Abstract
Umbilical cord ulceration has been associated with congenital upper intestinal (duodenal or jejunal) atresia and can lead to fatal fetal intrauterine hemorrhage. We report a case of spontaneous hemorrhage from the umbilical cord, incidentally noted at the time of ultrasound in a 33-week fetus with suspected duodenal atresia, in which immediate delivery resulted in a good outcome. Despite many reports in the literature of congenital upper intestinal atresia and its association with umbilical cord ulceration, the propensity for this lesion for fetal hemorrhage, and the resulting perinatal morbidity and mortality, there appears to be a gap in the dissemination of this knowledge. In fetuses with suspected congenital upper intestinal atresia, recognition of the entity of umbilical cord ulceration may be improved by ultrasound with special attention to the amount of Wharton's jelly within the cord. Routine antepartum fetal surveillance may reduce perinatal morbidity and mortality from this condition. A high index of suspicion is needed to make the diagnosis of umbilical cord ulceration in association with congenital upper intestinal atresia. The role of amniotic fluid bile acids in the genesis of this disorder needs further study.
Highlights
Umbilical cord ulceration is an unusual lesion characterized by degenerative changes in Wharton’s jelly and overlying amnion, thinning of vessel walls, and superficially located, friable vessels prone to hemorrhage. This lesion, its association with congenital upper intestinal atresia, and its association with increased perinatal morbidity and mortality have been well documented in the literature; despite this, awareness of this condition and the potential associated lethality remains limited [1]
We describe a case of umbilical cord ulceration in a 33-week fetus with suspected duodenal atresia where active intramniotic hemorrhage from the umbilical cord was identified during real-time ultrasound imaging that led to a prompt delivery and a good outcome
They devised a grading scheme to characterize the degree of ulceration: grade 0, no ulceration; grade 1, loss of amniotic epithelium; grade 2, detachment of basal lamina of amniotic epithelium; grade 3, loss of Wharton’s jelly with widespread grade 2 change; and grade 4, exposed umbilical artery or vein. They found that 28/28 controls had no or grade 1 ulceration as compared to 16/20 duodenal or jejunal atresia cases that had grade 2 or greater ulceration. They concluded that these findings suggested gradual pathologic changes in the umbilical cords of fetuses affected by upper intestinal atresia that precede clinical presentation
Summary
Umbilical cord ulceration has been associated with congenital upper intestinal (duodenal or jejunal) atresia and can lead to fatal fetal intrauterine hemorrhage. Despite many reports in the literature of congenital upper intestinal atresia and its association with umbilical cord ulceration, the propensity for this lesion for fetal hemorrhage, and the resulting perinatal morbidity and mortality, there appears to be a gap in the dissemination of this knowledge. In fetuses with suspected congenital upper intestinal atresia, recognition of the entity of umbilical cord ulceration may be improved by ultrasound with special attention to the amount of Wharton’s jelly within the cord. A high index of suspicion is needed to make the diagnosis of umbilical cord ulceration in association with congenital upper intestinal atresia. The role of amniotic fluid bile acids in the genesis of this disorder needs further study
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