A case of vascular Ehlers–Danlos syndrome with a ruptured hepatic artery after surgical treatment of peritonitis caused by the perforation of the colon

• Main Authors: Daisuke Kakinuma, Takeshi Yamada, Yoshikazu Kanazawa, Kunihiko Matsuno, Tomoko Sahara, Hiroshi Yoshida
• Format: Article
• Language: English
• Published: SpringerOpen 2021-03-01
• Series: Surgical Case Reports
• Subjects: Vascular type of Ehlers–Danlos syndrome; Sigmoid colon perforation; Hepatic artery rupture; Pan-peritonitis; Remote vascular catastrophe
• Online Access: https://doi.org/10.1186/s40792-021-01156-0

Abstract Background Ehlers–Danlos syndrome (EDS) is an inherited disorder that causes connective tissue fragility. The vascular type of EDS (vEDS) caused by defective collagen type III production accounts for 5%–10% of all EDS cases. Patients can develop gastrointestinal or arterial ruptures, which cause poor prognosis. We report a case of a patient who experienced colonic rupture, which was immediately followed by arterial rupture. Case presentation A 40-year-old man who had been genetically diagnosed with vEDS 6 years previously was admitted to our hospital with ischemic colitis. After 3 days of conservative treatment, his abdominal pain worsened, and computed tomography (CT) revealed free air in the abdominal cavity. Pan-peritonitis due to perforation of the sigmoid colon was diagnosed. Intraperitoneal lavage and drainage and Hartmann’s operation were urgently performed. Because the patient had confirmed vEDS, we performed the surgery in a protective manner. The postoperative course was initially good, and he was transferred to the general ward 3 days after surgery. However, 5 days after surgery, massive intra-abdominal hemorrhage suddenly occurred, and contrast-enhanced CT showed an aneurysm in the common hepatic artery that had ruptured; this aneurysm was not present before surgery and was far from the surgical field. Although we considered an emergency operation, the patient suddenly experienced cardiac arrest and was unresponsive to resuscitation. Conclusions In cases of vEDS, vascular rupture can occur immediately after surgery for intestinal rupture. We recommend paying special attention to vascular complications in patients in their forties, as such complications are the most common causes of death.