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

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Paolo Marra ◽  
Ludovico Dulcetta ◽  
Claudia Pellegrinelli ◽  
Lorenzo D’Antiga ◽  
Sandro Sironi

Abstract 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.

2002 ◽  
Vol 44 (5) ◽  
pp. 261-264 ◽  
Author(s):  
Marcos MUCENIC ◽  
Manoel de Souza ROCHA ◽  
Antônio Atílio LAUDANNA ◽  
Eduardo Luiz Rachid CANÇADO

Portal vein aneurysm is a rare medical entity that can be caused by chronic hepatic diseases with portal hypertension. We describe a 45-year-old man with variceal bleeding from hepatosplenic schistosomiasis and an incidentally found intrahepatic aneurysm. Diagnosis was confirmed with non-invasive imaging exams, arteriography and liver biopsy. Following splenectomy, the aneurysm diameter decreased substantially.


Author(s):  
Mohamed S. Alwarraky ◽  
Hasan A. Elzohary ◽  
Mohamed A. Melegy ◽  
Anwar Mohamed

Abstract Background Our purpose is to compare the stent patency and clinical outcome of trans-jugular intra-hepatic porto-systemic shunt (TIPS) through the left branch portal vein (TIPS-LPV) to the standard TIPS through the right branch (TIPS-RPV). We retrospectively reviewed all patients (n = 54) with refractory portal hypertension who were subjected to TIPS-LPV at our institute (TIPS-LPV) between 2016 and 2018. These patients were matched with 56 control patients treated with the standard TIPS-RPV (TIPS-RPV). The 2 groups were compared regarding the stent patency rate, encephalopathy, and re-interventions for 1 year after the procedure. Results TIPS-LPV group showed 12 months higher patency rate (90.7% compared to 73.2%) (P < 0.005). The number of the encephalopathy attacks in the TIPS-LPV group was significantly lower than that of the TIPS-RPV group at 6 and 12 months of follow-up [P = 0.012 and 0.036, respectively]. Re-bleeding and improvement of ascites were the same in the two groups [P > 0.05]. Patients underwent TIPS-LPV needed less re-interventions and required less hospitalizations than those with TIPS-RPV [P = 0.039 and P = 0.03, respectively]. Conclusion The new TIPS approach is to extend the stent to LPV. This new TIPS-LPV approach showed the same clinical efficiency as the standard TIPS-RPV in treating variceal bleeding and ascites. However, it proved a better stent patency with lower rates of re-interventions, encephalopathy, and hospital admissions than TIPS through the right branch.


2021 ◽  
Vol 27 ◽  
pp. 107602962110109
Author(s):  
Le Wang ◽  
Xiaozhong Guo ◽  
Xiangbo Xu ◽  
Shixue Xu ◽  
Juqiang Han ◽  
...  

Portal venous system thrombosis (PVST), a common complication of liver cirrhosis, is closely associated with thrombophilia. To explore the association of homocysteine (Hcy), anticardiolipin antibody (aCL), and anti-β2 glycoprotein I antibody (aβ2GPI), which are possible thrombophilic factors, with PVST in liver cirrhosis. Overall, 654 non-malignant patients (219 with and 435 without liver cirrhosis) admitted between January 2016 and June 2020 were retrospectively evaluated. Presence of PVST, degree of main portal vein (MPV) thrombosis, and clinically significant PVST were identified. Hcy level, hyperhomocysteinemia (HHcy), aCL positivity, and aβ2GPI positivity were compared according to the presence of liver cirrhosis and PVST. Positive aβ2GPI was significantly more frequent in patients with liver cirrhosis than those without, but Hcy level and proportions of HHcy and positive aCL were not significantly different between them. PVST could be evaluated in 136 cirrhotic patients. Hcy level [10.57 μmol/L (2.71-56.82) versus 9.97 μmol/L (2.05-53.44); P = 0.796] and proportions of HHcy [4/44 (9.1%) versus 13/81 (16.0%); P = 0.413] and positive aCL [1/23 (4.3%) versus 10/52 (19.2%); P = 0.185] and aβ2GPI [9/23 (39.1%) versus 21/52 (40.4%); P = 0.919] were not significantly different between cirrhotic patients with and without PVST. There was still no significant association of Hcy level, HHcy, aCL, or aβ2GPI with PVST based on Child-Pugh classification, MPV thrombosis >50%, and clinically significant PVST. Hcy, aCL, and aβ2GPI may not be associated with PVST in liver cirrhosis, suggesting that routine screening for Hcy, aCL, and aβ2GPI should be unnecessary in such patients.


2020 ◽  
Vol 53 (6) ◽  
pp. 424-429
Author(s):  
Alexandre Makoto Minoda ◽  
Raissa Brito Fernandes Cadete ◽  
Sara Reis Teixeira ◽  
Valdair Francisco Muglia ◽  
Jorge Elias Junior ◽  
...  

Abstract Portal vein thrombosis refers to complete or partial obstruction of the portal venous system, in the intrahepatic or extrahepatic venous tract or even in the splenic or superior mesenteric veins. This common and potentially fatal condition can develop in various clinical contexts, especially those of liver cirrhosis, hepatocellular carcinoma, and other solid tumors. Certain characteristics, such as the time since the onset of the thrombus (acute or chronic), its biology (hematic or tumoral), the presence of collateral vessels, and the magnetic resonance imaging aspects, are important components of a thorough, careful analysis, as well as informing decisions regarding the appropriate therapeutic strategy. Here, we present a brief review of the anatomy of the portal venous system and a systematic approach to analyzing the condition, using a mnemonic (ABCD, for age, biology, collaterals, and diffusion). We discuss the various imaging methods and illustrate our discussion with images selected from the case files archived at our facility.


2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 261-263
Author(s):  
L Tsang ◽  
J Abraldes ◽  
E Wiebe ◽  
G S Sandha ◽  
S van Zanten

Abstract Results A 41-year old Asian male, who immigrated to Canada many years ago, and who had previously been successfully treated for Helicobacter pylori infection underwent gastroscopy for investigation of dyspepsia. His gastroscopy was normal except for a large subepithelial abnormality that was noted close to the gastroesophageal junction. Routine gastric biopsies from the antrum and body were normal. Subsequent endoscopic ultrasound revealed flow through the anechoic tortuous lesion and confirmed it was a very large isolated gastric varix type 1. Abdominal CT scan revealed chronic occlusion of the portal vein, splenic vein, and the portal confluence with extensive collateralization in the upper abdomen. There was complete cavernous transformation of the portal vein. Of the numerous varices in the upper abdomen, a very large varix drained into the left renal vein and indented into the posterior wall of the fundus of the stomach which accounted for the endoscopic finding. Multiple mesenteric veins were identified that connected to varices adjacent to the inferior aspect of the pancreas and duodenum. Notably, there was no evidence of cirrhosis or chronic pancreatitis. Liver enzymes, albumin, and INR were normal. Further collateral history revealed that he was hospitalized as a neonate for pneumonia with catheterization of the umbilical vein, which is known to be associated with thrombosis of the portal vein. Conclusions Detection of congenital absence of the portal vein (CAPV) is recognized more often due to advances in diagnostic imaging. Radiologically, the absence of the portal vein in CAPV is distinguished from portal vein thrombosis by the lack of venous collaterals or sequalae of portal hypertension, such as ascites or splenomegaly. A more gradual thrombosis of the portal vein may permit collaterals to develop without acute changes and is not equivalent to portal vein aplasia or agenesis as intrahepatic bile ducts are normal. The gold standard for diagnosis of CAPV is histologic absence of the portal vein in the liver on catheter angiography. CAPV is associated with abnormal embryologic development of the portal vein and frequently presents with complications of portal hypertension or portosystemic encephalopathy or the sequalae of venous shunts, hepatic or cardiac abnormalities found on imaging. Our case is an incidentally discovered absence of the portal venous system due to chronic thrombosis with extensive collateralization and an enlarged gastric varix protruding into the proximal stomach. It is well documented that canalization of the umbilical vein in infancy is associated with portal vein thrombosis, with incidences up to 68%. This case highlights the importance of eliciting a childhood hospitalization history in cases of non-cirrhotic portal hypertension. Funding Agencies None


Author(s):  
Changchun Liu ◽  
Sizhe Chen ◽  
Xinwen Yan ◽  
Yi Xiang ◽  
Jialiang Hui ◽  
...  

Background: Portal vein velocity (PVV) has shown reasonable correlation with the presence of portal hypertension in patients with cirrhosis. This study aims to evaluate the value of PVV for diagnosing clinically significant portal hypertension (CSPH) and predicting the risk of variceal hemorrhage (VH) in patients with hepatitis B virus (HBV)-related cirrhosis. Material and Methods: A cohort of 166 consecutive adult patients with HBV-related cirrhosis was recruited in this retrospective study from two high-volume liver centers in China between April 2015 and April 2017. The performance of PVV and other non-invasive parameters for diagnosing CSPH and predicting risk of VH were studied. Results: PVV demonstrated the best performance for diagnosing CSPH (defined as an HVPG ≥10 mmHg) in patients with HBV-related cirrhosis among the included noninvasive predictors with the area under the receiver operating characteristic curve (AUC), specificity, and sensitivity of 0.745, 50%, and 93.5%, respectively. Other noninvasive markers, including APRI, AAR, LS, FIB-4, and diameter of portal vein, did not show sufficient performance with the AUCs of 0.565, 0.560, 0.544, 0.529, and 0.474, respectively. With regard to predicting the risk of VH (defined as an HVPG ≥12 mmHg), PPV also exhibited a moderate performance with an AUC of 0.762, which was superior to that of the aforementioned markers. By using two cutoff values of PVV to rule-out (11.65 cm/s) and rule-in (20.20 cm/s) CSPH, 30 (33.7%) patients showed definite results categories, with 23 (76.7%) patients were well classified and 7 (23.3%) were misclassified. Fifty-nine (66.3%) patients were with indeterminate results. By using PVV values of 13.10 cm/s and 21.40 cm/s to rule-out and rule-in HVPG ≥ 12mmHg, 34 (38.2%) patients has definite results, among whom 26 (76.5%) were well classified and 8 (23.5%) were misclassified. And 55 (61.8%) patients required further evaluation. Conclusion: PPV is not good enough to serve as a non-invasive parameter for identifying CSPH and predicting risk of VH in patients with HBV-related cirrhosis.


1980 ◽  
Vol 78 (2) ◽  
pp. 197-205 ◽  
Author(s):  
G. Smith-Laing ◽  
M.E. Camilo ◽  
R. Dick ◽  
S. Sherlock

2013 ◽  
Vol 2013 ◽  
pp. 1-10 ◽  
Author(s):  
Lanning Yin ◽  
Haipeng Liu ◽  
Youcheng Zhang ◽  
Wen Rong

Aim. To compare the effectiveness of surgical procedures (selective or nonselective shunt, devascularization, and combined shunt and devascularization) in preventing recurrent variceal bleeding and other complications in patients with portal hypertension. Methods. A systematic literature search of the Medline and Cochrane Library databases was carried out, and a meta-analysis was conducted according to the guidelines of the Quality of Reporting Meta-Analyses (QUOROM) statement. Results. There were a significantly higher reduction in rebleeding, yet a significantly more common encephalopathy () in patients who underwent the shunt procedure compared with patients who had only a devascularization procedure. Further, there were no significant differences in rebleeding, late mortality, and encephalopathy between selective versus non-selective shunt. Next, the decrease of portal vein pressure, portal vein diameter, and free portal pressure in patients who underwent combined treatment with shunt and devascularization was more pronounced compared with patients who were treated with devascularization alone (). Conclusions. This meta-analysis shows clinical advantages of combined shunt and devascularization over devascularization in the prevention of recurrent variceal bleeding and other complications in patients with portal hypertension.


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