Molecular genetic and clinical review of Ehlers-Danlos Type VIIA: implications for management by the plastic surgeon in a multidisciplinary setting.
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.
Whitaker
Iain S
IS
Welsh Centre for Burns and Plastic Surgery, Morriston Hospital, Swansea, UK. iainwhitaker@fastmail.fm
Rozen
Warren M
WM
Cairns
Scott A
SA
Howes
John
J
Pope
Francis Michael
FM
Hamish Laing
J
J
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