Intrahepatic portal venous systems in adult patients with cavernous transformation of portal vein: Imaging features and a new classification.

BACKGROUND: Cavernous transformation of the portal vein (CTPV) due to portal vein obstruction is a rare vascular anomaly defined as the formation of multiple collateral vessels in the hepatic hilum. This study aimed to investigate the imaging features of intrahepatic portal vein in adult patients with CTPV and establish the relationship between the manifestations of intrahepatic portal vein and the progression of CTPV. METHODS: We retrospectively analyzed 14 CTPV patients in Beijing Tsinghua Changgung Hospital. All patients underwent both direct portal venography (DPV) and computed tomography angiography (CTA) to reveal the manifestations of the portal venous system. The vessels measured included the left portal vein (LPV), right portal vein (RPV), main portal vein (MPV) and the portal vein bifurcation (PVB). RESULTS: Nine males and 5 females, with a median age of 40.5 years, were included in the study. No significant difference was found in the diameters of the LPV or RPV measured by DPV and CTA. The visualization in terms of LPV, RPV and PVB measured by DPV was higher than that by CTA. There was a significant association between LPV/RPV and PVB/MPV in term of visibility revealed with DPV (P = 0.01), while this association was not observed with CTA. According to the imaging features of the portal vein measured by DPV, CTPV was classified into three categories to facilitate the diagnosis and treatment. CONCLUSIONS: DPV was more accurate than CTA for revealing the course of the intrahepatic portal vein in patients with CTPV. The classification of CTPV, that originated from the imaging features of the portal vein revealed by DPV, may provide a new perspective for the diagnosis and treatment of CTPV.

Effectiveness of Preoperative Intrahepatic Portal Venous Classification System in Guiding Preoperative Surgical Decisions and Predicting Hypotensive Effects After Meso-rex Bypass for Children With EHPVO.

BACKGROUND: The categorization of intrahepatic portal venous system (IHPS) patterns using wedged hepatic venous portography (WHVP) has proven to be an effective tool in the preoperative evaluation of Rex recessus and in identifying pediatric patients with extrahepatic portal vein obstruction (EHPVO) who are suitable for meso-Rex bypass (MRB). Despite this classification system being proposed a decade ago, its clinical application remains underutilized. METHODS: A single-center retrospective study of 182 children with EHPVO was conducted between October 2014 and July 2023 when MRB was attempted. Data on demographics, etiology, imaging examinations, procedures, and follow-up were collected for 161 patients included. Two interventional radiologists used deVille's method to classify patients into types A to E based on WHVP imaging, with interobserver agreement analyzed. Associations between IHPS patterns and surgical outcomes following MRB were investigated. RESULTS: Two radiologists had a high level of agreement on identifying IHPS patterns and suitable patients for MRB. Of the 161 cases, 130 were type A, 10 were type B, 5 were type C, 7 were type D, and 9 were type E. One hundred and forty-five patients with types A, B and C underwent successful MRB, showing feasibility for 90% of patients. Children categorized as types A and B experienced more significant benefits than type C, including intraoperatively decreased portal vein pressure, esophageal/gastric varices relief, decreased portal venous collaterals and a lower rate of bypass occlusion after one year. The surgical outcomes of patients with types A and B were not influenced by the diameter of the Rex recessus as suggested by WHVP. CONCLUSIONS: The majority of pediatric patients with EHPVO in mainland China have opportunities to receive successful MRB. There are potential differences in the etiology of Chinese and Western patients. The IHPS classification system aids in guiding preoperative surgical decisions and predicting hypotensive effects after MRB. Type C patients should be carefully chosen for MRB.

Congenital absence of the portal vein: translated version.

Congenital portosystemic shunt (CPS) is classified into type I (congenital absence of the portal vein) and type II, depending on the presence or absence of the intrahepatic portal vein. Reports still exist on cases in which psychiatric diseases or mental retardation was diagnosed or cases in which diagnosis and treatment are not performed until severe pulmonary complications occur, indicating the need to recognize CPS. Herein, we reviewed epidemiology, etiology, classification, symptom, diagnosis, and treatment based on clinical view points of CPS. For clinical view points, classification according to (1) the presence or absence of the intrahepatic portal venous system (IPVS) and (2) the extrahepatic or intrahepatic of shunt sites, facilitates the understanding of pathophysiological conditions and is useful in selecting a treatment for symptomatic CPS. Radiological and pathological examinations are important in IPVS evaluations, and IPVS evaluations are currently essential to make diagnoses by portography with balloon occlusion and liver biopsy. Symptomatic CPS (hepatic nodular lesions, portosystemic encephalopathy, and pulmonary complications, etc.) is an indication of treatment by shunt closure, but an indication of treatment for asymptomatic CPS is the challenge going forward.