Surgical Management of Colonic Perforation in a Patient with Vascular Ehlers-Danlos Syndrome with no Family History: A Case Report

• Main Authors: Shintaro Kanaka, Takeshi Yamada, Akihisa Matsuda, Goro Takahashi, Keisuke Hara, Hiroshi Yoshida
• Format: Article
• Language: English
• Published: The Japan Society of Coloproctology 2020-10-01
• Series: Journal of the Anus, Rectum and Colon
• Subjects: ehlers-danlos syndrome; colonic perforation; acute abdomen
• Online Access: https://www.jstage.jst.go.jp/article/jarc/4/4/4_2020-029/_pdf/-char/en

Vascular Ehlers-Danlos syndrome (vEDS) is a rare autosomal dominant connective tissue disease. Patients with vEDS are at a high risk of developing severe complications (such as arterial aneurysm, arterial rupture, intestinal rupture) at an early age. We report a case of colonic perforation in a vEDS patient with no family history of that disease. A 28-year-old man with abdominal pain arrived at our hospital in an ambulance. The preoperative diagnosis was panperitonitis due to gastrointestinal perforation. Although his parents had not suffered from vEDS, he had been diagnosed with the disease at 25 years of age because of his history of arterial dissection. We performed an emergency operation using Hartmann's procedure to construct a descending colostomy. There remains a lack of consensus on surgical management in vEDS patients with gastrointestinal perforation because of the limited number of reported cases.