Man with vEDS survives ruptured aneurysm after bowel surgery

Diagnosis of vascular EDS was received 5 years earlier

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

Share this article:

Share article via email
A patient on a gurney is seen in a hallway, heading to a surgery room.

A man with vascular Ehlers-Danlos syndrome (vEDS) had a ruptured splenic artery, a blood vessel that supplies the spleen, days after surgery for a perforated bowel, leading researchers in Japan to call for careful monitoring after surgery.

“Surgeons should cooperate with physicians and interventional radiologists to perform surgery in an environment that allows the prompt diagnosis and treatment of postoperative [after surgery] complications,” the researchers wrote about the case, “Successful management of splenic artery dissection after sigmoid colon perforation in vascular Ehlers–Danlos syndrome,” which was published in Surgical Case Reports.

Along with overly mobile joints and unusually stretchy skin, which are common with other types of EDS, people with vEDS have fragile arteries and internal organs that are prone to bulging, rupturing, or tearing, called dissecting.

Here, researchers described the case of a man who had rupture of a dissecting splenic artery aneurysm caused by the perforation of the sigmoid colon, the S-shaped part of the large bowel. An aneurysm is an abnormal swelling or bulge in a weakened blood vessel.

The man, 48, had received a diagnosis of vEDS about five years before. He suddenly began having intense pain in the lower abdomen, which led him to seek medical help. A CT scan revealed fluid and free air around the sigmoid colon, suggesting a perforation and prompting emergency surgery.

Recommended Reading
A strand of DNA is shown.

Mutations linked to vascular EDS alter ECM properties: Study

Surgery and complications from vEDS

The doctors performed a Hartmann’s procedure, a type of surgery on the colon, to remove a part of the sigmoid colon while performing a colostomy to create an opening for the large intestine through the abdomen.

The removed section showed a small area of depression around the perforation and an abcess, that is, a painful lump that contains pus, and fluid leakage. The muscle layer of the colon was thinner than usual.

After the surgery, the man seemed to be recovering, but developed severe pain in his upper abdomen nine days later. A CT scan showed internal bleeding caused by a rupture of a dissecting splenic artery aneurysm.

“The aneurysm was not observed on preoperative [before surgery] CT and was distant from the surgical site,” the researchers wrote.

The doctors then performed a transcatheter arterial embolization, which involved inserting a thin tube into the splenic artery and guiding it into the bleeding blood vessel so the splenic artery could be blocked and blood flow stopped.

The doctors encountered some challenges during the procedure because the dissection had spread, “owing to the fragility of the arterial wall,” which affected nearby arteries. Still, they managed to successfully stop the bleeding from the splenic artery and the man was discharged from the hospital after 19 days, following his recovery.

“Rupture was diagnosed early using contrast-enhanced CT, and [transcatheter arterial embolization] was performed immediately, which saved the patient’s life,” the researchers wrote. “Postoperative complaints of sudden abdominal pain require close examination considering the possibility of arterial rupture.” Cooperating with “interventional radiologists is important for prompt treatment of vascular complications,” they said.