SynthesisFrontiers in medicine2023
Extracutaneous features and complications of the Ehlers-Danlos syndromes: A systematic review.
Synthesis in Frontiers in medicine, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 25 citations in OpenAlex.
- Prevalence of vascular complications in Ehlers-Danlos syndrome: a systematic review and meta-analysis.Orphanet journal of rare diseases · 2025Pooled it
- Gastrointestinal involvement in Ehlers-Danlos syndrome classical-like type 2 associated with a novel AEBP1 splice-site variant.Journal of human genetics · 2026Article
- Cases - Ehlers-Danlos syndromes: Review on the urologic implications.Canadian Urological Association journal = Journal de l'Association des urologues du Canada · 2026Article
- A Nurse's Perspective Shaped by Illness: What Becoming the Zebra Revealed.Journal of patient experience · 2026Article
- Assessment of Collagen and Fibroblast Properties via Label-Free Higher Harmonic Generation Microscopy in Three-Dimensional Models of Osteogenesis Imperfecta and Ehlers-Danlos Syndrome.International journal of molecular sciences · 2025Article
- Cervical Oculopathy: The Cervical Spine Etiology of Visual Symptoms and Eye Diseases-A Hypothesis Exploring Mechanisms Linking the Neck and the Eye.Diagnostics (Basel, Switzerland) · 2025Article
- Complex Wound Reconstruction in a Patient With Ehlers-Danlos Syndrome.Plastic and reconstructive surgery. Global open · 2025Article
- Prevalence of Pain and Effect of Joint Strength on Perceived Physical Function in Pediatric Patients With Ehlers-Danlos Syndrome.Archives of rehabilitation research and clinical translation · 2025Article
- Neurobiological and neuropsychological disturbance in EDS.Frontiers in neurology · 2025Review
- Investigating the association between osteopenia and bowel perforation through a multicenter radiologic analysis.Scientific reports · 2024Article
- Syndromic scoliosis in a patient with arthrochalasia Ehlers-Danlos syndrome corrected with a Wood-Rigo-Cheneau derotational brace.BMJ case reports · 2024Article
- Functional benefit of joint surgery in patients with non-vascular Ehlers-Danlos syndrome: results of a retrospective study.Orphanet journal of rare diseases · 2024Article
- Surgical and irradiated case of early breast cancer in a patient with Ehlers-Danlos syndrome.Surgical case reports · 2024Article
- Headache disorders in patients with Ehlers-Danlos syndromes and hypermobility spectrum disorders.Frontiers in neurology · 2024Review
- Article
- The Effectiveness of Compression Garments for Reducing Pain in Non-Vascular Ehlers-Danlos Syndromes: A Prospective Observational Cohort Study.Healthcare (Basel, Switzerland) · 2023Article
- Barriers to the Diagnosis, Care, and Management of Pediatric Patients With Ehlers-Danlos Syndrome in the United States: A Qualitative Analysis.Global pediatric health · 2023Article
Corrections and comments
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Authors and funding
8 authors at 8 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Introduction: The Ehlers-Danlos syndromes (EDS) comprise a group of inherited connective tissue disorders presenting with variable fragility to skin, soft tissue, and certain internal organs, which can cause significant complications, particularly arterial rupture, bowel perforation and joint difficulties. Currently, there are 14 proposed subtypes of EDS, with all except one subtype (hypermobile EDS) having an identified genetic etiology. An understanding of the extracutaneous features and complications within each subtype is key to maximizing clinical care and reducing the risk of further complications. Methods: A systematic review of EDS-related extracutaneous features and complications was undertaken. Results: We identified 839 EDS cases that met the inclusion criteria. We noted a high prevalence of joint hypermobility amongst kyphoscoliotic (39/39, 100%), spondylodysplastic (24/25, 96.0%), and hypermobile (153/160, 95.6%) EDS subtypes. The most common musculoskeletal complications were decreased bone density (39/43, 90.7%), joint pain (217/270, 80.4%), and hypotonia/weakness (79/140, 56.4%). Vascular EDS presented with cerebrovascular events (25/153, 16.3%), aneurysm (77/245, 31.4%), arterial dissection/rupture (89/250, 35.5%), and pneumothorax/hemothorax. Chronic pain was the most common miscellaneous complication, disproportionately affecting hypermobile EDS patients (139/157, 88.5%). Hypermobile EDS cases also presented with chronic fatigue (61/63, 96.8%) and gastrointestinal complications (57/63, 90.5%). Neuropsychiatric complications were noted in almost all subtypes. Discussion: Understanding the extracutaneous features and complications of each EDS subtype may help diagnose and treat EDS prior to the development of substantial comorbidities and/or additional complications. Systematic review registration: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022308151, identifier CRD42022308151.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.