[Vascular Ehlers-Danlos syndrome discovered after splenic aneurysm rupture: a case report].

Abstract

We report a case of vascular Ehlers-Danlos syndrome in a 30-year-old male patient. He presented to his local doctor with sudden onset of epicardial pain at around 5:00 p.m. on July 3, XXXX. On the same day, he was transferred to our hospital for extensive intra-abdominal hemorrhage suspected on abdominal computed tomography (CT). During emergency transport, his abdomen was flat, soft, and tender in the pericardial area, and blood biochemistry tests revealed a decreased hemoglobin of 10.2g/dL. He was admitted to our hospital, and conservative treatment was initiated. Five years ago, he underwent Hartmann procedure at another hospital for idiopathic perforation of the sigmoid colon. A month after discharge from the hospital, he had to undergo emergency surgery again at our hospital for colon resection and colostomy reconstruction due to colon perforation at the mouth side of the colostomy. He reported a history of colorectal perforation on the mouth side of the colostomy. After 6 months, a colostomy was performed and he was diagnosed with a terminal illness. Based on a family history of idiopathic colorectal perforation on his mother's side and the ruptured aneurysm, vascular-type Ehlers-Danlos syndrome was suspected. After admission, his symptoms of pericardial pain improved, but a CT scan on the 16th day revealed enlarged hepatic and splenic aneurysms. On July 24, he was transferred to a local hospital for splenic artery aneurysm treatment and underwent coiling the next day. His postoperative course was good, and he was transferred back to our hospital on July 26. He continued to show good progress and was discharged from the hospital on August 1. After discharge, he visited the outpatient genetic clinic for further examination. However, on October 9 of the same year, he developed aortic dissection and passed away.