Persistent Ductus Venosus

Clinical History

An 11-year-old female had a history of focal nodular hyperplasia in the left lobe of the liver and was subjected to segmental hepatectomy at the age of 1. She has been asymptomatic to date. Blood tests revealed an increase in haemoglobin levels (16, 8 g/dl), and were otherwise unremarkable.

Imaging Findings

US findings:

Normal sized liver, with diffuse parenchymal heterogeneity and several slightly hyperechoic nodules, the largest measuring 3.2 cm (Fig. 1).

CT Angiography:

Abdominal CT angiography (Fig. 2) was performed with a 16-row multidetector CT (Philips Brilliance), with endovenous administration of 60 ml (2 ml/kg) of non-ionic low-osmolality contrast (2. 5 ml/s flow rate).

Arterial phase imaging revealed an absent celiac trunk; the splenic artery arised directly from the abdominal aorta; the common hepatic artery arised from the superior mesenteric artery.

Portal phase imaging revealed a normal splenic and superior mesenteric vein that join and originate the portal vein. The portal vein crosses the hepatic parenchyma as an engorged vessel that follows an ascendant trajectory towards the right auricle (persistent ductus venosus, Fig. 3).
There are anomalous hepatic veins (Fig. 4), joining near the inferior vena cava diaphragmatic hiatus and draining directly in the right auricle.

Evaluation of the liver parenchyma revealed 5 hypervascular nodules compatible with focal nodular hyperplasia.

Discussion

Congenital Portosystemic shunts may be extrahepatic or intrahepatic.

Extrahepatic portosystemic shunts can be divided in two types (1):

Type 1 – The portal vein is absent; the mesenteric and splenic veins may drain in the IVC independently or through a common trunk;

Type 2 – The portal vein is patent, but some blood flow is diverted to the inferior vena cava through extrahepatic venous branches
Intrahepatic portosystemic shunts have been classified in four types (2):
- a single vessel connects the right portal vein to the inferior vena cava
- one/multiple communications between portal/hepatic veins in one liver segment
- peripheral and hepatic veins are connected through an aneurism
- multiple peripheral communications exist diffusely in the liver parenchyma

A persistent ductus venosus can also be considered in this category.

There is a well-recognized relationship between porto-systemic shunts and nodular liver lesions such as nodular regenerative hyperplasia, focal nodular hyperplasia, adenomatous hyperplasia, and hepatocellular carcinoma (3 -6), most likely due to an abnormal cellular response to lack of portal venous flow (6). These findings correlate well with our case report.

Our patient was subjected to embolization of the ductus venosus with adequate opening of peripheral portal venous branches.

Differential Diagnosis List

Final Diagnosis

Congenital Portosystemic Shunt: Persistent Ductus Venosus.Focal nodular hyperplasia.

URL:	https://www.eurorad.org/case/9662
DOI:	10.1594/EURORAD/CASE.9662
ISSN:	1563-4086