Uterine torsion as an elusive obstetrical emergency in pregnancy: is there an association between gravid uterus torsion and Ehlers–Danlos syndrome?: a case report

Timely diagnosis of uterine torsion can lead to acceptable maternal and fetal outcomes. This article presents the case of a 42-year-old pregnant woman, diagnosed with a rare 270° uterine torsion, in whom proper management led to good maternal outcomes but, unfortunately, severe prematurity and metabolic acidosis led to neonatal death. Moreover, the mother was clinically suspected for Ehlers–Danlos syndrome.

In December 2020, a 42-year-old pregnant Iranian woman, Gravid 3 para2 live2, at 30 weeks of gestation presented to the obstetric emergency department of Vali-Asr Hospital (Birjand, Iran) suffering from acute severe generalized abdominal pain, nausea, vomiting, and dizziness while she was hemodynamically unstable. After resuscitation, owing to persistent fetal bradycardia on fetal heart rate monitoring, she underwent an emergency cesarean section. Infra-umbilical midline skin incision was made, and when the abdominal cavity was opened, owing to abnormal appearance of the uterus, we further investigated the abdominopelvic cavity. Surprisingly, the uterus was dextrorotated by 270°. After uterine detorsion through a Kerr incision, a nonvigorous male baby was born with severe metabolic acidosis that led to his death soon after birth. Interestingly, we could find no predisposing factors such as pelvic abnormalities during surgery. Nevertheless, as her postoperative detailed physical examination revealed skin hyperextensibility, joint laxity, pelvic organ prolapse, and trivial exophthalmos, connective tissue disorders, mainly Ehlers–Danlos syndrome, were suspected. Unfortunately, for significant financial, cultural, and religious reasons, the patient refused to undergo further investigations. Additionally, despite severe congested uterus and subsequent uterine atony, timely diagnosis and anatomical correction of the gravid uterus before uterine incision prevented iatrogenic complications. The mother was discharged 2 days later without any postpartum complications.

Although uterine torsion is an extremely rare condition during pregnancy, based on severe associated maternal and perinatal complications, it is important to take this diagnosis into consideration as an differential diagnosis. Moreover, connective tissue disorders seem to be a potential risk factor for uterine torsion, although further studies on this subject are required.