Classical-like Ehlers-Danlos Syndrome with Intestinal Perforation due to Uterine Evacuation—A Case Report—

Abstract

Ehlers-Danlos syndrome (EDS) is a congenital connective tissue disorder that is characterized by skin hyperextensibility, excessive range of joint motion, and tissue fragility. A case of classical-like EDS (clEDS), a rare type of EDS, was seen in a patient with gastrointestinal perforation after uterine evacuation by manual vacuum aspiration. A 34-year-old woman had a history of clEDS associated with repeated joint dislocations. At 10 weeks and 5 days of pregnancy, she was diagnosed with a missed miscarriage and underwent manual vacuum aspiration. Postoperatively, she developed a fever with lower abdominal pain, and contrast-enhanced computed tomography showed small bowel and uterine perforations. Consequently, emergency surgery was performed. A perforation in the anterior wall of the uterine cervix and two perforations in the jejunum were identified, and a small bowel resection and repair of the uterine perforation were performed. Intraoperative findings showed that the intestinal tract and mesentery were extremely fragile and easily damaged by traction, as has been previously reported in the literature. In conclusion, we recommend careful intraoperative manipulation in clEDS patients.