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.

Absence of Wharton’s jelly: case report

“…The pathogenesis has been attributed to Wharton's jelly degeneration, early incomplete fusion of amniotic and mesenchymal umbilical tissue, or hypoplasia of amnion and secondary loss of Wharton's jelly (6) . A relationship to meconium, suggested by Labarrere et al (7) but refuted by Thomlon & Hoo (10) , is probable since it was present in ours and in four out of the five previously reported cases.…”

“…There are only six cases of absence of Wharton's jelly reported in three articles in literature: four cases were presented by Labarrere et al (1985) (7) , one case was presented by Thomlon and Hoo (1996) (10) , and one case was presented by Kulkarni et al (2007) (6) . This article describes the seventh case of the Absence of Wharton's Jelly, diagnosed by pathological examination of the placenta (Table).…”

“…Wharton's jelly is a specialized tissue which is wrapped around the umbilical blood vessels, characterized by cellular elements and extracellular matrix, with the primary function of protecting the umbilical cord (6,10) . There are only six cases of absence of Wharton's jelly reported in three articles in literature: four cases were presented by Labarrere et al (1985) (7) , one case was presented by Thomlon and Hoo (1996) (10) , and one case was presented by Kulkarni et al (2007) (6) .…”

Absence of Wharton’s jelly around the umbilical arteries

“…(1996) who described such a case of "linear disruption of the umbilical cord" in a severely retarded child but without meconium stain as in our case. 4 But no association with any intrauterine infections or that of HIV infection with this anomaly or as being a cause of it has been described. The association of patent vitellointestinal duct and HIV reactive status of mother with that of this anomaly couldn't be studied further as the baby was discharged against medical advice in a morbid condition.…”

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

“…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][3][4][5], to which we contribute another one seen in a severely macerated 13-week-old fetus.…”