Vein graft safely repairs damaged artery in man with vascular EDS

30-year-old had multiple areas of vessel wall damage in major arm artery

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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A man with vascular Ehlers-Danlos syndrome (EDS) underwent a vein graft surgery to replace a damaged section of artery in his right arm with good results, as described in a recent report.

“It has been four years after the operation … and the patient has good right-hand function,” the scientists reported.

The case report, “Atypical presentation of forearm compartment syndrome in a case of vascular type Ehlers–Danlos syndrome,” was published in the journal Case Reports in Plastic Surgery and Hand Surgery.

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Risk of Aorta Damage With Vascular EDS May Be Due Mostly to Aging

Case raises possibility that vein grafting is ‘safe, durable option’ with vEDS

Vascular EDS, or vEDS, is a severe type of EDS characterized by fragile tissue in blood vessels, with a high risk of bleeding-related problems.

A 30-year-old Chinese man went to the hospital due to redness and swelling in his forearms, which was more severe on the right side. He had a diagnosis of EDS based on his family history.

The man’s condition was initially assumed to be an infection, and he was given antibiotics. During his hospital stay, clinicians noted that his forearm swelling continued to worsen. His more affected right forearm also showed areas of unusual hardness and a weakened pulse.

Emergency imaging showed multiple pseudoaneurysms — areas where the blood vessel wall has been damaged, allowing blood to bulge out — along the major arteries in his right forearm, called the radial and ulnar arteries.

An urgent surgery was undertaken, during which doctors found a particular region of the radial artery that showed extensive damage along a length of about 15 cm (about 6 inches).

This piece of blood vessel was removed, and then the section of artery was replaced with new blood vessel tissue removed from a large superficial vein in the patient’s leg. This technique is called autologous saphenous vein grafting. The surgery site then was covered with a skin graft to promote healing.

In the four years since the surgery when the study was concluded, the man’s arm had healed well. “Upon follow-up, the wound healed well and the patient has good right-hand function and he can write nicely with his right hand. To our knowledge, there were no complications,” the researchers wrote.

Still, they stressed that it’s impossible to draw firm conclusions from one isolated case, emphasizing the need for further study.

“We believe that autologous saphenous vein grafting is a safe, durable option to reconstruct a long segment of an arterial defect in this group of patients,” the scientists wrote. “However, in view of underlying fragility of the patient’s own veins, further study is recommended to confirm the long-term outcome of using autologous vein graft in treating patients with vEDS.”

To prevent complications, “meticulous technique and gentle soft tissue handling” are necessary throughout the surgery, they added.

Genetic testing, undertaken after the surgery, confirmed a mutation in the COL3A1 gene. Mutations in this gene are known to cause vascular EDS. The researchers noted that the specific disease-causing mutation identified in this man, referred to as c. 1852 G > C, has not been documented in prior scientific literature.