Percutaneous transhepatic treatment of a unique portal vein malformation with portal hypertension in a pediatric patient

Paolo Marra,Sandro Sironi,Claudia Pellegrinelli,Lorenzo D’Antiga,Ludovico Dulcetta

doi:10.1186/s42155-021-00239-1

Abstract

BackgroundAnomalies of the portal venous system can be congenital or acquired, the latter being related to spontaneous thrombosis or iatrogenic alterations such as complications of perinatal catheterization of the umbilical vein. These conditions can be clinically silent for years and then manifest abruptly causing severe clinical emergencies.Case presentationThis case report describes the diagnosis and interventional management of a singular abnormality in the portal venous system of an 8-year-old female that led to severe portal hypertension and acute variceal bleeding. Peculiar imaging findings were not pathognomonic for any of the known congenital and acquired portal vein anomalies: absence of a normal extrahepatic portal vein; splenic and mesenteric veins merging into a dilated left gastric vein; presence of an aberrant mesenteric venous collateral with a stenotic connection with the intrahepatic right portal branch; and absence of porto-systemic shunt. The case was successfully managed with percutaneous transhepatic portography and angioplasty.ConclusionsPrompt non-invasive imaging characterization allowed to understand the singular vascular abnormality and mini-invasive interventional radiology management resolved portal hypertension and variceal bleeding.

Highlights

Anomalies of the portal venous system can be congenital or acquired, the latter being related to spontaneous thrombosis or iatrogenic alterations such as complications of perinatal catheterization of the umbilical vein
Percutaneous venography of the splanchnic venous system confirmed the stenosis at the confluence between the mesenteric venous collateral and the right portal branch, with retrograde flow to the gastric vein and esophageal varices (Fig. 2a)
The 4 Fr introducer was Conclusions Developmental anomalies and acquired alterations of the portal venous system lead to a wide spectrum of uncommon clinical conditions

Summary

Conclusions

Developmental anomalies and acquired alterations of the portal venous system lead to a wide spectrum of uncommon clinical conditions. We describe a unique and singular case which has some clinical features common to PVT In this case portal hypertension was not associated with ascites, hypersplenism or altered liver morphology: we hypothesize this was due to absence of liver disease and to the peculiar vascular anatomy, with spontaneous portal system decompression through an enlarged left gastric vein; reduced by the stenosis, liver flow was probably enough. The case presents peculiar aspects suggesting a possible developmental anomaly: we hypothesize the persistence of the caudal ventral anastomosis of the right vitelline vein that may have compensated a portal vein agenesis or embryonic thrombosis. To our knowledge this vascular abnormality has been never reported with this clinical presentation.

Background