Treatment of the wide open wound in the Ehlers-Danlos syndrome

Abstract

Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels, other organs such as the eyes and the intestine can be affected in this syndrome. Accordingly, interdisciplinary cooperation is necessary for a successful treatment. Three basic surgical problems are arising due to an EDS: decreased the strength of the tissue causes making the wound dehiscence, increased bleeding tendency due to the blood vessel fragility, and delayed wound healing period. Surgery patients with an EDS require an experienced surgeon in treating EDS patients; the treatment process requires careful tissue handling and a long postoperative care. A surgeon should also recognize whether the patient shows a resistance to local anesthetics and a high risk of hematoma formation. This report describes a patient with a wide open wound on the foot dorsum and delayed wound healing after the primary approximation of the wound margins.

Pelvic Organ Prolapse in Ehlers-Danlos Syndrome

“…Specific surgical consideration due to tissue characteristics must be taken into account when planning to operate. Intraoperatively, the tissue may be friable and fragile making it difficult to hold suture material, resulting in diffuse- and difficult-to-control bleeding [ 13 ]. Gentle handling of tissues during surgery can help avoid injury caused by the reduced ability to resist mechanical forces [ 14 ].…”

“…Gentle handling of tissues during surgery can help avoid injury caused by the reduced ability to resist mechanical forces [ 14 ]. Basic wound closure principles of preventing hematomas and performing tension-free closures are essential in avoiding cutaneous complications like wound dehiscence, poor suture holding, and wide scar formation [ 13 ]. Delaying or avoiding dressing changes minimizes frequent trauma to healing tissues and helps avoid overstretching of the skin [ 14 ].…”

Management of invasive ductal carcinoma in hypermobile Ehlers-Danlos syndrome: a case report

“…Advanced therapeutic wound dressings are produced using a variety of materials, impregnation formulations, and drug-delivery systems (6). Baik et al (7) illustrate that use of a mixed material, alginate-based collagen dressing precipitated wound healing in a patient with EDS following partial skin necrosis and delayed closure of a lateral foot wound repaired in primary approximation. This case report is promising, however, further clinical studies are needed to inform evidence-based utilization of advanced therapeutic wound dressings in EDS.…”

Appendiceal torsion in Ehlers-Danlos syndrome: A case report of a rare phenomenon in a rare disease

“…Surgeries in patients with EDS are associated with significant complications, including poor wound healing, dehiscence and intraoperative bleeding [ 10 ]. Poor wound healing is a characteristic feature of classical type EDS with poor or absent type V collagen.…”

“…Type V collagen is a regulatory fibrillar protein that strengthens tissue scaffolds by interaction with other collagens. The absence of this protein contributes to basic surgical problems: extreme fragility of tissue and blood vessels, decreased strength of tissue and ability to hold sutures, and increased bleeding risk [ 10 ]. This causes prolonged healing time and wide atrophic scars.…”