Congenital short-bowel syndrome: prenatal sonographic findings of a fatal anomaly.

Abstract

The sonographic findings in a fatal case of congenital short-bowel syndrome are reported. Sonography at 11 weeks of gestation showed a 11 x 6 mm hyperechoic mass interpreted to be a midgut umbilical hernia. A repeat scan 2 weeks later showed an intact anterior abdominal wall, no umbilical herniation, and appropriate fetal growth. Forty-eight hours after full-term, vaginal delivery, the infant began vomiting bile and passing blood rectally. Imaging studies showed distended bowel loops without air-fluid levels and incomplete bowel obstruction. Laparotomy showed malrotation and short small bowel without volvulus. The infant died at 9 weeks of age. When delayed return of the midgut to the abdominal cavity is noted on prenatal sonograms, follow-up sonograms should be done throughout the second trimester, especially in patients with a family history of short-bowel syndrome, to search for dilated short bowel loops. If such loops are found, patients should be given options for pregnancy termination.