scholarly journals Pelvic Girdle Pain, Hypermobility Spectrum Disorder and Hypermobility-Type Ehlers-Danlos Syndrome: A Narrative Literature Review

2020 ◽  
Vol 9 (12) ◽  
pp. 3992
Author(s):  
Ahmed Ali ◽  
Paul Andrzejowski ◽  
Nikolaos K. Kanakaris ◽  
Peter V. Giannoudis
Keyword(s):  
Pelvic Ring ◽  
Patient Reported Outcome Measures ◽  
Pelvic Girdle ◽  
Spectrum Disorder ◽  
Pelvic Girdle Pain ◽  
Treatment Modalities ◽  
Ehlers Danlos Syndrome ◽  
Ongoing Research ◽  
Patient Reported ◽  
Danlos Syndrome

Pelvic girdle pain (PGP) refers specifically to musculoskeletal pain localised to the pelvic ring and can be present at its anterior and/or posterior aspects. Causes such as trauma, infection and pregnancy have been well-established, while patients with hypermobile joints are at greater risk of developing PGP. Research exploring this association is limited and of varying quality. In the present study we report on the incidence, pathophysiology, diagnostic and treatment modalities for PGP in patients suffering from Hypermobility Spectrum Disorder (HSD) and Hypermobility-Type Ehlers-Danlos Syndrome (hEDS). Recommendations are made for clinical practice by elaborating on screening, diagnosis and management of such patients to provide a holistic approach to their care. It appears that this cohort of patients are at greater risk particularly of mental health issues. Moreover over, they may require a multidisciplinary approach for their management. Ongoing research is still required to expand our understanding of the relationship between PGP, HSD and hEDS by appropriately diagnosing patients using the latest updated terminologies and by conducting randomised control trials to compare outcomes of interventions using standardised patient reported outcome measures.

scholarly journals Ehlers–Danlos Syndrome and Hypermobility Syndrome Compared with Other Common Chronic Pain Diagnoses—A Study from the Swedish Quality Registry for Pain Rehabilitation

2020 ◽  
Vol 9 (7) ◽  
pp. 2143 ◽  
Author(s):  
Peter Molander ◽  
Mehmed Novo ◽  
Andrea Hållstam ◽  
Monika Löfgren ◽  
Britt-Marie Stålnacke ◽  
...  
Keyword(s):  
Chronic Pain ◽  
Pain Intensity ◽  
Spinal Pain ◽  
Patient Reported Outcome Measures ◽  
Patient Reported Outcome ◽  
Ehlers Danlos Syndrome ◽  
Quality Registry ◽  
Quality Register ◽  
Patient Reported ◽  
Danlos Syndrome

Although chronic pain is common in patients with Ehlers–Danlos syndrome (EDS) and hypermobility syndromes (HMS), little is known about the clinical characteristics of these groups. The main aim was to compare EDS/HMS with common local and generalized pain conditions with respect to Patient Reported Outcome Measures (PROMs). Data from the Swedish Quality Register for Chronic Pain (SQRP) from 2007 to 2016 (n = 40,518) were used, including patients with EDS/HMS (n = 795), fibromyalgia (n = 5791), spinal pain (n = 6693), and whiplash associated disorders (WAD) (n = 1229). No important differences in the PROMs were found between EDS and HMS. Women were represented in > 90% of EDS/HMS cases and fibromyalgia cases, and in about 64% of the other groups. The EDS/HMS group was significantly younger than the others but had a longer pain duration. The pain intensity in EDS/HMS was like those found in spinal pain and WAD; fibromyalgia had the highest pain intensity. Depressive and anxiety symptoms were very similar in the four groups. Vitality—a proxy for fatigue—was low both in EDS/HMS and fibromyalgia. The physical health was lower in EDS/HMS and fibromyalgia than in the two other groups. Patients with EDS/HMS were younger, more often female, and suffered from pain for the longest time compared with patients who had localized/regional pain conditions. Health-care clinicians must be aware of these issues related to EDS/HMS both when assessing the clinical presentations and planning treatment and rehabilitation interventions.

scholarly journals Case series of pubic bone diastasis causing severe pelvic girdle pain in pregnancy

2019 ◽  
Vol 8 (11) ◽  
pp. 4612
Author(s):  
Pooja Gupta ◽  
Renuka Malik
Keyword(s):  
Conservative Management ◽  
Post Partum ◽  
Treatment Guideline ◽  
Case Series ◽  
Pelvic Girdle ◽  
Pelvic Girdle Pain ◽  
Treatment Modalities ◽  
Pubic Bone ◽  
X Rays ◽  
Pubic Symphysis Diastasis

Pelvic girdle pain (PGP) is a pregnancy discomfort that causes pain and limitation of mobility and functioning in any of the three pelvic joints. The patient usually presents antenatally with persistent suprapubic pain which is exaggerated during moving, walking or climbing stairs. Intrapartum, this could be associated with disruption of sacroiliac joint, hematuria and bladder dysfunction in severe cases. Pelvic X-rays, ultrasound, and magnetic resonance imaging aid in confirmation of diagnosis by measuring the degree of separation of symphysis. Treatment modalities range from conservative management to orthopedic interventions in form of pelvic strapping, open reduction and internal fixation. Postpartum pain often masks clinicians to make the diagnosis of pubic symphysis diastasis. A case series of three cases which were diagnosed and confirmed with ultrasound and managed with orthopedic consultation. till their delivery and in post-partum period till recovery. Although there is still no specific consensus on treatment guideline, management generally of conservative management to surgical in the form of pelvic bracing or strapping. Awareness of this rare condition can help in management of pain and associated disability which improves post-delivery.

scholarly journals Sagittal adjusting screws for the correction of grade IV spondylolisthesis in a patient with Ehlers-Danlos syndrome: illustrative case

2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Jake Jasinski ◽  
Doris Tong ◽  
Connor Hanson ◽  
Teck Soo
Keyword(s):  
Lumbar Fusion ◽  
Surgical Techniques ◽  
Foot Drop ◽  
Illustrative Case ◽  
Open Approach ◽  
High Grade ◽  
Ehlers Danlos Syndrome ◽  
Patient Reported ◽  
Lumbosacral Spondylolisthesis ◽  
Danlos Syndrome

BACKGROUND Ehlers-Danlos syndrome (EDS) and its connective tissue laxity often result in high-grade lumbosacral spondylolisthesis. Patients present with debilitating symptoms and neurological deficits. Reports of surgical techniques in non-EDS patients for the treatment of high-grade lumbosacral spondylolisthesis mainly described an open approach, multilevel fusions, and multiple stages with different circumferential approaches. Sagittal adjusting screws (SASs) can be used in a minimally invasive (MI) fashion, allowing intraoperative reduction. OBSERVATIONS A 17-year-old female with EDS presented to the authors’ institute with severe lower back and left L5 radicular pain in 2017. She presented with a left foot drop and difficulty ambulating. Magnetic resonance imaging showed grade IV L5–S1 spondylolisthesis. She underwent lumbar fusion for intractable back pain with radiculopathy. Intraoperatively, percutaneous SASs and extension towers were used to distract the L5–S1 disc space and reduce the spondylolisthesis. MI transforaminal lumbar interbody fusion was completed with significant symptomatic relief postoperatively. The patient was discharged to home 3 days postoperatively. Routine follow-up visits up to 3 years later demonstrated solid fusion radiographically and favorable patient-reported outcomes. LESSONS The authors used SASs in a MI approach to successfully correct and stabilize grade IV spondylolisthesis in an EDS patient with a favorable long-term patient-reported outcome.

scholarly journals A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder

2020 ◽  
Vol 90 (2) ◽  
Author(s):  
Claudia Celletti ◽  
Beatrice Borsellino ◽  
Marco Castori ◽  
Federica Censi ◽  
Giovanni Calcagnini ◽  
...  
Keyword(s):  
Baroreflex Sensitivity ◽  
Orthostatic Intolerance ◽  
Autonomic Regulation ◽  
Spectrum Disorder ◽  
Postural Tachycardia Syndrome ◽  
Management Approach ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Head Up Tilt ◽  
Danlos Syndrome

There is an association between joint hypermobility, hypermobile Ehlers-Danlos syndrome (hEDS) and different forms of orthostatic intolerance. Objective: to explore autonomic profile in a large cohort of adults with hEDS and hypermobility spectrum disorder (hEDS/HSD) with a multimodal approach. In this observational retrospective study, heart rate, blood pressure and baroreflex sensitivity were estimated in 102 hEDS/HSD subjects during deep breathing, Valsalva maneuver, standing up: 30-15 ratio, Head-Up Tilt and sustained handgrip. Abnormal results and head-up tilt test were common and included postural orthostatic tachycardia syndrome (POTS; 48%), orthostatic intolerance (25.5%) and hypotension (3.9%). Baroreflex sensitivity was significantly different in individuals with POTS compared to the others. This study confirms the high rate and heterogeneity of abnormal autonomic regulation in hEDS/HSD, and suggests the baroreflex sensitivity might distinguish comorbid POTS from other profiles in this subgroup of patients. Abnormal autonomic regulation is common in adults with hEDS/HSD and should be regularly assessed for tailoring the management approach.

scholarly journals Eosinophilic Gastroenteritis in a Patient with Ehlers-Danlos Syndrome - A Rare Combination

1992 ◽  
Vol 6 (3) ◽  
pp. 129-132
Author(s):  
I Al-Traif ◽  
L Jewell ◽  
ABR Thomson
Keyword(s):  
Weight Loss ◽  
Abdominal Pain ◽  
Elemental Diet ◽  
Eosinophilic Gastroenteritis ◽  
Treatment Modalities ◽  
Ehlers Danlos Syndrome ◽  
Gastrointestinal Manifestations ◽  
Rare Combination ◽  
Danlos Syndrome

A case of eosinophilic gastroenteritis in a patient with Ehlers-Danlos syndrome who presented with abdominal pain, vomiting, diarrhea and profound weight loss is presented. Because of inability to tolerate glucocorticosteroids, symptoms were treated with sodium chromoglycate and an elemental diet given by jejunostomy. The patient did well on this program. The gastrointestinal manifestations of eosinophilic gastroenteritis and Ehlers-Danlos syndrome are reviewed as well as the treatment modalities available for eosinophilic gastroenteritis.

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