At the Royal Perth Hospital, Perth, Western Australia, David Andrew Prentice, MBBS, FRACP, is General Physician, Department of Internal Medicine; Wendy Ann Pearson, MNg, is Clinical Nurse Consultant, Stomal Therapy Service; and Janice Fogarty, MSc, is Medical Scientist in Charge: Cell and Tissue Therapy Department. Acknowledgments: The authors thank Dr Benedict Carnley of Cell and Tissue Therapy at Royal Perth Hospital, along with the extended multidisciplinary team including psychiatry, dietetics, pain specialists, general surgeons, intensivists, gastroenterologists, palliative care, pastoral care, social work, and the multitude of nurses who cared for this patient during his extended inpatient stays. Special thanks also to his wife whose dedication brought the patient home. The authors have disclosed no financial relationships related to this article. Submitted June 26, 2020; accepted in revised form September 21, 2020; published online ahead of print April 5, 2021.
Adv Skin Wound Care. 2021 Jul 1;34(7):1-6. doi: 10.1097/01.ASW.0000741524.79369.7a.
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.
血管型埃勒斯-当洛斯综合征（EDSv）可导致危及生命的手术并发症。本文描述了一例 EDSv 患者出现全腹部切口裂开和多个肠皮瘘的病例。采用异体间充质基质细胞（MSCs）和大剂量维生素 C 静脉输注的方法进行治疗，获得了成功。腹部切口裂开几乎完全愈合，伤口床面积缩小了 94%。肠皮瘘也随之成熟。目前对于 EDSv 患者的大型皮肤创面处理尚无共识。本文讨论了与营养需求和生长因子相关的创面愈合的病理生理学，特别提到了 EDSv 中胶原蛋白的缺乏。根据患者的良好转归，提出了 IV 维生素 C 补充和 MSCs 治疗的潜在疗法。中剂量 MSCs 和大剂量 IV 维生素 C 可能会为复杂和棘手的创面带来显著益处。
