Surgical Recommendations in Ehlers-Danlos Syndrome(s) Need Patient Classification: The Example of Ehlers-Danlos Syndrome Hypermobility Type (a.k.a. Joint Hypermobility Syndrome)
Marco Castori
Abstract
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Marco Castori
Abstract
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In particular, EDS-HT is probably the most common EDS subtype [3] and likely represents one and the same disorder as the joint hypermobility syndrome (JHS) [4] .Therefore, it is reasonable that JHS/EDS-HT is the EDS form most frequently encountered in many surgery subspecialties, except perhaps in vascular and chest clinics.Not many data have been published concerning surgical aspects of JHS/EDS-HT.In contrast to vascular EDS in which surgery is typically associated with high risks, daily practice indicates that preventive contraindication to surgery is not a feature of JHS/EDS-HT.Nevertheless, a series of recently accumulated evidence and anecdotal reports depicts an extraordinarily complex pathophysiology of JHS/EDS-HT, which extends much beyond the involvement of the integumentary and musculoskeletal systems.Accordingly, many factors may influence the outcome of surgical and anesthetic procedures and should be considered during treatment planning.Table 1 summarizes available data and the ensuing recommendations in JHS/EDS-HT.I hope that this additional information will help to offer a more tailored surgical approach for patients affected by the JHS/EDS-HT subtype.It is expected that future studies will improve our knowledge on the widespread consequences of JHS/EDS-HT, as well as other major EDS subtypes, in order to select more efficient and personalized management interventions.
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In particular, EDS-HT is probably the most common EDS subtype [3] and likely represents one and the same disorder as the joint hypermobility syndrome (JHS) [4] .Therefore, it is reasonable that JHS/EDS-HT is the EDS form most frequently encountered in many surgery subspecialties, except perhaps in vascular and chest clinics.Not many data have been published concerning surgical aspects of JHS/EDS-HT.In contrast to vascular EDS in which surgery is typically associated with high risks, daily practice indicates that preventive contraindication to surgery is not a feature of JHS/EDS-HT.Nevertheless, a series of recently accumulated evidence and anecdotal reports depicts an extraordinarily complex pathophysiology of JHS/EDS-HT, which extends much beyond the involvement of the integumentary and musculoskeletal systems.Accordingly, many factors may influence the outcome of surgical and anesthetic procedures and should be considered during treatment planning.Table 1 summarizes available data and the ensuing recommendations in JHS/EDS-HT.I hope that this additional information will help to offer a more tailored surgical approach for patients affected by the JHS/EDS-HT subtype.It is expected that future studies will improve our knowledge on the widespread consequences of JHS/EDS-HT, as well as other major EDS subtypes, in order to select more efficient and personalized management interventions.
Key concepts: Ehlers–Danlos syndrome, Joint hypermobility, Medicine, Hypermobility (travel), Surgery, Dermatology, Anatomy, Physical therapy