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      Pelvic Organ Prolapse in Ehlers-Danlos Syndrome

      case-report

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          Abstract

          Ehlers-Danlos syndrome (EDS) is a hereditary tissue and collagen synthesis disorder that can predispose patients to gynecologic and obstetric complications. Female patients often suffer from bothersome pelvic floor disorders, but due to the medical complexity of EDS, special considerations are needed for the treatment of pelvic organ prolapse and associated incontinence. In this paper, we present three unique cases of pelvic organ prolapse (POP) in EDS patients and delve deeper into the multidisciplinary approach involving urogynecology, rheumatology, physiatry, gastroenterology, and anesthesiology required to appropriately manage this condition.

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          Most cited references15

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          The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome

          Mark Scheper, Jane Simmonds, Leslie Russek … (2017)
          Article 0 comments Cited 53 times – based on 0 reviews     Review now
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            Urinary incontinence and pelvic organ prolapse in women with Marfan or Ehlers Danlos syndrome.

            Robert J Schaffer, Meg Carley (2000)
            This study was undertaken to determine the prevalence of urinary incontinence and pelvic organ prolapse in a cohort of women with either Marfan syndrome or Ehlers-Danlos syndrome. Female patients with either Marfan syndrome or Ehlers-Danlos syndrome were identified through a medical records search at two urban hospitals. Each patient's medical record was reviewed, and the history of pelvic organ prolapse and urinary incontinence was obtained through telephone interview. Twelve women with Marfan syndrome were identified. Among these women 5 (42%) reported a history of urinary incontinence and 4 (33%) reported a history of pelvic organ prolapse. Eight women with Ehlers-Danlos syndrome were identified. Among these women 4 (50%) reported a history of urinary incontinence and 6 (75%) reported a history of pelvic organ prolapse. Women with Marfan or Ehlers-Danlos syndrome have high rates of urinary incontinence and pelvic organ prolapse. This finding supports the hypothesized etiologic role of connective tissue disorders as a factor in the pathogenesis of these conditions.
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              Hypermobility and the hypermobility syndrome.

              Jane Simmonds, Rosemary J. Keer (2007)
              Hypermobile joints by definition display a range of movement that is considered excessive, taking into consideration the age, gender and ethnic background of the individual. Joint hypermobility, when associated with symptoms is termed the joint hypermobility syndrome or hypermobility syndrome (JHS). JHS is an under recognised and poorly managed multi-systemic, hereditary connective tissue disorder, often resulting in a great deal of pain and suffering. The condition is more prevalent in females, with symptoms frequently commencing in childhood and continuing on into adult life. This paper provides an overview of JHS and suggested clinical guidelines for both the identification and management of the condition, based on research evidence and clinical experience. The Brighton Criteria and a simple 5-point questionnaire developed by Hakim and Grahame, are both valid tools that can be used clinically and for research to identify the condition. Management of JHS frequently includes; education and lifestyle advice, behaviour modification, manual therapy, taping and bracing, electrotherapy, exercise prescription, functional rehabilitation and collaborative working with a range of medical, health and fitness professionals. Progress is often slow and hampered by physical and emotional setbacks. However with a carefully considered management strategy, amelioration of symptoms and independent functional fitness can be achieved.
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                Author and article information

                Contributors
                Azadeh Nazemi:
                ORCID: https://orcid.org/0000-0002-4265-5705
                Journal
                Journal ID (nlm-ta): Case Rep Urol
                Journal ID (iso-abbrev): Case Rep Urol
                Journal ID (publisher-id): CRIU
                Title: Case Reports in Urology
                Publisher: Hindawi
                ISSN (Print): 2090-696X
                ISSN (Electronic): 2090-6978
                Publication date Collection: 2023
                Publication date (Electronic): 23 February 2023
                Volume: 2023
                Electronic Location Identifier: 6863711
                Affiliations
                Department of Urology, New York University Grossman School of Medicine New York, NY, USA
                Author notes

                Academic Editor: Walid Farhat

                Author information
                https://orcid.org/0000-0002-4265-5705
                Article
                DOI: 10.1155/2023/6863711
                PMC ID: 9981282
                PubMed ID: 36875296
                SO-VID: 7161e1eb-f865-49b9-9aa6-c473195d8126
                Copyright © Copyright © 2023 Azadeh Nazemi et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 10 August 2022
                Date revision received : 5 January 2023
                Date accepted : 9 January 2023
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Urology
                Data availability:
                ScienceOpen disciplines: Urology

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