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Abstract

A 20-year-old primigravida was referred due to increased risk of trisomy 21 in the triple test result at 21 weeks of gestation, which was 1/59. Sonographic examination revealed hypoplastic nasal bone (NB=4.3 mm) and hyperechogenic bowel with mildly dilated segments (bowel diameter=4.5 mm) (Figure 1). Past medical histories of both parents were insignificant. There was no consanguineous marriage. Amniocentesis was performed. Quantitative fluorescence polymerase chain reaction (PCR), karyotype, and cystic fibrosis panel were normal. Due to hyperechogenic bowel and mildly dilated segments of the bowel, close surveillance was planned. At 24 weeks and 25 weeks, these segments of the bowel continued to dilate and increased to 9.8 mm and 11.2 mm, respectively. At 25 weeks of gestation, anorectal complex was observed intact. Dilatation of the bowel progressed and reached to 23.8 mm at 29 weeks of gestation, which was also coincided with an increase in the amount of amniotic fluid (Figure 2). Besides dilated bowel segments, a cystic structure with hyperechogenic inner fluid than the other dilated bowel segments had become noticeable during the examination at 29 weeks. Due to the risk of volvulus, weekly follow-up visits were scheduled. From 29 to 37 weeks, the size of the cyst and amount of amniotic fluid continued to increase, although the diameter of the dilated bowel remained relatively stable (Figure 3). Within last 4 weeks before delivery, in addition to the cyst and bowel dilatation, ascites began to accumulate in the fetal abdomen. In this period, Doppler of the middle cerebral artery and umbilical artery remained normal, however a-waves in the ductus venosus started to deepen and fetal ascites progressively increased. We, therefore, decided to perform paracentesis via ex utero intrapartum treatment (EXIT) procedure in the intrapartum period. At 37 weeks, planned cesarean under deep general anesthesia was performed. Male infant weighing 2910 g was delivered. Umbilical cord was not clamped, and oxytocin administration was withheld. Newborn was laid between the mothers legs. Under the ultrasound guidance, paracentesis was performed and 240 mL of green fluid was aspirated, which effectively ruled out hemorrhagic ascites. The newborn was neither intubated nor required mechanical ventilation, and after a brief stay in the neonatal intensive care unit, he was operated. After an uneventful postoperative course, he was discharged. At 2 years of age, he was faring well.