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Ehlers-Danlos syndrome in pregnancy

A 17-year-old girl with a known diagnosis of vascular type (IV) Ehlers-Danlos syndrome was seen in the antenatal clinic at 9 weeks’ gestation. She had a strong family history of EhlersDanlos syndrome. Her mother had a spontaneous bowel perforation at 3 years of age and after giving birth to three children, died at 30 years old due to spontaneous rupture of an abdominal aortic aneurysm. The patient herself had a spontaneous bowel perforation at 14 years of age. A Hartman’s type procedure was performed in 2003. This was followed by completion of colectomy, ileorectal anastomosis and ileostomy in April 2004 and finally a reversal was done in July 2004. Her last menstrual period was on 12 April 2006. The transabdominal scan confirmed 9 weeks viable gestation. Considering the type of collagen disorder, she was offered medical termination, as it was considered to be the safest option. The high risk of pregnancy complications, including up to 25% maternal mortality was explained, and the couple opted for termination of pregnancy. She had Mifepristone and passed products of conception after receiving three doses of 400 mg Misoprostol. The transvaginal scan, which was performed the next day, showed some mixed echoes measuring 5.761.5 mm. The patient had a suction curettage because of heavy vaginal bleeding and a significant amount of products were obtained on evacuation. Postoperative recovery was uneventful. The patient remained stable and was sent home on the same day.