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      Cesarean Delivery in a Patient With Hypermobile Ehlers-Danlos Syndrome: A Case Report

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          Abstract

          Patients with hypermobile Ehlers-Danlos syndrome (hEDS) usually present with generalized joint hypermobility and pain, soft and hyperextensible skin with atrophic scars and easy bruising, periodontitis, mitral valve prolapse, and aortic root dilation. It may also lead to cervical spine instability or collapse of larynx cartilage/trachea, which can result in intubation difficulties and possible mucosal damage. Lung protective ventilation must be performed to prevent pneumothorax. Bruising, scoliosis, spondylosis, meningeal Tarlov cysts, increased risk for postdural puncture headache (PDPH), and resistance to local anesthetics may affect neuraxial anesthesia. This article aims to report an anesthetic approach of a 24-year-old woman with hEDS proposed for cesarean delivery. Additional past medical history included cutaneous psoriasis, polycystic ovarian syndrome, and bilateral hip dysplasia. No previous allergies were reported. She has been previously submitted to an uneventful general anesthesia. After rapid-sequence induction of general anesthesia, endotracheal intubation was performed with videolaryngoscopy. Balanced anesthesia with volatile anesthetics was used. The birth and recovery were safely managed without adverse events. hEDS can present challenges for both neuraxial anesthesia and orotracheal intubation. In this case, the initial anesthetic plan included general anesthesia and an airway approach with a videolaryngoscope. Patient positioning and padding were carefully executed to prevent bruising and joint dislocations.

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          Psychiatric and psychological aspects in the Ehlers-Danlos syndromes.

          There is increasing amount of evidence pointing toward a high prevalence of psychiatric conditions among individuals with hypermobile type of Ehlers-Danlos syndrome (JHS/hEDS). A literature review confirms a strong association between anxiety disorders and JHSh/hEDS, and there is also limited but growing evidence that JHSh/hEDS is also associated with depression, eating, and neuro-developmental disorders as well as alcohol and tobacco misuse. The underlying mechanisms behind this association include genetic risks, autonomic nervous system dysfunction, increased exteroceptive and interoceptive mechanisms and decreased proprioception. Recent neuroimaging studies have also shown an increase response in emotion processing brain areas which could explain the high affective reactivity seen in JHS/hEDS. Management of these patients should include psychiatric and psychological approaches, not only to relieve the clinical conditions but also to improve abilities to cope through proper drug treatment, psychotherapy, and psychological rehabilitation adequately coupled with modern physiotherapy. A multidimensional approach to this "neuroconnective phenotype" should be implemented to ensure proper assessment and to guide for more specific treatments. Future lines of research should further explore the full dimension of the psychopathology associated with JHS/hEDS to define the nature of the relationship. © 2017 Wiley Periodicals, Inc.
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            Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)

            Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. As many tissues can be involved, the underlying molecular defect can manifest itself in many organs and with varying degrees of severity, with widespread implications for anesthesia and perioperative management. This review focuses on issues relevant for anesthesia for elective and emergency surgery in EDS. We searched the literature for papers related to all EDS variants; at the moment most of the published data deals with the vascular subtype and, to a lesser extent, classic and hypermobility EDS. Knowledge is fragmented and consists mostly of case reports, small case series and expert opinion. Because EDS patients commonly require surgery, we have summarized some recommendations for general, obstetrical and regional anesthesia, as well as for hemostatic therapy.
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              Resistance to local anesthesia in people with the Ehlers-Danlos Syndromes presenting for dental surgery

              Background People with the Ehlers-Danlos Syndromes (EDS), a group of heritable disorders of connective tissue, often report experiencing dental procedure pain despite local anesthetic (LA) use. Clinicians have been uncertain how to interpret this apparent LA resistance, as comparison of EDS and non-EDS patient experience is limited to anecdotal evidence and small case series. The primary goal of this hypothesis-generating study was to investigate the recalled adequacy of pain prevention with LA administered during dental procedures in a large cohort of people with and without EDS. A secondary exploratory aim asked people with EDS to recall comparative LA experiences. Methods We administered an online survey through various social media platforms to people with EDS and their friends without EDS, asking about past dental procedures, LA exposures, and the adequacy of procedure pain prevention. Among EDS respondents who both received LA and recalled the specific LA used, we compared agent-specific pain prevention for lidocaine, procaine, bupivacaine, mepivacaine, and articaine. Results Among the 980 EDS respondents who had undergone a dental procedure LA, 88% (n = 860) recalled inadequate pain prevention. Among 249 non EDS respondents only 33% (n = 83) recalled inadequate pain prevention (P < 0.001 compared to EDS respondents). The agent with the highest EDS-respondent reported success rate was articaine (30%), followed by bupivacaine (25%), and mepivacaine (22%). Conclusions EDS survey respondents reported nearly three times the rate of LA non-response compared to non-EDS respondents, suggesting that LAs were less effective in preventing their pain associated with routine office dental procedures.

                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                1 October 2024
                October 2024
                : 16
                : 10
                : e70667
                Affiliations
                [1 ] Anesthesiology, Unidade Local de Saúde de Braga, Braga, PRT
                Author notes
                Article
                10.7759/cureus.70667
                11528175
                39493052
                7ae36182-b86c-43b1-9cc3-4641e002061f
                Copyright © 2024, Antunes et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 28 September 2024
                Categories
                Obstetrics/Gynecology
                Rheumatology
                Anesthesiology

                cesarean delivery,general anesthesia,hypermobile ehlers-danlos syndrome,neuraxial anesthesia,perioperative management,regional anesthesia

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