Drainage of ascites in cirrhosis.

For cirrhotic refractory ascites, diuretics combined with albumin and vasoactive drugs are the first-line choice for ascites management. However, their therapeutic effects are limited, and most refractory ascites do not respond to medication treatment, necessitating consideration of drainage or surgical interventions. Consequently, numerous drainage methods for cirrhotic ascites have emerged, including large-volume paracentesis, transjugular intrahepatic portosystemic shunt, peritoneovenous shunt, automated low-flow ascites pump, cell-free and concentrated ascites reinfusion therapy, and peritoneal catheter drainage. This review introduces the advantages and disadvantages of these methods in different aspects, as well as indications and contraindications for this disease.

Enhancing transjugular intrahepatic portosystemic shunt procedure efficiency with digital subtraction angiography image overlay technology in esophagogastric variceal bleeding.

BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is a pivotal intervention for managing esophagogastric variceal bleeding in patients with chronic hepatic schistosomiasis. AIM: To evaluate the efficacy of digital subtraction angiography image overlay technology (DIT) in guiding the TIPS procedure. METHODS: We conducted a retrospective analysis of patients who underwent TIPS at our hospital, comparing outcomes between an ultrasound-guided group and a DIT-guided group. Our analysis focused on the duration of the portosystemic shunt puncture, the number of punctures needed, the total surgical time, and various clinical indicators related to the surgery. RESULTS: The study included 52 patients with esophagogastric varices due to chronic hepatic schistosomiasis. Results demonstrated that the DIT-guided group experienced significantly shorter puncture times (P < 0.001) and surgical durations (P = 0.022) compared to the ultrasound-guided group. Additionally, postoperative assessments showed significant reductions in aspartate aminotransferase, B-type natriuretic peptide, and portal vein pressure in both groups. Notably, the DIT-guided group also showed significant reductions in total bilirubin (P = 0.001) and alanine aminotransferase (P = 0.023). CONCLUSION: The use of DIT for guiding TIPS procedures highlights its potential to enhance procedural efficiency and reduce surgical times in the treatment of esophagogastric variceal bleeding in patients with chronic hepatic schistosomiasis.

Research status and hotspots in transjugular intrahepatic portosystemic shunts based on CiteSpace bibliometric analysis.

BACKGROUND: The transjugular intrahepatic portosystemic shunt (TIPS) is an important technique for treating complications related to portal hypertension in patients with cirrhosis, and the number of publications in the TIPS field continues to rise. AIM: To facilitate an understanding of the research status and hotspots in the field of TIPS using CiteSpace bibliometric analysis. METHODS: CiteSpace is a software that depicts the strength of relationships through graphics and connections with diverse functionalities and can be used to analyze the status and hotspots of areas of research. Articles on TIPS in the Web of Science Core Collection were retrieved, and CiteSpace software was used to visualize and analyze the number of publications, journals, countries, institutions, authors, keywords, and citations. RESULTS: A total of 985 relevant documents were included in the analysis. From January 2013 to December 2022, the number of publications increased annually. The journal, institution, and author with the greatest number of publications in the field of TIPS are the Journal of Vascular and Interventional Radiology, the University of Bonn, and Jonel Trebicka, respectively. The main keywords used in this field are "transjugular intrahepatic portosystemic shunt", "portal hypertension", "cirrhosis", "management", "stent", "hepatic encephalopathy", "refractory ascite", "survival", "risk", and "variceal bleeding". The greatest obstacle to TIPS placement is currently the occurrence of hepatic encephalopathy. The research hotspots are the mechanism, risk factors, management, and control of hepatic encephalopathy. CONCLUSION: This bibliometric analysis reported the research status and hotspots of TIPS. Research on postoperative hepatic encephalopathy is the research hotspot in this field.

Physiological and behavioural implications of the portosystemic shunt in C57Bl/6J mice.

A significant fraction of the popular inbred C57Bl/6J mice show structural and biochemical features of the congenital portosystemic shunt (PSS). How this hepatic abnormality affects physiological and behavioural parameters has not been explored in detail. Here, we confirmed the frequent occurrence of the PSS in C57Bl/6J mice by three different methods. We screened a cohort of 119 C57Bl/6J mice for total bile acids (TBA) in plasma, identified 11 animals (9.2%) with high TBA (>11 µm; 171.1 ± 76.8 µm), and confirmed PSS presence in that subset by magnetic resonance angiography and 1H-magnetic resonance spectroscopy of brain metabolites in the hippocampal area. In addition to the high glutamine and low myo-inositol levels, we detected lower levels of several neurotransmitters and metabolites in the hippocampus, higher brain weight and volume, as well as enhanced brain glucose utilisation in the PSS mice. We also observed differences in peripheral organ weights, haematological cell counts and clinical chemistry parameters in C57Bl/6J mice with and without PSS. Animals with PSS were slightly hyperlocomotive, had better balance on the rotarod, showed altered gait properties, and displayed attenuated fear memory in the fear conditioning test. Furthermore, we revealed a significant alteration of the pharmacokinetic profile of diazepam in C57Bl/6J mice with PSS. Our data support previous reports of hepatic disturbances and demonstrate an altered neurobiological phenotype in C57Bl/6J mice with PSS. Such congenital differences between inbred C57Bl/6J littermates may significantly distort experimental outcomes of pharmacological, behavioural and genetic studies. KEY POINTS: A significant proportion of C57Bl/6J mice, an inbred strain popular in preclinical research, have congenital portosystemic shunts (PSS) that allow venous blood to enter systemic circulation bypassing the liver. In this study, we extended existing knowledge of PSS consequences, particularly with respect to the effects on brain structure and function. We demonstrated that C57Bl/6J mice with PSS differ from their normal counterparts in brain size and contents of several neuroactive substances, as well as in peripheral organ weights, rate of glucose utilisation, blood cell counts and blood clinical chemistry parameters. C57Bl/6J mice with PSS showed altered locomotor behaviour, performed worse in a memory test and had abnormal blood pharmacokinetics of a benzodiazepine drug after a single administration. PSS presence may significantly complicate the interpretation of experiments in C57Bl/6J mice; therefore, we propose that before their use in biomedical studies, these mice should be screened with a simple blood test.

Living Donor Liver Transplantation for Congenital Portosystemic Shunt Presenting With Hyperinsulinemic Hypoglycemia.

BACKGROUND: A congenital portosystemic shunt (CPSS) is defined as abnormal vascular communications between the portal vein and the systemic vein. Encephalopathy, hepatopulmonary syndrome, and portopulmonary hypertension are manifestations in patients with CPSS. Hyperinsulinemic hypoglycemia is also one of the manifestations of CPSS. Hyperinsulinemic hypoglycemia secondary to CPSS is caused by a lack of hepatic first-pass elimination of insulin, which is secreted from pancreatic beta cells. CASE PRESENTATION: A 7-month-old boy had hypergalactosemia detected by newborn mass screening. Enhanced abdominal computed tomography showed the absence of the portal vein trunk and extrahepatic portosystemic communication between the superior mesenteric vein and the inferior vena cava. He had suffered from uncontrollable hyperinsulinemic hypoglycemia under protein and lactose restriction. We performed living donor liver transplantation (LDLT) using a left lateral segment graft from his father. The postoperative course was uneventful and the hypoglycemic attacks disappeared. CONCLUSION: We believe that uncontrolled hyperinsulinemic hypoglycemia secondary to CPSS is an indication of LDLT.

Transhepatic Antegrade Gastric Variceal Sclerotherapy: Comparing Outcomes with and without Initial Efferent Vein Embolization.

PURPOSE: To compare the efficacies of percutaneous transhepatic obliteration (PTO) with initial afferent vein embolization (PTO-A) and with initial efferent vein embolization (PTO-E) for gastric varices (GVs). MATERIALS AND METHODS: Consecutive patients undergoing PTO-A or PTO-E for type 1 isolated gastric varices or type 2 gastroesophageal varices between June 2007 and February 2023 were included. PTO-A or PTO-E was indicated when balloon-occluded retrograde transvenous obliteration failed, or there was no portosystemic shunt for the retrograde transvenous approach. PTO-A was performed for patients before March 2009 and for patients with multiple efferent channels from the GVs. PTO-E was attempted for patients after April 2009. Twenty-six patients were included, of whom 18 underwent PTO-A, and 8 underwent PTO-E. In 12 patients, PTO-E was attempted without success, and they underwent PTO-A instead. The primary outcome was successful complete eradication of GVs within 1 week after the procedure. Secondary outcomes were the GV recurrence-free period and overall survival after the procedure. RESULTS: Technical success was achieved in all 26 patients, with a complete GV eradication rate of 33.3% in PTO-A and 100% in PTO-E, significantly higher in PTO-E (p = 0.002). The median recurrence-free period was 8.7 months with PTO-A and 75.1 months with PTO-E; PTO-E had a significantly longer recurrence-free period (p = 0.042). Median overall survival was 37.2 months with PTO-A and 49.9 months with PTO-E, with no significant difference (p = 0.955). CONCLUSION: PTO-E had a better complete eradication rate and longer recurrence-free period than PTO-A for GVs.

Improving Clinical Decisions in IR: Interpretable Machine Learning Models for Predicting Ascites Improvement after TIPS Procedures.

PURPOSE: To evaluate the potential of interpretable machine learning (ML) models to predict ascites improvement in patients undergoing transjugular intrahepatic portosystemic shunt (TIPS) placement for refractory ascites. MATERIALS AND METHODS: In this retrospective study, 218 patients with refractory ascites who underwent TIPS were analyzed. Twenty-nine demographic, clinical, and procedural features were collected. Ascites improvement was defined as reduction of paracentesis need by 50% or more at one-month follow-up. Univariate statistical analysis was performed. Data was split into train and test sets. Feature selection was performed using a wrapper-based sequential feature selection (SFS) algorithm. Two ML models were built using support vector machine (SVM) and CatBoost algorithms. Shapley additive explanations values were calculated to assess interpretability of ML models. Performance metrics were calculated using the test set. RESULTS: Refractory ascites improved in 168(77%) patients. Higher sodium (136mEq/L vs 134mEq/L,p=0.001) and albumin levels (2.91 g/dLvs2.68 g/dL,p=0.03), lower creatinine (1.01 mg/dL vs 1.17 mg/dL,p=0.04), model for end-stage liver disease (MELD) (13 vs 15,p=0.01) and MELD-Na (15 vs 17.5, p=0.002) scores were associated with significant improvement. While, main portal vein puncture was associated with a lower improvement rate (p=0.02). SVM and CatBoost models had accuracy ratios of 83% and 87%, with area under curve values of 0.83 and 0.87, respectively. No statistically significant difference was found between performances of the models in DeLong test (p=0.3). CONCLUSION: According to this study, machine learning models may have potential in patient selection for TIPS placement by predicting the improvement in refractory ascites.

A case of the Abernethy malformation: An exceptionally rare clinical entity.

I review a case of a 30-year-old woman who presented with diarrhea, nausea and vomiting for several days. She was ultimately diagnosed with acute gastroenteritis but was incidentally found to have a congenital extrahepatic portosystemic shunt, also known as the Abernethy malformation. The Abernethy malformation, first described by Dr John Abernethy in the year 1793, is an exceptionally rare clinical entity.

Optimal timing for TIPS and PSE combination treatment in patients with cirrhosis-related variceal bleeding and hypersplenism.

RATIONALE AND OBJECTIVES: A consensus has not yet been reached regarding the optimal timing for the combination of transjugular intrahepatic portosystemic shunt (TIPS) and partial splenic embolization (PSE) in patients with cirrhosis-related variceal bleeding and hypersplenism. This study aimed to compare the clinical outcomes of patients who underwent either an early or late combination of TIPS and PSE. METHODS: A total of 84 consecutive patients with cirrhosis-related variceal bleeding and hypersplenism who underwent TIPS and PSE between September 2016 and April 2023 were included in this retrospective multicenter study. These patients were subsequently divided into early combination (n = 36) and late combination (n = 48) groups based on the timing of the combination therapy. RESULTS: Kaplan-Meier curves revealed a significant increase in cumulative survival in the late combination group, compared with that in the early combination group (log-rank P = 0.018). Additionally, the late combination group exhibited a lower cumulative incidence of overt hepatic encephalopathy (OHE), compared with the early combination group (log-rank P = 0.002). In Cox regression models, noninfarcted splenic volume (hazard ratio [HR] = 0.995, 95% confidence interval [CI] = 0.991-0.999, P = 0.044) and grouping (HR = 0.101, 95% CI = 0.011-0.921, P = 0.034) were identified as independent risk factors for mortality. Furthermore, the independent risk factors for OHE were serum albumin (ALB) level (P = 0.032) and grouping (P = 0.028). CONCLUSION: The early combination of TIPS and PSE was associated with higher risks of death and OHE than the late combination.

Recompensation after TIPS reduces the incidence of hepatocellular carcinoma and increases survival in patients with cirrhosis.

BACKGROUND AND AIMS: It has been described that recompensation can improve prognosis in patients with cirrhosis. However, recompensation after transjugular intrahepatic portosystemic shunt (TIPS) has not been studied. We evaluated the impact of recompensation after TIPS on the risk of hepatocellular carcinoma (HCC) and death, and we compared it with compensated cirrhosis patients. METHODS: An observational study of consecutive patients with cirrhosis undergoing TIPS between 2008 and 2022 was performed. Baveno VII definition of recompensation was used including patients with or without diuretics/Hepatic encephalopathy prophylaxis. A prospective cohort of consecutive compensated cirrhosis patients was used for comparison. RESULTS: Overall, 208 patients with cirrhosis were included, 92 compensated and 116 decompensated who underwent TIPS. After 1 year, 24% achieved recompensation. Liver function (MELD 12 ± 5 vs. 15 ± 6; p = .049), LDL-cholesterol (97 mg/dL vs. 76 mg/dL, p = .018), white cell count (7.96 × 109/dL vs. 6.24 × 109/dL, p = .039) and platelets (129 × 109/dL vs. 101 × 109/dL, p = .039) were associated with recompensation. Recompensation was associated with a reduction in the risk of HCC (p = .020). Multivariable analysis showed that this risk was significantly higher in non-recompensated patients (p = .003) but no differences were observed in recompensated compared with compensated patients (p = .816). Similarly, decompensated patients presented lower survival rates (p = .011), while no differences were observed between recompensated and compensated patients (p = .677). CONCLUSIONS: Recompensation after TIPS has a clear impact on the incidence of HCC and death, with a similar prognosis than patients with compensated cirrhosis. Liver function is associated with recompensation, suggesting the importance of considering early TIPS in patients with indication.

Hepatic recompensation according to the Baveno VII criteria via a transjugular intrahepatic portosystemic shunt: Is this true?

Hepatic recompensation is firstly described in the Baveno VII criteria, which requires the fulfillment of strict criteria. First, a primary cause of cirrhosis must be addressed, suppressed, or cured. Second, complications of liver cirrhosis, including ascites, encephalopathy, and variceal hemorrhage, must disappear without any intervention. Finally, liver function indicators must be improved. Moreover, without addressing/suppressing/curing cirrhosis and improvement in liver synthetic function, complications, including ascites and variceal hemorrhage can be improved by a transjugular intrahepatic portosystemic shunt (TIPS), which is not evidence of hepatic recompensation. Therefore, on the basis of the definition of hepatic recompensation, TIPS does not achieve hepatic recompensation.

The Freiburg Index of Post-TIPS Survival accurately predicts mortality in patients with acute decompensation of cirrhosis.

INTRODUCTION: The recently developed Freiburg Index of Post-TIPS Survival (FIPS) allows improved risk classification of patients with decompensated cirrhosis allocated to transjugular intrahepatic portosystemic shunt (TIPS) implantation. This study investigated the prognostic value of the FIPS in patients hospitalized with acute decompensation of cirrhosis (AD), outside the setting of TIPS implantation. METHODS: A total of 1133 patients with AD were included in a retrospective, multi-centre study. Ninety-day, 180-day and 1-year mortality were recorded and the FIPS' performance in predicting mortality at these time points was analysed using ROC analyses. RESULTS: Ninety-day, 180-day and 1-year mortality were 17.7%, 24.4% and 30.8%. Uni- and multivariable Cox regression models showed that the FIPS independently predicted 1-year mortality in the study cohort (HR 1.806, 95% CI 1.632-1.998, p < .0001). In ROC analyses, the FIPS offered consistently high performance in the prediction of mortality within 1 year after AD (area under the receiver operator characteristic [AUROC]: 1-year mortality .712 [.679-.746], 180-day mortality .740 [.705-.775] and 90-day mortality .761 [.721-.801]). In fact, in the subgroup of patients presenting with variceal bleeding, the FIPS even showed significantly improved discriminatory performance in the prediction of long-term mortality (AUROC 1-year mortality: .782 [.724-.839]) in comparison with established prognostic scores, such as the CLIF-C AD score (.724 [.660-.788], p = .0071) or MELD 3.0 (.726 [.662-.790], p = .0042). CONCLUSIONS: The FIPS accurately predicts mortality in patients with AD and seems to offer superior prognostication of long-term mortality in patients with variceal bleeding.

Effects of Transjugular Intrahepatic Portosystemic Shunt on Renal and Pulmonary Function in Hepatic Decompensation with and without Hepatorenal and Hepatopulmonary Syndromes: A Review.

Cirrhosis is often characterized by decreased liver function, ranging from a compensated, typically asymptomatic phase to a decompensated phase characterized by the appearance of ascites or variceal bleeding, and ultimately hepatorenal syndrome (HRS) or hepatopulmonary syndrome (HPS). The latter two complications are associated with a poor prognosis and limited treatment efficacy. In cases of ascites or variceal bleeding resistant to medical therapy, transjugular intrahepatic portosystemic shunt (TIPS) is effective and safe. Shunting blood by TIPS diverts portal blood to the systemic circulation, potentially increasing systemic blood volume and benefiting renal function. However, TIPS could also divert nitric oxide to the systemic circulation, potentially worsening systemic hypotension and perfusion, which could be detrimental to renal function. Available evidence indicates that TIPS often improves renal function in patients with portal hypertension, with or without HRS. No studies have shown persistently decreased renal function after TIPS. However, these data are insufficient to support a recommendation for the use of TIPS specifically for HRS. In patients without pre-existing HPS, TIPS does not appear to significantly affect pulmonary gas exchange. Results of TIPS in HPS have been inconsistent; some studies have shown improvement, but effects were transient. No studies have shown a persistent decline in pulmonary function after TIPS. The evidence supports the need for large randomized controlled trials to investigate the beneficial effects of TIPS for HRS. Similar pulmonary function data are less clear regarding TIPS for HPS. The aim of the current report was to review the literature regarding the effects of TIPS on renal and pulmonary function in hepatic decompensation, with or without the development of HRS or HPS.

Enhancing Cone-Beam CT Image Quality in TIPSS Procedures Using AI Denoising.

Purpose: This study evaluates a deep learning-based denoising algorithm to improve the trade-off between radiation dose, image noise, and motion artifacts in TIPSS procedures, aiming for shorter acquisition times and reduced radiation with maintained diagnostic quality. Methods: In this retrospective study, TIPSS patients were divided based on CBCT acquisition times of 6 s and 3 s. Traditional weighted filtered back projection (Original) and an AI denoising algorithm (AID) were used for image reconstructions. Objective assessments of image quality included contrast, noise levels, and contrast-to-noise ratios (CNRs) through place-consistent region-of-interest (ROI) measurements across various critical areas pertinent to the TIPSS procedure. Subjective assessments were conducted by two blinded radiologists who evaluated the overall image quality, sharpness, contrast, and motion artifacts for each dataset combination. Statistical significance was determined using a mixed-effects model (p ≤ 0.05). Results: From an initial cohort of 60 TIPSS patients, 44 were selected and paired. The mean dose-area product (DAP) for the 6 s acquisitions was 5138.50 ± 1325.57 µGy·m2, significantly higher than the 2514.06 ± 691.59 µGym2 obtained for the 3 s series. CNR was highest in the 6 s-AID series (p < 0.05). Both denoised and original series showed consistent contrast for 6 s and 3 s acquisitions, with no significant noise differences between the 6 s Original and 3 s AID images (p > 0.9). Subjective assessments indicated superior quality in 6 s-AID images, with no significant overall quality difference between the 6 s-Original and 3 s-AID series (p > 0.9). Conclusions: The AI denoising algorithm enhances CBCT image quality in TIPSS procedures, allowing for shorter scans that reduce radiation exposure and minimize motion artifacts.

Transjugular antegrade transvenous obliteration, with and without portal decompression, for management of rectal variceal hemorrhage.

PURPOSE: To report antegrade transvenous obliteration, with or without concurrent portosystemic shunt creation, for the treatment of hemorrhagic rectal varices. MATERIALS AND METHODS: Eight patients, including five (62.5%) females and three (37.5%) males, with mean age of 55.8 ± 13.8 years (range: 30-70 years), underwent transjugular-approach antegrade transvenous obliteration of rectal varices, with or without portosystemic shunt creation. Demographic data, procedural details, technical success of variceal obliteration, clinical success, adverse events, and follow-up outcomes were retrospectively recorded. Clinical success was defined as resolution of rectal hemorrhage. RESULTS: Portal venous access was achieved via a transjugular intrahepatic approach in all patients. The inferior mesenteric vein was selected, and foamy sclerosant (1:2:3 mixture by volume of ethiodized oil: sodium tetradecyl sulfate: air) was injected into the rectal varices with antegrade balloon occlusion in seven (87.5%) and without balloon occlusion in one (12.5%). Five of eight (62.5%) patients underwent concomitant transjugular intrahepatic portosystemic shunt (TIPS) creation (mean diameter 8.4 ± 0.9-mm) immediately following transvenous obliteration. Technical success of variceal obliteration was achieved in all patients. There were no immediate post-procedural adverse events. There were no reported occurrences of rectal ischemia, perforation, or stricture following obliteration. Two (40%) of the patients who underwent concomitant TIPS creation developed hepatic encephalopathy within 30 days of the procedure, which was medically managed. Clinical resolution of hemorrhage was achieved in all patients with no recurrent rectal variceal hemorrhage during mean follow-up of 666 ± 396 days (range: 14 - 1,224 days). CONCLUSION: Transvenous obliteration, with or without concurrent TIPS creation, is feasible with promising results for the management of rectal variceal hemorrhage.

Abernethy Malformation and Gastrointestinal Bleeding: A Case Report and Literature Review.

Background: Congenital extrahepatic portosystemic shunt (CEPS), also termed Abernethy malformation (AF) is a rare anomaly of the splanchnic venous system. Several approaches, including shunt closures through surgical or radiological interventions and liver transplantations, have been proposed, but clear comparisons among different treatment strategies are still unavailable. Purpose: We report a case in which an unusual portosystemic shunt was present between the dilated inferior mesenteric vein (IMV) to the right ovarian vein. A mini literature review of AF patients presented with gastrointestinal (GI) tract bleeding. Research design: Case report and literature review. Data Collection: An electronic search of PubMed was performed from inception to December 2023. Results: 34 AF patients presented with GI tract bleeding were identified published in the literature. The proportion of type II AF patients presenting with GI bleeding is greater (79%). Conclusions: We regard that both surgical ligation and endovascular closure of the shunt are effective and safe treatments for these patients, but coils embolization alone may not be sufficient to completely close the shunt when the shunt flow is high.

Road to recompensation: Baveno VII criteria and transjugular intrahepatic portosystemic shunt in liver cirrhosis.

Liver cirrhosis has long been considered a point of no return, with limited hope for recovery. However, recent advancements, particularly the Baveno VII criteria and the utilization of transjugular intrahepatic portosystemic shunt (TIPS), have illuminated the concept of hepatic recompensation. In this editorial we comment on the article by Gao et al published in the recent issue. This editorial provides a comprehensive overview of the evolution of understanding cirrhosis, the criteria for recompensation, and the efficacy of TIPS in achieving recompensation. We discuss key findings from recent studies, including the promising outcomes observed in patients who achieved recompensation post-TIPS insertion. While further research is needed to validate these findings and elucidate the mech-anisms underlying recompensation, the insights presented here offer renewed hope for patients with decompensated cirrhosis and highlight the potential of TIPS as a therapeutic option in their management.

Computed tomographic features of segmental aplasia of the caudal vena cava, portocaval shunt and Situs ambiguous in two dogs.

A 3-year-old crossbreed dog (case 1) and a 3-month-old German Shorthaired Pointer (case 2) were presented for acute signs of encephalopathy. A portosystemic shunt (PSS) was suspected based on clinical context and laboratory exam results and was confirmed on computed tomography (CT) angiography in both cases. A left-sided azygos (case 1) and right-sided azygos (case 2) continuation of an interrupted caudal vena cava (CVC) and a situs ambiguous (SA) were also observed and considered as incidental findings. Both dogs underwent PSS surgical correction. Postsurgical follow-up imaging procedures suggested concomitant primary hypoplasia of the portal vein (PHPV) in both cases.

Interventions in Budd-Chiari syndrome: an updated review.

Budd Chiari syndrome is a potentially treatable disease, and imaging is the key to its diagnosis. Clinical presentations may vary, ranging from asymptomatic to fulminant disease. Subacute BCS is the most common type encountered in clinical practice, characterized by ascites, hepatosplenomegaly, dilated abdominal wall veins, and varicosities in the lower limb and scrotum. While hepatic vein thrombosis is the leading cause in the West, membranous and short segmental occlusion are predominant in the Asian populations. These geographical variations have an impact on the treatment algorithm in managing BCS. Anticoagulation alone often fails to prevent disease progression, demanding further interventional therapy. Interventional therapy carries a lower morbidity and mortality than surgery. Anatomical recanalization and portosystemic shunting form the basis of endovascular management. Membranous or short-segment occlusion are best treated by angioplasty, which restores the physiological venous outflow and possibly disease reversal. Suboptimal results with angioplasty require stenting. Transjugular intrahepatic shunt (TIPS) or direct IVC to portal vein shunt (DIPS) decompresses the portal pressure and reduces the sinusoidal congestion, which in turn diminishes hepatocellular damage and hepatic fibrosis. Despite its ability to modify the disease course, TIPS carries several procedure and shunt-related complications, mainly hepatic encephalopathy. Thus, anatomical recanalization precedes TIPS in the traditional step-up approach in managing BCS. However, this concept is challenged by some authors, necessitating future reseach. TIPS is a valid bridge therapy in BCS with acute live failure awaiting liver transplantation. Despite all, interventional therapies fail in a subset of BCS patients, leaving them with only option of liver transplantation.