title: Molecular genetic and clinical review of Ehlers-Danlos Type VIIA: implications for management by the plastic surgeon in a multidisciplinary setting.
abstract: A syndrome now known as Ehlers-Danlos, comprising laxity and fragility of the skin associated with hypermobility of the large joints, was published in 1892 by Tschernogobow. Ehlers-Danlos type VIIA is an extremely rare form of the syndrome. While the UK-based Ehlers-Danlos Support Group recommends that the surgical management of patients with Ehlers-Danlos VIIA should be carried out in conjunction with a plastic surgeon, there is nothing in the plastic surgery literature regarding this syndrome. The management of patients suffering from Ehlers-Danlos VIIA is highly complex, as a result of the breadth of genetic and phenotypic presentations, and resulting complications. We present a review of the literature regarding this syndrome and, in particular, the surgical problems that may be encountered. A case report outlining our experience of successfully managing this condition is also presented.
authors.affiliation: Welsh Centre for Burns and Plastic Surgery, Morriston Hospital, Swansea, UK. iainwhitaker@fastmail.fm
authors.forename: Iain S
authors.initials: IS
authors.lastname: Whitaker
authors.forename: Warren M
authors.initials: WM
authors.lastname: Rozen
authors.forename: Scott A
authors.initials: SA
authors.lastname: Cairns
authors.forename: John
authors.initials: J
authors.lastname: Howes
authors.forename: Francis Michael
authors.initials: FM
authors.lastname: Pope
authors.forename: J
authors.initials: J
authors.lastname: Hamish Laing
mesh_terms: D002675:Child, Preschool; D002921:Cicatrix / Q000473:pathology; D062109:Dermatologic Surgical Procedures; D004535:Ehlers-Danlos Syndrome / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D019651:Plastic Surgery Procedures / Q000379:methods*; D012600:Scoliosis / Q000601:surgery; D012867:Skin / Q000473:pathology; D014945:Wound Healing