Umbilical cord ulcer: a serious in utero complication of intestinal atresia

We have experienced six cases of umbilical cord ulcer that resulted in life-threatening antenatal fetal haemorrhage. Fetal distress and bloody amniotic fluid that led to intrauterine and neonatal death in four patients complicated the pregnancy courses. The remaining two infants are still alive. All the infants had upper intestinal atresia below the papilla of Vater. Pathologically, umbilical arteries and surrounding Wharton's jelly were necrotic in all cases. Activated macrophages containing pigment granules were present within the ulcer bed, necrotic areas of Wharton's jelly and fetal membranes. During the same period, we encountered 38 other cases of upper intestinal atresia and the placentae were examined in 23 cases. Microscopic degeneration of Wharton's jelly was seen in three out of 23 cases. Total bile acids content in amniotic fluid was elevated in one case of umbilical cord ulcer. We speculate that the umbilical cord ulcer is closely related to in utero regurgitation of the bile. We also think that the incidence of this serious association is higher than has been reported previously.