 25% with HSD and 58% had concurrent postural orthostatic tachycardia syndrome. A majority of women with h-EDS/HSD (52%) did not engage in any sexual activity, and only 25% reported having sexual intercourse with a partner in the past 6 months. The mean COMPASS-31 score was 51.5 (s.d. 13.8), mean BDI-II score was 24.6 (s.d. 11.4) and mean FSFI score was 15.3 (s.d. 7.9) in the patient group. Compared with healthy controls, women with h-EDS/HSD had decreased FSFI scores in the subdomains of desire, arousal, lubrication, orgasm and sexual satisfaction. Neither BDI-II nor COMPASS-31 scores were predictive of the FSFI score.

Compared with healthy women, we found significant sexual dysfunction in women with h-EDS/HSD, which did not correlate with depressive or autonomic symptoms in this cohort. Given its health implications, sexual dysfunction represents a significant unmet need that calls for development of targeted diagnostic and therapeutic approaches in the care of women with h-EDS/HSD.

Past research has indicated that individuals with Ehlers-Danlos Syndromes (EDS) and Generalized Hypermobililty Spectrum Disorder (G-HSD) report psychological and psychiatric symptoms, particularly anxiety disorders and depressive symptoms, at much greater rates than the general population. However, these studies have been primarily conducted in small samples at European centres. We report a retrospective chart review from 1035 consecutive patients (88% female) assessed for EDS/G-HSD at the GoodHope EDS Clinic at Toronto General Hospital between June 2019 and June 2021. Prior to assessment, all patients completed self-reported mental health screening questions, the Inventory of Depressive and Anxiety Symptoms - Dysphoria scale, and the Borderline Symptom List-23. The majority of patients reported current or past anxiety or depressive symptoms (53-87%), and a substantial minority reported significant mental health concerns, including Posttraumatic Stress Disorder (4.7-34.8%), disordered eating (19%), self-harm (3-29.2%), and suicidal behaviour (7.8-18.6%). Patients did not differ by diagnostic category on self-report measures of dysphoria or borderline symptoms. Individuals with G-HSD reported higher rates of anxiety and depression in clinical interview than those diagnosed with non-hypermobile EDS, and endorsed a higher rate of having "struggled with anxiety or depression" on the mental health screening questionnaire than individuals not diagnosed with EDS/G-HSD. No other differences emerged across diagnostic groups. These findings highlight the need for psychological support for individuals with EDS or G-HSD.

Anxiety symptoms are elevated among people with joint hypermobility. The underlying neural mechanisms are attributed theoretically to effects of variant connective tissue on the precision of interoceptive representations contributing to emotions.

To investigate the neural correlates of anxiety and hypermobility using functional neuroimaging.

We used functional magnetic resonance neuroimaging to quantify regional brain responses to emotional stimuli (facial expressions) in people with generalised anxiety disorder (GAD) (N = 30) and a non-anxious comparison group (N = 33). All participants were assessed for joint laxity and were classified (using Brighton Criteria) for the presence and absence of hypermobility syndrome (HMS: now considered hypermobility spectrum disorder).

Participants with HMS showed attenuated neural reactivity to emotional faces in specific frontal (inferior frontal gyrus, pre-supplementary motor area), midline (anterior mid and posterior cingulate cortices) and parietal (precuneus and supramarginal gyrus) regions. Notably, interaction between HMS and anxiety was expressed in reactivity of the left amygdala (a region implicated in threat processing) and mid insula (primary interoceptive cortex) where activity was amplified in people with HMS with GAD. Severity of hypermobility in anxious, compared with non-anxious, individuals correlated with activity within the anterior insula (implicated as the neural substrate linking anxious feelings to physiological state). Amygdala-precuneus functional connectivity was stronger in participants with HMS, compared with non-HMS participants.

The predisposition to anxiety in people with variant connective tissue reflects dynamic interactions between neural centres processing threat (amygdala) and representing bodily state (insular and parietal cortices). Correspondingly, interventions to regulate amygdala reactivity while enhancing interoceptive precision may have therapeutic benefit for symptomatic hypermobile individuals.

Hypermobility spectrum disorders (HSD) and hypermobility Ehlers-Danlos syndrome (hEDS) are frequently associated with gastrointestinal symptoms, although the underlying mechanisms remain unclear. This study aimed to compare antroduodenal motility in patients with and without HSD/hEDS. We included 239 patients (50 HSD/hEDS and 189 non-HSD/hEDS) with gastrointestinal symptoms undergoing antroduodenal manometry (ADM). The prevalence of antroduodenal dysmotility was not different between both groups, but enteric dysmotility was less common in the HSD/hEDS group (13% vs. 34%, p = 0.006). Delayed gastric emptying rates were similar, suggesting that delayed gastric emptying is more relevant for patients with HSD/hEDS and gastrointestinal symptoms.

Craniocervical instability (CCI) is a condition commonly found in patients with connective tissue disorders such as Ehlers-Danlos Syndrome (EDS), leading to various symptoms. Assessing patients for surgical fusion as a treatment for CCI is challenging due to the complex nature of EDS-related symptoms. This study aimed to evaluate the role of pre-fusion halo-vest traction in alleviating symptoms and determining suitable candidates for fusion surgeries.

EDS patients (n = 21) with neurological symptoms underwent insertion of halo-vest traction between 2019 and 2024. Patients completed a CCI Questionnaire before and after the halo-vest traction, reporting symptoms related to headache, vision, hearing, equilibrium, and function. Symptom groups were assigned scores based on patient responses, with one point for each affirmative answer. Before and after scores were analyzed using paired Student's t-test. Patients experiencing over 50 % improvement in the majority of symptoms were considered for definitive fusion surgery. 16 out of 21 patients subsequently underwent fusion for CCI.

The average age of the patients was 35 years, with a female-to-male ratio of 20:1, consistent with existing literature. Significant improvements were observed in various symptom groups after halo-vest traction, including headache (57 % improvement, p < 0.001), brainstem functions (71 % improvement, p < 0.001), cerebellar functions (55 % improvement, p < 0.001), hearing (63 % improvement, p < 0.001), motor functions (51 % improvement,p < 0.001), vision (60 % improvement, p < 0.001), cardiovascular functions (46 % improvement, p < 0.05), sensory and pain (53 % improvement, p < 0.001), high cortical functions (54 % improvement, p < 0.001), GI functions (52 % improvement, p < 0.05), bladder functions (52 % improvement, p < 0.05), and Modified Karnofsky score (30 % improvement, p < 0.05).

Halo-vest traction proved to be a simple and effective method for evaluating patients for surgery while providing symptomatic relief in EDS-related CCI cases. It allows surgeons to monitor patients with a now post halo stable craniocervical junctions (CCJ) before committing to surgery.

To examine the rate of (1) second anterior cruciate ligament (ACL) rupture (graft rupture or contralateral ACL rupture) and (2) graft rupture alone in patients with generalized joint hypermobility (GJH) at 12 months, at 24 months, and at the longest available time (LAT) following return to sport (RTS) after ACL reconstruction depending on graft choice, that is, hamstring tendon (HT) autograft or bone-patellar tendon-bone (BPTB) autograft.

Data were extracted from a rehabilitation registry that comprised patients who had undergone ACL reconstruction between 2014 and 2022. Patients with GJH aged between 16 and 50 years who had minimum 24-month follow-up following RTS after ACL reconstruction with HT autograft (GJH-HT) or BPTB autograft (GJH-BPTB) were included. A Cox proportional hazard regression model was used to examine the rate of (1) second ACL rupture (graft rupture or contralateral ACL rupture) and (2) graft rupture alone at 12 months, at 24 months, and at the LAT after RTS.

This study included 82 patients (54 in the GJH-HT group and 28 in the GJH-BPTB group), of whom 72.0% were female patients, and the average age was 22.7 ± 7.4 years. The proportion of second ACL ruptures was greater in the GJH-HT group than in the GJH-BPTB group at 12 months (11 of 54 [20.4%] vs 0 of 28 [0%], P = .013), at 24 months (13 of 54 [24.1%] vs 1 of 28 [3.6%], P = .028), and at the LAT (16 of 54 [29.6%] vs 2 of 28 [7.1%], P = .024) after RTS. The rate of second ACL rupture was greater in the GJH-HT group than in the GJH-BPTB (hazard ratio = 4.98, P = .032) at the LAT after RTS. The proportion of patients with graft rupture was greater in the GJH-HT group than in the GJH-BPTB group at 12 months (8 of 54 [14.8%] vs 0 of 28 [0%], P = .046), at 24 months (10 of 54 [18.5%] vs 0 of 28 [0%], P = .013), and at the LAT (12 of 54 [22.2%] vs 0 of 28 [0%], P = .006) after RTS.

An over 4 times greater rate of second ACL rupture after RTS was reported for patients with GJH who underwent ACL reconstruction with HT autograft compared with BPTB autograft. Patients with GJH who were treated with BPTB autograft for ACL reconstruction did not experience graft rupture after RTS.

Level III, retrospective study.

People with joint hypermobility have excessive joint flexibility, which is more common in young women. The people with symptomatic hypermobility report poor balance and even falls. This scoping review aims to identify and map the available evidence related to balance and falling in adults with joint hypermobility to support research planning and ideas for treatment direction.

A framework for the search was constructed using the Joanna Briggs Institute (JBI) approach. Electronic searches of primary evidence were performed using the following databases: Medline, Web of Science, CINAHL, Scopus and EMBASE. Papers written in English and published between 1946 and November 2023 were included. Titles, abstracts, and full papers were independently screened by two reviewers. Data extracted related to the population, the concept under investigation, the method of measurement, the level of evidence and the result.

Nineteen of 1,950 screened articles were included. In terms of the population, details related to ethnicity of the research participants was missing. The hypermobility classification criteria varied; it was not always clear if people who were hypermobile had symptoms. Concepts related to fear of falling, balance and adaptive strategies prompted by different postural tasks with and without vision were explored. Centre of pressure, muscle activity, kinematics and psychological factors were measured using force plates, electromyography (EMG), motion capture, patient and clinician reported outcome measures, focus groups and interviews. Most papers were low on the hierarchy of evidence (i.e. equal or lower than a case control study). The participants with joint hypermobility had increased sway, different muscle activity, and different kinematics compared to people without hypermobility. When surveyed, they commonly fell and had a fear of falling.

It is unclear whether the participants represent the population of people with hypermobility. Different classification systems were used making it difficult to generalise the results. Although the methods used suggest a low level of evidence, it seems clear that people who are hypermobile have poor balance. The underlying mechanisms driving poor balance have not been explored in depth.

Joint hypermobility (JH) is mobility beyond the normal range of motion. JH can be an isolated finding or a characteristic of a syndrome. Characteristics related to the sitting position with atypical body positions, such as sitting in splits (S), with the foot on the head (F), in W (W), in a concave shape (C), episodes of dislocations, and subluxations, suggest impacts on body mechanics since childhood, with damage to the conformation of the joints.

Identify preclinical signs of JH, in addition to Beighton Score (BS), through signs that are easily recognized early by pediatricians and family members to avoid possible joint deformities in the future.

The medical records of 124 children (59.7% girls) between one and nine years old were analyzed. JH was assessed using the BS, a history of luxations/subluxations, and the concave (C), "W", "splits" (S), and foot (F) on head sitting positions.

The concave sitting position was the most common, followed by W, F, and S in decreasing order. A total of 52.4% of the children had BS > 6, with a higher prevalence among girls (60.8%) compared to boys (40.0%); a difference statistically significant (p = 0.024, Fisher's exact test). Thirty-two patients (27.4%) had luxations/subluxations with the higher scores.

Sitting in S, F, W, and C positions are preclinical phenotypic characteristics of JH, easily identified by pediatricians and family members to prevent possible joint deformities. BS ≥ 6 is more frequently observed in all positions. The majority of the total sample has BS > 6, with a significant female gender influence. Among those with a history of occasional joint dislocations and subluxations, half of them have the highest BS scores.

We aimed to assess the typical experiences, desired outcomes, satisfaction with clinical and anticipated outcomes, and the importance of improvements for individuals with Hypermobile Ehlers-Danlos Syndrome (hEDS) and Generalized Hypermobility Spectrum Disorder (G-HSD). A cross-sectional survey was conducted among adults aged 18 and above with hEDS and G-HSD. The survey included the Patient-Centered Outcome Questionnaire and an adapted version addressing common concerns in these individuals. Descriptive statistics were used for analysis. The survey received 483 responses with an 82% completion rate. Most respondents were females (90%), aged 21-30 (30%), living in North America (76%), and diagnosed with hEDS (80%). Participants diagnosed with hEDS reported higher typical levels of pain compared to those diagnosed with G-HSD and higher expected levels of pain and interference with daily activities post-treatment (p < 0.05). The areas of most significant concern were pain, fatigue, interference with daily activities, and walking issues. Our findings revealed no differences in how individuals from both groups rated their treatment expectations, except for the usual pain level and the expected pain level and interference with daily activities post-treatment. Patients' perspectives are essential for developing appropriate treatment plans and improving outcomes for this patient population. Our results will hopefully inform the development of new interventions to impact outcomes that matter to individuals with hEDS and G-HSD.

Hypermobile Ehlers-Danlos syndrome (hEDS) and joint hypermobility syndrome (JHS), among other conditions, comprise a collection of heritable disorders of connective tissue. There are recognized challenges in diagnosing JHS/hEDS. Despite a lack of identifiable molecular etiology for these conditions, referrals to medical geneticists for evaluation are commonplace, and they continue to rise. Because of an absence of nationally recognized referral guidelines for JHS/hEDS, health care institutions are left to develop their own policies. The purpose of our study was to characterize these ad hoc policies systematically and at a nationwide level.

We conducted a mixed-methods study of 71 board-eligible or board-certified genetic counselors, including 15 qualitative interviews.

Cross-case analysis revealed multiple motivations for creating these policies, methods to more effectively manage referrals for hypermobility and concern for EDS, and participants' evaluations of the successes and shortcomings of these policies at their institutions. We found diverse and unstandardized policies that were meant to address numerous perceived challenges. This lack of standardization is a concern because it may result in inconsistent access to care for patients with JHS/hEDS and create barriers to diagnosis and treatment.

Our findings demonstrate that policies vary widely, and genetic counselors are concerned about the potential impact of this variability on the quality of care for patients with JHS/hEDS.

Sow lameness results in premature culling, causing economic loss and well-being issues. A study, utilizing a pressure-sensing mat (GAIT4) and video monitoring system (NUtrack), was conducted to identify objective measurements on gilts that are predictive of future lameness. Gilts (N = 3,656) were categorized to describe their lifetime soundness: SOUND, retained for breeding with no detected mobility issues; LAME_SOW, retained for breeding and detected lame as a sow; CULL_STR, not retained due to poor leg structure; LAME_GILT, not retained due to visible signs of lameness; and CULL, not retained due to reasons other than leg structure. The GAIT4 system creates a series of measurements for each hoof and a lameness score (GLS) while NUtrack records animal movement and posture durations each day. To determine if measurements from the GAIT4 and NUtrack systems were associated with lifetime soundness, mixed model analyses were conducted in R including fixed effects of breed of sire, contemporary group and lifetime soundness score, and random effect of animal. A second mixed model was run without lifetime soundness score and estimates of animal effects were then used to conduct ssGBLUP analyses using three generations of pedigree and genotypes from ~50k SNP on > 60% of phenotyped animals. Genomic heritabilities were estimated, SNP effects were back-solved and significance was based on Bonferroni-corrected permutation tests. GAIT4 traits indicative of lameness (LAME_GILT and CULL_STR vs. SOUND; P < 0.05) were the standard deviation of GLS, average stride length, and average stance time, while significant NUtrack measurements were eating, standing, lateral lying, total lying, speed, distance, and rotations. In addition, rotations differed (P < 0.05) between SOUND vs. LAME_SOW and distance tended to be different (P < 0.10). Estimates of heritability for predictive NUtrack traits were ~0.3 and GAIT4 traits were ~0.2. There were 382 significant SNP effects in 47 genomic regions, four regions on chromosomes 1, 4, 11, and 14 accounted for over 60% of the associations. Genome-level imputed genotypes linked several regions with possible causative genes. Objective measurements from the GAIT4 and NUtrack systems at 5 mo of age were heritable, able to detect unsound animals, and were associated with lifetime soundness.

Generalized Joint Hypermobility (GJH) offers flexibility that could enhance motor activities. However, if it leads to injury and pain, it increases functional difficulties and activity limitations. The far-reaching consequences of activity limitations and restricted participation include poor physical fitness and diminished quality of life. This study investigated whether variations in joint mobility are associated with physical activity levels, physical fitness, and overall quality of life (QoL) among children and whether these factors change over a 2-year period.

One hundred and sixty-five school-aged children were recruited at the beginning of a two-year longitudinal study. One hundred and eleven children were measured three times at one-year intervals. Joint mobility was classified as normal mobile, mobile, or hypermobile. The children were administered the FACES pain scale, the child activity limitation interview, a physical activity questionnaire, and the pediatric quality of life inventory questionnaire. Additionally, the 20-meter shuttle run was used to estimate aerobic fitness.

In this study, pain was unrelated to joint mobility. The activity limitations of our study population were not different at baseline or at the end of the study, irrespective of joint mobility. Children with GJH had significantly lower physical activity levels at the end of the study. Overall, QoL increased over time, and aerobic capacity decreased. However, changes in children with GJH were not significantly different from those in children with normal mobility in this respect.

Children with GJH were moderately active, however significantly less than children with normal mobility. Joint mobility had no significant effect on activity limitations, physical fitness or QoL.

Whether non-syndromic connective tissue hyperlaxity is associated with myopia is unknown. The aim of this study was to examine the association between systemic signs of tissue hyperlaxity and myopia among adolescents.

Included were adolescents assessed before mandatory military service at the age of 16-18 years between 2011 and 2022. Diagnoses of hernias, pes planus, genu varus, genu valgum, and scoliosis, as well as joint injuries were used as surrogate markers for tissue hyperlaxity. The prevalence of these events among adolescents with myopia was evaluated and compared to the non-myopic population.

Included were 920 806 adolescents. The mean age was 17.4 ± 1.4 years and 58.6% were men. Myopia was diagnosed in 290 759 adolescents (31.6%) and high myopia in 24 069 adolescents (2.6%). The prevalence of hernias was higher among adolescents with myopia, (2.76%, 95% confidence interval (95% CI): 2.69%-2.82% vs. 2.60%, 95% CI: 2.57%-2.65%), as were pes planus (14.92%, 95% CI: 14.79-15.05 vs. 13.51%, 95% CI: 13.42-13.59) and scoliosis (9.14%, 95% CI: 9.03-9.24 vs. 7.69%, 95%CI: 7.62-7.76). The prevalence of joint injuries was clinically similar between groups (less than 0.1% difference for ankle, shoulder, and knee injuries), as were genu varum and genu valgum (0.66%, 95%CI: 0.64%-0.69% vs. 0.68%, 95% CI: 0.66-0.70, respectively). Adjusted for possible confounders results remained consistent.

Among a large sample of Israeli adolescents, those with myopia had a higher prevalence of hernias, pes planus, and scoliosis. These results could suggest a propensity for systemic conditions related to tissue laxity among myopic adolescents.

Pain is a very common complaint among patients with hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSDs). Often challenging to treat, insights into the underpinnings of pain in this population have been fleeting. Central sensitization (CS) has been postulated as a potential etiological factor.

In this retrospective study, 82 consecutive patients with hEDS/HSDs were reviewed. Demographic information and Central Sensitization Inventory (CSI) results were collected.

71 of 82 (86.5%) patients demonstrated CS. Scores ranged from 12 to 94 with a median of 56. Pain scores as measured on the numerical rating scale (NRS) ranged from 2 to 10 with a mean and median of 6.

A large percentage of patients with pain and a diagnosis of hEDS/HSDs demonstrated evidence of central sensitization as measured using the CSI. The CSI is simple to administer. The CSI may provide clinical insights that are key to successfully managing patients with hEDS/HSDs. Further research is needed to explore the ability to classify pain phenotypes in this patient population and the impact on precision medicine.

Diagnosing hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD), common overlapping multisystemic conditions featuring symptomatic joint hypermobility, is challenging due to lack of established causes and diagnostic tools. Currently, the 2017 diagnostic criteria for hEDS are used, with non-qualifying cases classified as HSD, although the distinction remains debated. We previously showed extracellular matrix (ECM) disorganization in both hEDS and HSD dermal fibroblasts involving fibronectin (FN), type I collagen (COLLI), and tenascin (TN), with matrix metalloproteinase-generated fragments in conditioned media. Here, we investigated these fragments in patient plasma using Western blotting across diverse cohorts, including patients with hEDS, HSD, classical EDS (cEDS), vascular EDS (vEDS), rheumatoid arthritis (RA), psoriatic arthritis (PsA), and osteoarthritis (OA), and healthy donors, uncovering distinctive patterns. Notably, hEDS/HSD displayed a shared FN and COLLI fragment signature, supporting their classification as a single disorder and prompting reconsideration of the hEDS criteria. Our results hold the promise for the first blood test for diagnosing hEDS/HSD, present insights into the pathomechanisms, and open the door for therapeutic trials focused on restoring ECM homeostasis using an objective marker. Additionally, our findings offer potential biomarkers also for OA, RA, and PsA, advancing diagnostic and therapeutic strategies in these prevalent joint diseases.

After diagnosis of Ehlers Danlos Syndrome (EDS), it is unclear what information patients and parents need and understand about EDS. The objective of this study is to characterize patient and parent knowledge and concerns about EDS after a diagnosis of EDS is made to determine patient and parent concerns and identify barriers that cause discomfort with the diagnosis.6 METHODS: A convenience sample of patient and parent dyads were recruited after new diagnosis of EDS. Patients and parents completed questionnaires that assessed knowledge, comfort, and barriers of EDS before and after diagnosis, EDS education materials accessed, and additional clinical needs and concerns.

Seventy-two dyads completed the survey.

Many respondents actively seek information on the diagnosis and management of EDS. Parents and patients look for information about EDS differently. Parents have more concerns after diagnosis and both want well-constructed, empirically supported educational materials delivered via multiple modalities, which makes clinical guidelines more essential.

Background/Objectives: Generalized joint hypermobility (GJH) is a common condition characterized by an increased range of motion across multiple joints. Previous studies have suggested a possible association between GJH and temporomandibular disorders (TMDs). This study aimed to assess the prevalence of GJH in a Portuguese population of young university adults and to explore the relationship between GJH, temporomandibular joint (TMJ) symptoms/clinical findings, chronic painful TMDs, and chronic painful TMDs subtypes (myalgia, arthralgia, or combined myalgia and arthralgia). Methods: A cross-sectional study was carried out in Oporto university institutions, involving 1249 students (18-25 years). GJH was assessed using the Beighton score cut-off ≥ 4. TMJ symptoms and clinical findings were collected using the Research Diagnostic Criteria for TMD protocol, as well as TMD diagnoses. Univariate and multivariate analyses were carried out to examine the associations between GJH and the variables of interest. Results: The overall prevalence of GJH was 41.9%, with females exhibiting a significantly higher likelihood of GJH (p < 0.001). A statistically significant association was found between GJH and TMJ clicking (p < 0.05). Although no overall association was found between GJH and chronic painful TMDs, GJH was significantly associated with the combined diagnosis of myalgia and arthralgia (p < 0.05). Conclusions: The results suggest that GJH may be associated with the more complex subtypes of chronic painful TMDs. However, due to the small size effect of this association, future longitudinal studies with large samples using GJH broader diagnostic criteria are essential to elucidate the relationship between GJH and painful TMDs in asymptomatic nonsyndromic joint hypermobility populations.

Ehlers-Danlos syndromes (EDS) represent a group of heritable connective tissue disorders characterized by skin hyperelasticity, joint hypermobility and generalized tissue fragility. Many patients remain undiagnosed years after initial symptoms and an accurate diagnosis is difficult despite all efforts. Currently, Germany lacks a patient registry and a specialized EDS centre.

In early 2020, a dermatological-orthopaedic EDS outpatient service was established at the University Hospital of Cologne. Medical records of all patients presenting in 2020 were retrospectively analysed.

Forty-three adults were examined. Fifteen patients were diagnosed with EDS (different types), 13 with hypermobility spectrum disorder, and 1 with likely Loeys-Dietz syndrome (LDS) based on patient history and a suspicious variant in the gene TGFBR1. Excluding hypermobile EDS (6 patients), molecular confirmation was achieved in a total of 4 of 9 patients. The combination of symptomatic generalized hypermobility and skin manifestations was diagnostic in more than two-thirds of the EDS patients. Arterial involvement (aneurysms, dissection and rupture) and distinctive cutaneous signs (thin translucent skin with haematomas) indicated vascular EDS and LDS in altogether 3 patients.

With the present analysis, we discuss our diagnostic approach in patients with a suspected diagnosis of EDS in order to raise awareness of this rare group of genodermatoses and review recent developments in EDS nosology.

Ehlers-Danlos syndrome (EDS) and Joint Hypermobility syndrome (JHS) are still poorly understood, with a prevalence of 1/5000 for EDS and 1/500 for JHS. They are characterized by multisystem involvement. Urological involvement has been little studied. The aim was to carry out a review of the literature on the urological involvement of EDS and JHS.

A review of the literature was carried out using the following databases: Pubmed, Canadian Hospitals and EMBASE. Search terms were "Ehlers-Danlos" or "Joint Hypermobility" associated with "Urology", "Bladder", "Pelvic Organ Prolapse", "Urinary Retention", "Leak", "Leakage", "Urinary Incontinence", "Urinary Tract Infection" and "Urdodynamic", no filters were added.

Seventy-three articles were included for a total of 259 found. The prevalence of urinary incontinence in EDS is estimated at 50-60%, and that of pelvic organ prolapse (POP) at 29-75%. Bladder diverticula are also frequently reported. For JHS, the prevalence of urinary incontinence is estimated between 40 and 73%, that of POP increased with 73% of stage greater than 2, g-JHS patients are almost 3 times more affected by prolapses (OR=2.37) which seem more severe. Patients with vesicoureteral reflux, most often severe, are more affected by joint hypermobility (OR=2.79). Few studies have been carried out on urological assessment and treatment modalities.

EDS patients often have urinary incontinence, pelvic organ prolapse or bladder diverticula. JHS patients frequently have urinary incontinence, pelvic organ prolapse and vesicoureteral reflux.

Vascular Ehlers-Danlos, Marfan and Loeys-Dietz syndromes have increased risk of aortic dilation and dissection. Previous early studies showed hypermobile Ehlers-Danlos syndrome (hEDS) may also have increased risk, with echocardiography screening recommended; subsequent studies have not confirmed the risk or recommended echocardiography. This pediatric-based study assessed aortic dilation prevalence in those with hEDS by serial echocardiographic examinations and assessed family history for aortic dissections. We retrospectively identified individuals with hEDS who had echocardiography studies from the electronic medical records at one pediatric center. Aortic root Z-scores >2.0 were found in 15/225 subjects (average age 12.9 years) on initial echocardiograms, with no Z-score >3.0. Subsequent studies (n = 68) found statistically significant decline in aortic root Z-scores. Repeat echocardiography in those with initial aortic root Z-score >2.0 (n = 10) demonstrated a decline in Z score <2.0 in seven. On final examination, 9/225 (4.0%) had a Z-score >2.0, not statistically different from the general population. No aortic dissection occurred in first- or second-degree relatives. In conclusion, aortic root dilation rate in hEDS is likely not different from the general population. We propose that in the absence of other cardiac findings or suspicion for another disorder, echocardiography is not required in hEDS.

This study was conducted to investigate the relationship between kinesthesia and motor performance in young adults with generalized joint hypermobility (GJH). A systematic review was conducted in accordance with the PRISMA guidelines. PubMed, Embase, Cochrane Library, Web of Science, PsycINFO, CINAHL, Scopus, and Google Scholar were scoured to select relevant papers. Ten papers were selected for the review. The study found no significant impact of GJH on the kinesthetic domains. Several studies suggested varying impacts of GJH on motor performance, with some indicating potential benefits and others indicating possible detriments. Interestingly, the impact of GJH on motor performance was found to be influenced by other factors such as fitness, body mass index, and the use of specific assessment tools. The results suggest that GJH does not significantly impact motor performance in young adults. However, the relationship between GJH and motor performance appears to be complex and may be influenced by several other factors.

Hypermobility spectrum disorders (HSD) and Ehlers-Danlos syndromes (EDS) are multisystem conditions marked by dysfunctional connective tissue. This feasibility study evaluated a 9-week integrative medicine program in this population.

Using a single-arm study design, adults with HSD or EDS were given recommendations for an anti-inflammatory Mediterranean diet and self-management with additional behavioral and psychosocial support. Preliminary data on feasibility based on recruitment and retention, adherence to the diet, mobile app tracking, changes to perceived well-being via health outcomes, and satisfaction with care were obtained.

Thirteen participants were enrolled within a 4-month timeframe. Eight participants completed the study. Three participants met dietary tracking requirement in at least 4 of 8 intervention weeks and met the macronutrient requirements in at least half of the weeks tracked. No decreases in VAS pain scores after 5 and 9 weeks were noted; however, 62.5% (n = 5) of participants had decreased pain at 9 weeks, compared to baseline. There were significant improvements (p<.05) in six of twelve measurements of satisfaction with care at the end of the intervention.

This study provides a foundation for future research on patient experience and introduces a novel treatment paradigm focused on nutrition and self-management.

Trial Registration: National Institutes of Health clinicaltrials.gov; identifier: NCT04734041.

Hypermobile Ehlers-Danlos syndrome (EDS) is an inherited condition marked by joint hypermobility, instability, chronic pain and fatigue, significantly impacting quality of life and autonomy. Management focuses solely on symptom alleviation. After experiencing a rapid decline in functional abilities, a patient in late adolescence with hypermobile EDS underwent hippotherapy rehabilitation (30 hours). She saw substantial improvements in fatigue, chronic pain and regained walking abilities. Hippotherapy played a crucial role in refining her postural balance, motor skills, proprioception, muscle function and endurance. It also positively affected her cognitive and emotional regulation by stimulating sensory inputs that activate neural pathways, providing relief from pain and fatigue. Notably, the patient transitioned from wheelchair reliance to walking with crutches, and then greatly enhanced both gait quality and speed. These findings demonstrate the relevance of hippotherapy for the rehabilitation of hypermobile EDS, reducing the need for pharmacological interventions and fostering a proactive approach to future challenges.

Hypermobility Spectrum Disorder (HSD) and Hypermobile Ehlers-Danlos Syndrome (hEDS) are two overlapping heritable connective tissue disorders characterized by joint hypermobility, chronic pain, impaired body perception, and musculoskeletal symptoms. Central sensitization has been proposed as a plausible explanation for symptoms like widespread pain, fatigue, mood disorders, and sleep disturbances in patients with HSD/hEDS.

The aim of this study was to investigate signs of central sensitization, including exercise-induced hypoalgesia (EIH), and fatigue severity in adolescents with HSD/hEDS.

In this prospective, experimental, case-control study, thirty-seven adolescents with HSD/hEDS and 47 healthy adolescents (all aged 13-17 years) were included. Pressure pain thresholds (PPTs) were measured at four muscle groups using a pressure algometer. EIH was evaluated by measuring PPTs on two muscle groups immediately after an exercise test on a bicycle ergometer. Participants also completed questionnaires on fatigue and cognitive/emotional factors.

The study demonstrated significantly lower PPTs in four different muscle groups in adolescents with HSD/hEDS compared to the healthy control group. Both groups achieved a significantly higher PPTs after exercise in the muscle involved in the activity. Adolescents with HSD/hEDS reported higher fatigue levels and more cognitive/emotional difficulties than the control group.

Adolescents with HSD/hEDS showed generalized hyperalgesia measured through PPTs at different body sites. EIH was partly affected in adolescents with HSD/hEDS, presenting as unchanged pain sensitivity in the remote muscle. Pain should be considered as a phenomenon that is influenced by different biopsychosocial factors, including possible central sensitization, which increase its complexity.

This study breaks new ground by showing signs of central sensitization, including diminished EIH, in adolescents with HSD or hEDS. Given that exercise is a key element in pain management, these findings offer valuable insights when developing treatment plans for adolescents with HSD or hEDS.

Extra-articular symptoms, including headaches, are frequently encountered in patients with Ehlers-Danlos syndrome (EDS) and hypermobility spectrum disorders (HSD), and may be the presenting complaint. Migraine is reported in up to three quarters of patients with symptomatic joint hypermobility, have a higher headache frequency, and an earlier age of onset compared to the general population. Orthostatic headache is an important presentation, and should raise suspicion of an underlying spinal cerebrospinal fluid leak, dysautonomia, and craniocervical pathology, which are all associated with heritable connective tissue disorders (HCTD) including EDS. Any proposed invasive procedure should be scrupulously balanced against its potential risks, taking into account the type of EDS (e.g., vascular EDS) and its systemic manifestations. This is particularly pertinent when suspecting craniocervical instability since it remains a controversial diagnosis with a limited treatment evidence-base. This article reviews the commonly encountered headache disorders in patients with joint hypermobility-related conditions with a focus on EDS and HSD, describes their diverse presentations, and an overview of the recommended management strategies. It also emphasises the need for increased awareness of comorbid conditions in EDS and HSD among clinicians treating headaches to ensure a patient-tailored approach and facilitate a multidisciplinary approach in managing often complex cases.

People with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSDs) are at greater risk of developing temporomandibular disorders (TMDs), perhaps due to the general joint hypermobility. There is, however, no information on how oral health-related quality of life (OHRQoL) is affected in people with hEDS or HSD with TMD. The authors' aim was to assess OHRQoL via the 14-item, short version Oral Health Impact Profile (OHIP-14), as well as associated risk factors in women with TMD symptoms and confirmed hEDS or HSD.

A digital questionnaire was sent to members of The Swedish National EDS Association who reported having a confirmed or suspected EDS or HSD diagnosis in the health care system from January through March 2022. Then, a sample of 133 women with confirmed hEDS or HSD and TMD symptoms was constructed, and information on the following variables was collected: TMD symptoms, age, general health, oral health-related factors, comorbid symptoms, and psychological factors. Linear regression analysis was conducted to investigate the association between these variables and the OHIP-14 summary score as the outcome.

Most participants reported TMD pain symptoms (93.9%), temporomandibular joint clicking (89.5%), and crepitation (55.6%). The mean (SD) total OHIP-14 summary score was 21.0 (13.2). Oral function had the lowest impact (2.0 [2.4]) and orofacial pain had the highest impact on OHRQoL (3.9 [2.5]). Self-reported bruxism, poor general health, and comorbid symptoms were significantly associated with impaired OHRQoL.

Women with confirmed hEDS or HSD and TMD symptoms have a considerably impaired OHRQoL.

The multidimensional phenomenon of OHRQoL in this group needs to be considered in management strategies.

Patients with joint-hypermobility and joint-hypermobility spectrum disorders (HSD), including hypermobile Ehlers-Danlos Syndromes (EDS) present numerous co-morbid concerns, and multidisciplinary care has been recommended. The complexity of these patient's needs and increased demand for medical services have resulted in long delays for diagnosis and treatment and exhausted extant clinical resources. Strategies must be considered to ensure patient needs are met in a timely fashion. This opinion piece discusses several potential models of care for joint-hypermobility disorders, several ways in which primary providers can be involved, and argues that primary providers should be an essential and integrated part of the management of these patients, in collaboration with multidisciplinary teams and pediatric subspecialists. We review several strategies and educational opportunities that may better incorporate primary providers into the care and management of these patients, and we also discuss some of the limitations and barriers that need to be addressed to improve provision of care. This includes establishing primary care physicians as the medical home, providing initial diagnostic and treatment referrals while connecting patients with specialty care, and collaboration and coordination with multi-disciplinary teams for more complex needs. Several barriers exist that may hamper these efforts, including a lack of available specialty trainings for providers interested in providing care to patients with EDS and HSD, a lack of expertly derived consensus guidelines, and limited time resources in extant primary care practices. Also, primary providers should have an active voice in the future for the further consideration and development of these presented strategies.

Clinical features of hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility syndrome (HMS) have classically focused on dysfunctions related to the musculoskeletal system. A growing body of literature suggests substantial multisystemic involvement, although this has not been recapitulated in a pediatric/young adult population. Leveraging a large United States healthcare claim database illuminates multisystem disorders among patients diagnosed with hEDS and HMS in the age range of 10 to 24. This was a retrospective review of patient records within the de-identified healthcare claims database, PearlDiver. Patients with a diagnosis of hEDS or HMS, and those without these diagnoses who were seen for their annual physical examination, between the ages of 10 and 24, were queried for the presence of additional medical conditions. Descriptive statistics were used to define the frequency of multisystem diagnoses. Nineteen thousand seven hundred ninety hEDS patients, 17,509 HMS patients, and 4,959,713 patients from the general population were analyzed. Within 2 years following hEDS or HMS diagnosis, digestive disorders were the most prevalent diagnosis, followed by cardiovascular conditions. Digestive disorders occurred in 54.6% of patients with hEDS and 41.6% of patients with HMS, compared to 28.5% of the general population. Cardiovascular disorders occurred in 43.6% of patients with hEDS and 21.8% of patients with HMS compared to 10.3% of the general population. Anxiety, respiratory disorders, and developmental disorders occurred in approximately 25% of the hEDS group and 20% of the HMS group, compared to ~15% of the general population, all statistically significantly higher in the hEDS and HMS groups. This study highlights multisystem diagnoses within the pediatric hEDS/HMS populations. hEDS patients had higher rates of multisystem diagnoses compared to HMS patients. These results suggest a high multisystem disease burden for young hEDS/HMS patients. Future research is needed to understand the timing and presentation of clinical symptoms for this population.

To assess the impact of a Physiotherapist-led Pilates Intervention for school aged children with Generalised Joint Hypermobility (GJH) on pain, physical function and Health Related Quality of Life (HRQoL).

Three children aged 8-12 years with GJH participated in an 8 week Physiotherapist-led Pilates Intervention within a single-case experimental design (multiple baseline design). Repeated measures were collected during baseline, intervention, withdrawal and follow-up, for: (i) pain, (ii) physical function as measured by muscle strength, postural control, fatigue and activity levels and (iii) HRQoL.

Within the intervention phase, two children showed reduced fatigue and one child improved in muscle group strength of hip abduction (gluteus medius) and scapula adduction/rotation (rhomboideus major/minor) and HRQoL. No improvements were seen in pain or postural control. Within the early withdrawal phase all children showed improved strength for at least two muscle groups and one child showed reduced fatigue, pain (worst in last week) and improved postural control (functional reach lateral).

Pilates may provide an effective intervention for children with GJH to reduce fatigue and improve muscle strength and HRQoL. Limited conclusions can be made regarding pain and postural control. Further research with a longer Pilates duration is needed to confirm the dose and benefits for this population.

In the realm of research, the single case study has been recognized as a valuable tool for sharing insights, demonstrating new concepts, discovering novel phenomena, consolidating hypotheses, and sparking original ideas. In this physician-guided narrative, phenomena previously unreported in the clinical context are explored. These case studies aim to offer insights that may inform an existing theoretical model that encapsulates a distinct therapeutic intervention. Original research in fascia-focused therapies presents many challenges, including the lack of universal terminology, inconsistent techniques, and difficulties in quantifying treatment effects. Tensegrity-based approaches, which concentrate on tissue tension, also face challenges in establishing their validity within living organisms. For centuries, fascia was seen only as ribbons and sheets of soft, inert, mostly fatty tissue. Consequently, most anatomy textbooks provide a sterile view of anatomical structures devoid of the context of unifying fascia. Now, however, consensus of research describes fascia as an omnipresent, ubiquitous, body-wide tissue that acts as a system, transmitting mechanical information via tensional force changes.

The clinical outcomes in the cases provided in this narrative report suggest that induction of thigh fascia resulted in immediate anatomically distant therapeutic benefit, as reported by the patients.

Rather than measuring treatment efficacy, these two case studies utilize a specific myofascial induction performed at high velocity to elucidate the possibility of a fascia-based, tensegrity-mediated, mechanism for commonly managed, yet often incurable conditions - Benign Paroxysmal Positional Vertigo (BPPV) symptoms and Temporomandibular Joint (TMJ) pain (within the spectrum of Temporomandibular Disorder (TMD)).

Anterior cruciate ligament (ACL) injuries continue to increase in incidence despite extensive research into prevention strategies. Many extrinsic and intrinsic risk factors for sustaining ACL injuries have been identified and continue to be investigated. Extrinsic risk factors for ACL injury relate to the athlete's environment, such as the shoe-surface interaction, weather conditions, and sport played. Intrinsic risk factors relate to the athlete's sex, hormones, knee anatomy, landing and pivoting biomechanics, and neuromuscular control. Recent research has highlighted the role of the bony morphology of the proximal tibia and distal femur on primary ACL injury risk, as well as the risk for ACL graft failure. Sex differences in bony and ligamentous morphology of the knee, neuromuscular control, and hormonal factors, such as serum relaxin levels and variations within the menstrual cycle, have been correlated with a higher risk of noncontact primary ACL injuries in female athletes compared with male athletes.

Hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) are the most common joint hypermobility conditions encountered by physicians, with hypermobile and classical EDS accounting for >90% of all cases. Hypermobility has been detected in up to 30-57% of patients with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), fibromyalgia, postural orthostatic tachycardia syndrome (POTS), and long COVID (LC) compared to the general population. Extrapulmonary symptoms, including musculoskeletal pain, dysautonomia disorders, cognitive disorders, and fatigue, are seen in both LC and HSD. Additionally, ME/CFS has overlapping symptoms with those seen in HSD. Mast cell activation and degranulation occurring in both LC and ME/CFS may result in hyperinflammation and damage to connective tissue in these patients, thereby inducing hypermobility. Persistent inflammation may result in the development or worsening of HSD. Hence, screening for hypermobility and other related conditions including fibromyalgia, POTS, ME/CFS, chronic pain conditions, joint pain, and myalgia is essential for individuals experiencing LC. Pharmacological treatments should be symptom-focused and geared to a patient's presentation. Paced exercise, massage, yoga, and meditation may also provide benefits.

Hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) are often accompanied by varied and complex multisystemic comorbid symptoms/conditions. The Spider questionnaire was developed to evaluate the presence and impact of eight common multisystemic comorbidities. Thirty-one questions across eight symptom domains assess neuromusculoskeletal, pain, fatigue, cardiac dysautonomia, urogenital, gastrointestinal, anxiety, and depression symptoms. This study aimed to evaluate the Spider's construct validity in adults.

A cross-sectional observational study was conducted over four stages. Three international patient charities aided recruitment of participants through social media and website advertisements. Adults aged 18 to 65 years, with and without HSD/hEDS, were invited to participate. Validated, frequently used comparator questionnaires were used to establish convergent validity of Spider symptom domains. A control group was recruited for known-group validity analysis. Participants answered each Spider domain and the corresponding comparator questionnaire via surveys hosted by REDCap. Anonymous data were analysed using SPSS. Convergent validity was assessed through Spearman's correlational analysis and known-group validity through Mann-Whitney U analysis.

A total of 11,151 participants were recruited across the four stages. Statistically significant, moderate-to-strong correlations were found between all Spider domains and their comparators (p < 0.001, r = 0.63 to 0.80). Known-group validity analysis showed statistically significant differences (p < 0.001) between the hypermobile and control groups in all eight domains.

Convergent and known-group validity of the Spider was established with adults. These results suggest the Spider can measure the presence and impact of multisystemic comorbid symptoms/conditions in adults with HSD/hEDS, providing a tool which guides multidisciplinary management. Key Points • The Spider questionnaire is a novel tool assessing the presence and impact of the multisystemic comorbid symptoms/conditions associated with HSD/hEDS. • Convergent and known-group validity of the Spider questionnaire was established in adults aged 18 to 65. • This tool provides a quick and easy method to visualise the symptom profile of those with HSD/hEDS to guide symptom management.