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

Background 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. These conditions can be clinically silent for years and then manifest abruptly causing severe clinical emergencies. Case presentation This 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. Conclusions Prompt non-invasive imaging characterization allowed to understand the singular vascular abnormality and mini-invasive interventional radiology management resolved portal hypertension and variceal bleeding.

Preoperative Evaluation of Endoscopic Ultrasonography and Portography in Selecting Devascularization Surgery for Esophagogastric Varices

This study assesses the role of endoscopic ultrasonography (EUS) and portography in selection of mode of devascularization surgery for esophagogastric varices (EGV) in patients with portal hypertension and reports our experience in the treatment of EGV with modified devascularization surgery. Forty-two cirrhotic patients with EGV were treated with devascularization surgery for variceal hemorrhage. Preoperatively, percutaneous transhepatic portography (PTP) and EUS were used as the guiding mode for therapy of EGV. In addition to devascularization and splenectomy, esophageal transection (ET) was performed in 26 patients with upward-flow varices (UFV), whereas 16 patients with downward-flow varices (DFV) underwent proximal gastrectomy instead of ET. In both UFV and DFV groups, grade II of intramural varices and extramural collaterals were most commonly observed on EUS imaging. There was no significant difference of EUS grading between these two groups ( P > 0.05). The incidence of postoperative complications, mortality, and recurrent varices were not significantly different between these two groups. The overall 5-year survival rate for UFV group was 69.2 per cent, whereas that for the DFV group was 68.7 per cent ( P > 0.05). Our study shows that devascularization surgery is highly effective for the prevention of recurrent bleeding from EGV, and it provides an alternative treatment modality. Combined PTP and EUS are very helpful in determining adequate modalities of devascularization surgery.