A Case of Incarceration of the Gravid Uterus & the Ehlers-Da
A 28-year-old Caucasian female, gravida 1 at 24 weeks and 2 days gestational age (GA) presented to her local obstetrician with a complaint of painful uterine contractions. On initial exam, she had a severely retroverted uterus with anterior displacement of the cervix behind the pubic symphysis. She had no urinary retention or constipation. Her medical history was significant only for mild mitral valve prolapse and patella subluxation. She had no prior surgical history other than right eyelid ptosis correction. She was transported to a tertiary care center due to concern for the incarceration of the gravid uterus.

Upon arrival, she was admitted to labor and delivery. Her vital signs were within normal limits. She was noted to have regular, painful uterine contractions every 3 minutes. The abdomen was gravid, soft, and non-tender to palpation. A pelvic exam revealed a bulging soft tissue mass palpable just inside the vaginal introitus. The cervix could not be visualized on speculum exam and was only palpable behind the pubic symphysis with suprapubic pressure. Cervix was undilated, not effaced, and without bloody show. Ultrasound demonstrated a vertex fetus with a posterior placenta. The fetal size was appropriate with an estimated fetal weight of 908g. The amniotic fluid volume was normal. A vaginal wet mount was negative. A course of betamethasone was initiated for fetal lung maturity; magnesium sulfate was ordered for fetal neuroprotection and tocolysis. A pelvic MRI was obtained and the diagnosis of IGU was confirmed. The cervix was 13.1 centimeters in length and the myometrium of the uterine fundus was thin. Given the frequency and intensity of uterine contractions despite tocolytic agents, there was a concern for uterine rupture without intervention. A neonatal consultation was obtained in case preterm delivery was warranted. On the second hospital day, the patient was taken to the operating suite for reduction of uterine incarceration. Neuroaxial anesthesia was administered and an attempt at manual reduction was unsuccessful. An exploratory laparotomy was performed via a vertical midline incision. The uterine fundus was too deep in the pelvis to reduce abdominally, so a third assistant elevated the uterine fundus from a vaginal approach, displacing the fundus enough to allow the primary surgeon to complete the anteversion maneuver restoring normal uterine polarity. The vaginal assistant was immediately able to palpate the cervix in its normal anatomic position. The cervix remained undilated. The fetus was now in breech presentation and the placenta was along the anterior uterine wall by ultrasound assessment. After several minutes of reassuring intraoperative fetal monitoring, the abdomen was closed in the normal fashion and the patient was taken to the recovery room. Continuous fetal monitoring was performed overnight. Her postoperative course was uncomplicated and she was discharged on hospital day 8.

After discharge, she resumed care with her local obstetrician. Her pregnancy was uncomplicated until 33 weeks gestation. At that point, she experienced preterm labor with resultant preterm, premature rupture of membranes. At 34 weeks GA, she was delivered by cesarean section secondary to breech presentation and advancing preterm labor. Her postoperative course was uncomplicated and the neonate did well. During her second pregnancy, the patient again experienced uterine incarceration at 14 weeks GA. This was quickly identified and corrected with a manual reduction in the lateral decubitus position under moderate sedation. Due to her history of preterm delivery, she was monitored with serial cervical lengths. At 21 weeks GA, there was significant cervical shortening with no measurable cervix on ultrasound (previous cervical length the week prior was 3 cm.) She was hospitalized for 2 weeks and was ultimately delivered by cesarean section at 23 weeks GA due to advancing preterm labor and breech presentation. There were no postpartum maternal complications. The neonate did well and was discharged home after a prolonged NICU stay.

The patient underwent a genetic evaluation and was found to have a variant in the COL5A1 gene. This variant has been described in patients meeting clinical criteria for diagnosis of the Ehlers Danlos syndromes. She has elected to have no further pregnancies.