Neonatal disseminated intravascular coagulation after thrombosis of a fetal intra-abdominal umbilical vein varix

Abstract

Case reportA G2P1 was referred at 32 weeks' gestation. Routine ultrasound had revealed a fetal intra-abdominal umbilical vein varix (FIUV) (Figure 1) without associated abnormalities. Amniocentesis showed a normal male karyotype 46, XY. On arrival, thrombosis within the FIUV was observed. Because of fetal bradycardia, an urgent caesarean section was performed. Apgars were 3 and 5 at 1 min and 5 min, respectively, and the umbilical cord pH was 7.049. The boy was intubated immediately after birth and ventilated for 48 h. On umbilical artery catheterisation, severe bleeding occurred for which he was treated with fresh frozen plasma and platelet transfusions. Laboratory investigations showed thrombopenia (18,000/mm), prolonged aPTT (54.9 s), nonmeasurable PT, fibrogen 560 mg/dl and D-dimers of 53.90 mg/ ml, confirming the diagnosis of disseminated intravascular coagulation (DIC). Thrombophilia screening showed no abnormalities. Histopathology of placenta and umbilical cord showed a haemorrhagic lesion in the umbilical cord. Bleeding was controlled, laboratory examinations returned to normal and the neonate recuperated well.

Case 253: Thrombosed Umbilical Venous Varix in an Infant

“…Thrombosis of the umbilical vein varix is a known complication, with only a few cases having been reported in the literature (3,5). It is thought to occur due to flow turbulence in the dilated vein, and it has been reported to result in disseminated intravascular coagulation and fetal demise (10). To our knowledge, clinical presentation and outcome of a thrombosed umbilical venous varix in the postnatal period has not been previously described in the literature.…”

Severe Consumptive Coagulopathy in an Extremely-Low-Birth-Weight Infant with Intra-Abdominal Umbilical Vein Varix: A Case Report

“…13 No patients had chromosomal abnormalities, but congenital anomalies were found in three cases (one case with an atrial septal defect and two cases with gastroschisis). 6,8 Coagulopathy was reported in four cases (27%), [9][10][11] and two cases had intracranial hemorrhage. 10 There were no apparent differences in gestational age and birth weight between the premature infants with coagulopathy and the ones without coagulopathy.…”

The Triple Test Markers Profile Among Fetuses and Children Diagnosed With Umbilical Vein Varix

“…31 An association between thrombosis of the UVV and disseminated intravascular coagulation also has been recently reported, suggesting that postnatal developmental delay abnormalities may be a result of consumption of coagulation factors on the thrombogenic surface of the UVV segment, with the subsequent occurrence of micro-infarctions in various areas throughout the brain. 32,33 Another hypothesis by which UVV may be associated with postnatal developmental delay abnormalities may mimic one or several of the mechanisms by which a varicocele (an abnormal dilation of the spermatic veins within the scrotum) can cause abnormalities of the testis and semen parameters by increased scrotal temperature, reflux of toxic metabolites, decreased volume of blood flow, and anoxia. 34 Despite the above hypotheses relating pre-and postnatal compromise to the fetal varix formation and its For AFP and hCG: comparison of mean log 10 for each marker.…”