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Keywords

dehiscence, Ehlers-Danlos syndrome, enterocutaneous fistula, mesenchymal stromal cells, vitamin C, vascular Ehlers-Danlos syndrome, wound care

 

Authors

  1. Prentice, David Andrew MBBS, FRACP
  2. Pearson, Wendy Ann MNg
  3. Fogarty, Janice MSc

Abstract

ABSTRACT: Vascular Ehlers-Danlos syndrome (EDSv) can present with life-threatening surgical complications. The article describes the case of a patient with EDSv who developed total abdominal wound dehiscence and multiple enterocutaneous fistulas. Treatment with IV allogeneic mesenchymal stromal cells (MSCs) and high-dose vitamin C was trialed with success. Near-complete wound healing of the abdominal dehiscence with a 94% reduction in the size of the wound bed occurred. Maturation of the enterocutaneous fistulas also ensued.

 

There is no current consensus on the management of large cutaneous wounds in EDSv. This article discusses the pathophysiology of wound healing with regard to nutrition requirements and growth factors with special reference to collagen deficits in EDSv. A potential therapy with IV vitamin C supplementation and MSCs is proposed following the patient's positive outcome. Medium-dose MSCs and high-dose IV vitamin C may offer significant benefits to complex and problematic wounds.