Covert Ehlers-Danlos syndrome in pregnancy

A 38-year-old primigravid woman presented to our hospital at 31 weeks of gestation with severe abdominal pain. She had no uterine contractions, vaginal bleeding, or trauma. She was hypotensive, her abdomen was peritonitic and a fetal heart rate tracing showed fetal distress with bradycardia (60 beats per min [normal range 110–160 beats per min]). We performed a laparotomy within minutes of admission, which revealed that the uterus had split open along its entire length, as well as 1·5 L of haemoperitoneum. We delivered the baby, repaired the rupture, and transfused blood products to prevent consumptive coagulopathy in the mother. Unfortunately, the neonate's Apgar scores were 2 points at 1 min and 4 points at 5 mins, and blood gases within the umbilical cord showed severe metabolic acidosis (pH 6·84, bicarbonate 10·5 mmol/L, partial CO2 pressure 61·6 mm Hg, base excess −23 mmol/L). He died despite resuscitation.