Linear disruption of umbilical cord: A rare anomaly of the cord associated with acute fetal distress and perinatal death/profound psychomotor retardation

Abstract

We report on a non‐malformed child with severe microcephaly and profound psychomotor delay. Review of the delivery/birth records documented descriptions consistent with linear disruption of the umbilical cord. This rare anomaly typically leads to acute fetal distress and perinatal death. Severe microcephaly and psychomotor delay without other anomalies should prompt a careful review of the delivery/birth records to search for umbilical cord descriptions consistent with this diagnosis. © 1996 Wiley‐Liss, Inc.

Splitting of the Umbilical Cord in a 13-Week-Old Fetus

“…There are still other cases in which Wharton's jelly deficiency is more pronounced, with variable separation of 1 artery, although keeping a single complete amniotic covering all around the cord. Finally, there are cases in which this amniotic covering is missing [3–5], as in the present case, resulting in isolated blood vessels with absent or minimal Wharton's jelly. The absence of amniotic epithelium around the split umbilical cord in placentas from living babies or fresh late fetal death [3–5] lends support to the assumption that both splitting and absent amniotic epithelium in the present case are not the result of maceration.…”

“…Whatever the cause, longitudinal deficiency of Wharton's jelly is a rare event, with only a few cases reported in the English literature [2][3][4][5]. Previous reported cases linked it with late fetal death [2,3], but other babies were born alive, although presenting different conditions, such as neurodevelopmental deficit [4] and patent omphalomesenteric duct [5].…”

“…Whatever the cause and presentation of linear Wharton's jelly deficiency, the umbilical vessels devoid of its usual surrounding of jelly are more prone to compression and kinking, predisposing thrombosis and its deleterious effects on the chorionic villi and, more importantly, in the fetus [4].…”

“…Transverse deficiency of Wharton's jelly is a common finding and is usually associated with umbilical cord hypercoiling. However, longitudinal deficiency leading to umbilical cord splitting is rare, with only a few cases reported in the English literature [2–5], to which we contribute another one seen in a severely macerated 13-week-old fetus.…”

Absence of Wharton's jelly around an umbilical artery

“…1 The absence of Wharton's jelly is an exceptional situation with a few rare cases described in the literature. [2][3][4][5][6][7][8] This situation, while rare, can cause acute fetal distress and even perinatal death due to compression of unprotected vessels. We report a case of absence of Wharton's jelly around an umbilical artery.…”