Reverse end-diastolic flow in a fetus with a rare liver malformation: a case report

Abstract

IntroductionWe describe a case of early and persistent reverse end-diastolic flow in the middle cerebral artery in a fetus with severe ascites. These features are associated with a rare liver malformation known as ductal plate malformation.Case presentationA 28-year-old Caucasian woman was referred to our high-risk obstetric unit at 24 weeks' gestation for fetal ascites detected during a routine ultrasound examination. During her hospitalization we performed medical investigations, including a fetal paracentesis, to detect the etiology of fetal ascites. The cause of fetal ascites (then considered non-immune or idiopathic) was not evident, but a subsequent ultrasound examination at 27 weeks' gestation showed a reverse end-diastolic flow in the middle cerebral artery without any other Doppler abnormalities. A cesarean section was performed at 28 weeks' gestation because of the compromised fetal condition. An autopsy revealed a rare malformation of intrahepatic bile ducts known as ductal plate malformation.ConclusionPersistent reverse flow in the middle cerebral artery should be considered a marker of adverse pregnancy outcome. We recommend careful ultrasound monitoring in the presence of this ultrasonographic sign to exclude any other cause of increased intracranial pressure. To better understand the nature of these ultrasonographic signs, additional reports are deemed necessary. In fact in our case, as confirmed by histopathological examination, the fetal condition was extremely compromised due to failure of the fetal liver. Ductal plate malformation altered the liver structures causing hypoproteinemia and probably portal hypertension. These two conditions therefore explain the severe hydrops that compromised the fetal situation.

Persistent reversed end‐diastolic flow of the middle cerebral artery: A rare and concerning finding

“…So far, only a few reports are similar to our case. 2,6,8,12,15 This reversed flow has been reported as a rare finding related to pathological fetal conditions, such as fetal growth restriction, 3,4,7,10,11,13 fetomaternal hemorrhage, 1,5,9 and congenital hydrocephalus. 14 Some cases had a transient period of reversed flow, which was possibly from temporary extracranial compression such as increased transducer compression or anhydramnios/oligohydramnios.…”

Persistent reversed end diastolic flow in the fetal middle cerebral artery: an ominous finding

“…Rarely, persistent MCA-REDF occurs and there are currently only six reported cases in the literature (Table 1). 1–6 These cases were caused by excessive intracranial pressure resulting from intracranial hemorrhage, 2 severe anaemia 1,5 or severe fetal growth restriction, 3,6 and a rare intrahepatic bile duct malformation. 4 All reported cases have had devastating outcomes with fetal or neonatal death occurring in four of the six cases (Table 1).…”

“…1–6 These cases were caused by excessive intracranial pressure resulting from intracranial hemorrhage, 2 severe anaemia 1,5 or severe fetal growth restriction, 3,6 and a rare intrahepatic bile duct malformation. 4 All reported cases have had devastating outcomes with fetal or neonatal death occurring in four of the six cases (Table 1). 1,2,4,6 One of the survivors suffered a grade III intraventricular hemorrhage and periventricular leukomalacia.…”

“…4 All reported cases have had devastating outcomes with fetal or neonatal death occurring in four of the six cases (Table 1). 1,2,4,6 One of the survivors suffered a grade III intraventricular hemorrhage and periventricular leukomalacia. 5 Neonatal outcomes were not recorded for the other survivor.…”

“…1 Persistent MCA-REDF is rare and there have only been six reported cases in the literature. [1][2][3][4][5][6] These cases have been associated with fetal growth restriction, anaemia, intraventricular hemorrhage, and a rare hepatic anomaly, which likely cause increased intra-cerebral pressure and result in the ultrasound finding of persistent MCA-REDF. All documented cases have resulted in devastating outcomes of fetal or neonatal death and disability.…”