Biliary Complications Post Transjugular Intrahepatic Portosystemic Shunt in a Child With Portal Vein Cavernous Transformation.

The transjugular intrahepatic portosystemic shunt is a rising interventional procedure with multiple indications and high technical success but with risks of biliary injuries, an underreported scenario. We present an 11-year-old patient with biliary injury with a leak, biloma formation, and biliary obstruction caused by the percutaneous procedure. Interventional radiology drainages addressed these complications by resolving the leak and biloma. These biliary complications in percutaneous procedures and their management are rarely reported in the medical literature, making their management not standard. We highlight drainage management and the importance of sharing it to add experience to this clinical scenario and encourage sharing cases with similar diagnoses.

Membranoproliferative glomerulonephritis in a child with congenital portosystemic shunt.

Congenital portosystemic shunts (CPSS) are rare congenital vascular anomalies characterized by abnormal connections between the portal vein and systemic circulation, bypassing the liver. They can lead to complications such as recurrent encephalopathy, liver nodules, portopulmonary hypertension, and neurocognitive issues due to hyperammonemia and rarely kidney involvement. Hepatic hemodynamic changes can lead to liver nodules and hepatocellular carcinoma, particularly in extrahepatic shunts. We describe here an 11-year-old girl with type 1 intrahepatic portosystemic shunt with focal nodular hyperplasia in the liver, presenting with nephrotic syndrome that was diagnosed as membranoproliferative glomerulonephritis on kidney biopsy and that responded partially to therapy with immunosuppressants.

A rare case of Gynura-segetum-related hepatic sinus obstruction syndrome complicated with alcoholic liver disease.

Hepatic sinus obstruction syndrome (HSOS) is easy to be misdiagnosed or missed, and there is no unified and effective treatment for it. A patient was considered to have Budd-Chiari syndrome. He underwent a transjugular liver biopsy, and pathological examination revealed HSOS without liver cirrhosis. After the failure of anticoagulation therapy, he successfully received a transjugular intrahepatic portosystemic shunt (TIPS). After discharge, he was followed-up for four years with a good prognosis. G. segetum-induced HSOS can be easily overlooked, especially in patients with underlying liver diseases. When medical therapy fails, TIPS can control ascites and portal hypertension, and the long-term prognosis is optimistic.

Advancing hepatic recompensation: Baveno VII criteria and therapeutic innovations in liver cirrhosis management.

The Baveno VII criteria redefine the management of decompensated liver cirrhosis, introducing the concept of hepatic recompensation marking a significant departure from the conventional view of irreversible decline. Central to this concept is addressing the underlying cause of cirrhosis through tailored therapies, including antivirals and lifestyle modifications. Studies on alcohol, hepatitis C virus, and hepatitis B virus-related cirrhosis demonstrate the efficacy of these interventions in improving liver function and patient outcomes. Transjugular intrahepatic portosystemic shunt (TIPS) emerges as a promising intervention, effectively resolving complications of portal hypertension and facilitating recompensation. However, optimal timing and patient selection for TIPS remain unresolved. Despite challenges, TIPS offers renewed hope for hepatic recompensation, marking a significant advancement in cirrhosis management. Further research is needed to refine its implementation and maximize its benefits. In conclusion, TIPS stands as a promising avenue for improving hepatic function and patient outcomes in decompensated liver cirrhosis within the framework of the Baveno VII criteria.

Milestones to optimize of transjugular intrahepatic portosystemic shunt technique as a method for the treatment of portal hypertension complications.

This editorial describes the milestones to optimize of transjugular intrahepatic portosystemic shunt (TIPS) technique, which have made it one of the main methods for the treatment of portal hypertension complications worldwide. Innovative ideas, subsequent experimental studies and preliminary experience of use in cirrhotic patients contributed to the introduction of TIPS into clinical practice. At the moment, the main achievement in optimize of TIPS technique is progress in the qualitative characteristics of stents. The transition from bare metal stents to extended polytetrafluoroethylene-covered stent grafts made it possible to significantly prevent shunt dysfunction. However, the question of its preferred diameter, which contributes to an optimal reduction of portal pressure without the risk of developing post-TIPS hepatic encephalopathy, remains relevant. Currently, hepatic encephalopathy is one of the most common complications of TIPS, significantly affecting its effectiveness and prognosis. Careful selection of patients based on cognitive indicators, nutritional status, assessment of liver function, etc., will reduce the incidence of post-TIPS hepatic encephalopathy and improve treatment results. Optimize of TIPS technique has significantly expanded the indications for its use and made it one of the main methods for the treatment of portal hypertension complications. At the same time, there are a number of limitations and unresolved issues that require further randomized controlled trials involving a large cohort of patients.

Successful Preoperative Transjugular Intrahepatic Portosystemic Shunt for Portal Decompression in Patients With Inflammatory Bowel Disease and Cirrhosis Requiring Surgical Intervention.

Background: Colorectal surgery in patients with inflammatory bowel disease (IBD) and cirrhosis has increased morbidity, which may preclude surgery. Preoperative transjugular intrahepatic portosystemic shunt (TIPS) is postulated to reduce surgical risk. In this retrospective single-center study, we characterized perioperative outcomes in patients with IBD and cirrhosis who underwent preoperative TIPS. Methods: We identified patients with IBD and cirrhosis who had undergone preoperative TIPS for portal decompression between 2010 and 2023. All other indications for TIPS led to patient exclusion. Demographic and medical data were collected, including portal pressure measurements. Primary outcome of interest was perioperative outcomes. Results: Ten patients met the inclusion criteria. The most common surgical indications were dysplasia (50%) and refractory IBD (50%). TIPS was performed at a median of 47 days (IQR 34-80) before surgery, with reduction in portal pressures (22.5 vs. 18.5 mmHg, P < .01) and portosystemic gradient (12.5 vs. 5.5 mmHg, P < .01). Perioperative complications occurred in 80% of patients, including surgical site bleeding (30%), wound dehiscence (10%), systemic infection (30%), liver function elevation (50%), and coagulopathy (50%). No patients required re-operation, with median length of stay being 7 days (IQR 5.5-9.3). The 30-day readmission rate was 40%, most commonly for infection (75%), with 2 patients having intra-abdominal abscesses and 1 patient with concern for bowel ischemia. Ninety-day and one-year survival was 100% and 90%, respectively. Patients with primary sclerosing cholangitis (PSC)-cirrhosis were noted to have higher perioperative morbidity and a 30-day readmission rate. Conclusions: In patients with IBD and cirrhosis, preoperative TIPS facilitated successful surgical intervention despite heightened risk. Nevertheless, significant complications were noted, in particular for patients with PSC-cirrhosis.

Novel Staged Device Occlusion of Portosystemic Shunt With Atrial Flow Regulator in IVC Stent Platform.

We present the cases of 2 children diagnosed with extrahepatic portosystemic shunts, a very rare vascular anomaly, on investigation of cardiac symptomatology. Poorly developed portal venous systems necessitated staged shunt occlusion. This was achieved using atrial flow regulator devices positioned in an inferior vena cava stent platform performed in the cardiac catheterization laboratory.

Management of ectopic variceal bleeding with transjugular intrahepatic portosystemic shunt: a systematic review of case reports.

Ectopic varices account for 5% of variceal bleedings and occur outside the gastro-esophageal region. This review evaluates the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for ectopic variceal management. A comprehensive search through PubMed, Scopus, Web of Science, and Embase was conducted until January 16, 2023, using relevant keywords. Case reports and case series with fewer than 10 patients on TIPS for ectopic variceal management were included. The quality assessment followed the Joanna Briggs Institute checklist for case reports. This systematic review evaluated 43 studies involving 50 patients with ectopic varices undergoing TIPS. Patients had a mean age of 54.3 years, half were female, and two were pregnant. Alcoholic liver disease (48%) and hepatitis C infection (26%) were common causes of portal hypertension. Ascites and splenomegaly were reported in 32% and 28% of the patients, respectively. Rectal, oral, and stomal variceal bleeding accounted for 62%, 16%, and 22% of the patients, respectively. Ectopic varices were mainly located in the duodenum (28%) and rectum (26%) regions. Complications affected 42% of the patients, re-bleeding in eleven and hepatic encephalopathy in seven. The follow-up lasted 12 months on average, and finally, 5 received a liver transplant. Mortality post-TIPS was 18%. Despite complications and a notable mortality rate, favorable outcomes were observed in almost half of the patients with ectopic variceal bleeding managed with TIPS. Further research is warranted to refine strategies and improve patient outcomes.

Comparison of specialized stent versus generic stent and bare stent combination for transjugular intrahepatic portosystemic shunt creation.

Transjugular intrahepatic portosystemic shunt (TIPS) creation using the Viatorr stent remains relatively uncommon in underdeveloped and high-burden disease regions in Asia-Pacific, and there is a lack of comparative studies regarding its prognostic effects compared with the generic stent-graft/bare stent combination. The purpose of this retrospective study is to compare the prognostic endpoints of these two treatments in patients who underwent TIPS creation. Clinical data from 145 patients were collected, including 82 in the combination group and 63 in the Viatorr group. Differences in prognostic endpoints (shunt dysfunction, death, overt hepatic encephalopathy [OHE], rebleeding) between the two groups were analyzed using Kaplan-Meier curves. The Cox proportional hazards model was used to identify independent risk factors for post-TIPS shunt dysfunction. The TIPS procedure was successful in all patients. After TIPS creation, both groups showed a significant decrease in porto-caval pressure gradient compared to that before TIPS creation. The stent patency rates at 6, 12, and 18 months were high in both the combination and Viatorr groups (93.7%, 88.5%, and 88.5% vs. 96.7%, 93.4%, and 93.4%, respectively). The stent patency rates was higher in the combination group than in the Viatorr group, although not statistically significant (HR = 2.105, 95% CI 0.640-6.922, Log-rank P = 0.259). There were no significant differences in other prognostic endpoints (death, OHE, rebleeding) between the two groups. The Cox model identified portal vein diameter (HR = 0.807, 95% CI 0.658-0.990, P = 0.040) and portal vein thrombosis (HR = 13.617, 95% CI 1.475-125.678, P = 0.021) as independent risk factors for post-TIPS shunt dysfunction. The shunt patency rates between the Viatorr stent and the generic stent-graft/bare stent combination showed no significant difference and the generic stent-graft/bare stent combination may be a viable alternative in areas where the Viatorr stent is not yet available.

Liver Transplantation as a Treatment for Unresectable Hepatic Adenoma in a Patient With Abernethy Syndrome.

Abernethy syndrome is a rare congenital anomaly characterized by an intrahepatic or extrahepatic portosystemic shunt. Most patients are asymptomatic; however, due to the alteration in, or lack of, a portovenous flow, patients with Abernethy syndrome are at high risk of developing sequelae of liver failure. Once these complications develop, the only definitive treatment is transplantation. Patients with Abernethy syndrome are also at a higher risk of developing benign and malignant liver lesions, including hepatic adenomas. Here, we describe the first case of deceased donor liver transplantation as a treatment for a patient with type 1 Abernethy syndrome complicated by large, unresectable hepatic adenoma, found to have focal hepatocellular carcinoma on pathologic examination. Our male patient was found to have elevated liver enzymes at age 33, during a routine outpatient medical appointment. Despite being asymptomatic, his history of prior liver resection prompted CT imaging, which revealed two large liver lesions concerning for hepatic adenomas. When surveillance imaging showed a significant growth of the liver lesions, biopsy was pursued, which confirmed a diagnosis of hepatic adenomas. However, given the size of these lesions, resection was not a viable option for the patient. Instead, the patient underwent liver transplantation at age 41, which he tolerated well. Our case demonstrates the utility of deceased donor liver transplantation as a treatment for patients with Abernethy syndrome complicated by unresectable adenomas.

Higher risk of in-hospital mortality and hepatic encephalopathy during weekend admission in Transjugular Intrahepatic Portosystemic Shunt procedure.

OBJECTIVES: Transjugular intrahepatic portosystemic shunt (TIPS) is a procedure used to alleviate portal hypertension in patients with decompensated liver cirrhosis. The weekend effect refers to a higher risk of adverse outcomes associated with procedures performed on weekends compared to weekdays. The goal of this study is to determine whether a weekend effect is evident in TIPS procedures. MATERIALS AND METHOD: The study identified patients who underwent TIPS procedures in the NIS database from 2015 to 2020. Patients who were admitted on the weekday or weekends were classified into two cohorts. Preoperative variables, including demographics, comorbidities, primary payer status, and hospital characteristics, were noted. Multivariable analysis was used to assess outcomes. RESULTS: Compared to patients admitted on the weekdays, weekend patients had higher in-hospital mortality (12.87 % vs. 7.96 %, aOR = 1.62, 95 CI 1.32-1.00, p < 0.01), hepatic encephalopathy (33.24 % vs. 26.18 %, aOR = 1.41, 95 CI 1.23-1.63, p < 0.01), acute kidney injury (39.03 % vs. 28.36 %, aOR = 1.68, 95 CI 1.46-1.93, p < 0.01), and transfer out (15.91 % vs. 12.76 %, aOR=1.33, 95 CI 1.11-1.60, p < 0.01). It was also found that weekend patients had longer wait from admission to operation (3.83 ± 0.15 days vs 2.82 ± 0.07 days, p < 0.01), longer LOS (11.22 ± 0.33 days vs 8.38 ± 0.15 days, p < 0.01), and higher total hospital charge (219,973 ± 7,352 dollars vs 172,663 ± 3,183 dollars, p < 0.01). CONCLUSION: Our research unveiled a significant relationship between weekend admission and a higher risk of mortality and morbidity post-TIPS procedure. Eliminating delays in treatment associated with the weekend effect may mitigate this gap to deliver consistent and high-quality care to all patients.

Unveiling the impact of cirrhotic cardiomyopathy on portal hemodynamics and survival after transjugular intrahepatic portosystemic shunt: a prospective study.

BACKGROUND AND AIMS: The placement of Transjugular intrahepatic portosystemic shunt (TIPS) results in a sudden increase in central circulating blood volume, which requires proper regulation of the cardiovascular system. We aimed to investigate the impact of TIPS on cirrhotic cardiomyopathy (CCM). METHOD: A consecutive case series of patients with cirrhosis who underwent TIPS were evaluated by echocardiography and pressure measurements before, immediately after TIPS and 2-4 days later (delayed). Furthermore, all patients underwent a one-year follow-up. RESULTS: In this study, 107 patients were enrolled, 38 (35.5%) with CCM. Echocardiography revealed an increase in postoperative left ventricular filling pressure accompanied by an elevation in left ventricular ejection fraction (LVEF). However, patients in the CCM group exhibited lower LVEF and mean arterial pressure (MAP) compared to the non-CCM group. Post-TIPS, CCM patients showed increased right atrium pressure (RAP) that normalized within 2-4 days, whereas non-CCM patients had lower RAP than baseline. Compared to patient without CCM, CCM patients revealed lower immediate (16.7 ± 4.4 vs. 18.9 ± 4.8, p = 0.022) and delayed 15.9 ± 3.7 vs. 17.7 ± 5.3, p = 0.044) portal vein pressures (PVP) and portal pressure gradients (PPG) (7.7 ± 3.4 vs. 9.2 ± 3.6, p = 0.032 and 10.1 ± 3.1 vs. 12.3 ± 4.9, p = 0.013). The 1-year mortality rates were 13.2% for CCM patients and 4.3% for non-CCM patients (log-rank test, p = 0.093), with MELD score, and preoperative RAP significantly associated with the mortality. CONCLUSION: Cirrhotic patients with CCM exhibit lower PVP and PPG immediately after TIPS and 2-4 days later, without significantly impacting one-year survival outcomes.

The evolution of the TIPS placement technique and its applications over four decades.

Transjugular intrahepatic portosystemic shunt (TIPS), a highly effective procedure reducing portal hypertension, has been in use for over seven decades and is now a cornerstone in managing portal hypertension-related complications such as variceal bleeding and ascites. Historically, TIPS has dealt with two main challenges: ensuring stent patency and preventing post-TIPS hepatic encephalopathy. The introduction of PTFE-coated stents markedly reduced the risk of TIPS dysfunction and stent patency is no longer a major concern. However, despite improved patient selection criteria, hepatic encephalopathy continues to be a significant and persistent issue. In addition, the broader application of TIPS in recent decades has brought to light additional, albeit less common, complications, such as post-TIPS heart failure. This review offers a comprehensive overview of TIPS historical evolution, advancements in technique, and its application in the treatment of portal hypertension.

The Significance of Congenital Portosystemic Shunts in Congenital Heart Disease and the Bizarre Phenomenon of Alternating Portosystemic and Systemic-Portal Shunting.

Congenital portosystemic shunts (CPSSs) are rare vascular anomalies characterized by abnormal connections between the portal/splanchnic veins and the systemic veins. CPSSs often occur as an isolated congenital anomaly, but they can also coexist with congenital heart disease (CHD). Owing to their myriad consequences on multiple organ systems, familiarity with CPSS is of tremendous importance to the care of patients with CHD. The rationale and timing for interventions to embolize CPSS in this scenario are discussed. Specific shunt embolization techniques are beyond the scope of this article.

Radiomics Analysis of Preprocedural CT Imaging for Outcome Prediction after Transjugular Intrahepatic Portosystemic Shunt Creation.

PURPOSE: To evaluate the role of radiomics in preoperative outcome prediction in cirrhotic patients who underwent transjugular intrahepatic portosystemic shunt (TIPS) using "controlled expansion covered stents". MATERIALS AND METHODS: This retrospective institutional review board-approved study included cirrhotic patients undergoing TIPS with controlled expansion covered stent placement. From preoperative CT images, the whole liver was segmented into Volumes of Interest (VOIs) at the unenhanced and portal venous phase. Radiomics features were extracted, collected, and analyzed. Subsequently, receiver operating characteristic (ROC) curves were drawn to assess which features could predict patients' outcomes. The endpoints studied were 6-month overall survival (OS), development of hepatic encephalopathy (HE), grade II or higher HE according to West Haven Criteria, and clinical response, defined as the absence of rebleeding or ascites. A radiomic model for outcome prediction was then designed. RESULTS: A total of 76 consecutive cirrhotic patients undergoing TIPS creation were enrolled. The highest performances in terms of the area under the receiver operating characteristic curve (AUROC) were observed for the "clinical response" and "survival at 6 months" outcome with 0.755 and 0.767, at the unenhanced and portal venous phase, respectively. Specifically, on basal scans, accuracy, specificity, and sensitivity were 66.42%, 63.93%, and 73.75%, respectively. At the portal venous phase, an accuracy of 65.34%, a specificity of 62.38%, and a sensitivity of 74.00% were demonstrated. CONCLUSIONS: A pre-interventional machine learning-based CT radiomics algorithm could be useful in predicting survival and clinical response after TIPS creation in cirrhotic patients.

Left Chylothorax Without Ascites in a Liver Transplant Patient.

Chylothorax is a rare condition that results from thoracic duct disruption with malignant and nonmalignant etiologies manifesting as a pleural effusion. Typically, chylothorax in the setting of cirrhosis is associated with the migration of chylous ascites. We present the case of a 64-year-old male with prior liver transplant who presented with new-onset transudative chylothorax without chylous ascites who responded to transjugular intrahepatic portosystemic shunt revision, diuresis, and serial thoracentesis.

Utilizing a suture-constrained covered stent for shunt reduction to treat transjugular intrahepatic portosystemic shunt-related refractory hepatic encephalopathy: a retrospective study.

PURPOSE: Refractory hepatic encephalopathy (RHE) can occur as a consequence of excessive shunting following the creation of a transjugular intrahepatic portosystemic shunt (TIPS). We describe a technique that utilizes a suture-constrained covered stent for shunt reduction to treat TIPS-related RHE. MATERIALS AND METHODS: Between January 2017 and September 2023, 25 patients with TIPS-related RHE who underwent shunt reduction utilizing a suture-constrained covered stent were reviewed. The procedure involved reducing the diameter of a polytetrafluoroethylene-covered stent from 8 to 5 mm with a non-absorbable suture and inserting it into the existing TIPS stent to reduce shunt flow. RESULTS: Twelve of the 25 patients were evaluated. Shunt reduction was technically successful in all patients and no immediate complications related to the procedures were observed. Varying degrees of improvement in HE symptoms were observed after shunt reduction, with a mean increase in portosystemic gradient of 5 mmHg compared to pre-procedure, and complete disappearance of symptoms was observed in seven (58.3%) individuals. After a median follow-up of 8.3 months, HE recurred in 4 patients (33.3%) and TIPS indication recurred in 2 patients (16.7%) in the form of ascites and variceal bleeding, respectively. One patient (8.3%) developed shunt dysfunction detected by Doppler ultrasound and was accompanied by the presence of hepatic hydrothorax and ascites. At the end of the study, 5 patients (41.7%) were alive, 5 (41.7%) succumbed to liver failure, and 2 (16.7%) succumbed to pneumonia. CONCLUSIONS: Constraining the stent diameter with a suture is feasible, and using this suture-constrained covered stent for shunt reduction can effectively improve TIPS-related RHE. Further investigations are warranted to precisely delineate the impact of the increased portosystemic gradient and to optimize patient survival.

Hepatic Encephalopathy Secondary to Non-cirrhotic Portosystemic Shunt.

Hepatic encephalopathy is uncommon in the absence of cirrhosis. We report a 71-year-old woman who presented with altered mental status in the setting of hyperammonemia for the second time in 6 months. Magnetic resonance imaging of the abdomen revealed an uncommon portosystemic shunt involving an enlarged posterior branch of the right portal vein and an accessory right hepatic vein, with no features of cirrhosis. Appropriate management of these patients with ammonia-lowering therapy can reduce repeat episodes and improve quality of life. This case demonstrates the importance of diagnosing non-cirrhotic hepatic encephalopathy in patients with altered mental status.

Experience of Performing Hepatic Interventional Radiological Procedures in a Tertiary Care Hospital in Odisha: A Case Series.

Introduction: During last few decades, radiological interventions have played crucial role in the management of the patients with chronic liver diseases. Various procedures including transjugualar intrahepatic portosystemic shunt (TIPS), transjugular liver biopsy (TJLB), transarterial chemoembilization (TACE)/transarterial radioembolization (TARE), balloon retrograde transvenous obliteration (BRTO) and plug-assisted retrograde transvenous obliteration (PARTO) are being performed safely and have significantly improved clinical outcomes in these patients. The technical and clinical success depend on appropriate patient selection along with thorough knowledge and experience to perform these procedures. On the other hand, few adverse events may also be associated with these procedures. The intervention radiologist and hepatologists should identify and treat these complications at the earliest so as to improve outcome of the patient. Materials and methods: About 25 hepatic intervention radiology procedures were performed in our center from January 2022 to 2023 May. Among these we have selected five patients who underwent TACE/TIPS/DIPS in our institute. We have selected these cases as in each of these cases we encountered some interesting outcomes/complications which were managed successfully. Results: The first case describes 33-year-old male with POEM syndrome and Budd Chiari Syndrome (BCS) who underwent TIPS and immediately had blockade of the stent. The second case is of a 43 years old male having BCS, refractory ascites with umbilical and inguinal hernia. The third case is of a 40 years old female with decompensated cirrhosis who underwent TIPS for portal hypertensive gastropathy. The fourth case is of a 51-years' female with decompensated cirrhosis with sarcopenia. Finally, the fifth case describes 24-year-old female with BCS and hepatocellular carcinoma. In this article we discuss the procedure and clinical course of the patients following the procedure. Conclusion: Hepatic radiological interventions though widely used can be associated with unusual albeit life threatening complications. Appropriate patient selection and thorough knowledge of procedure along with early diagnosis and management of these complications are key to obtain satisfying long term outcomes.

Beyond the Rib Cage: Unraveling Abernethy Syndrome as a Rare Cause of Cyanosis.

Abernethy syndrome is a rare congenital malformation stemming from a portosystemic shunt. Diagnosis proves challenging due to nonspecific clinical symptoms, with presentation varying based on age and disease severity. Consequences include hepatic, cardiovascular, renal, gastrointestinal, and neurological complications, and growth retardation. We report the case of a child presenting with perioral and digital cyanosis, observed in early childhood. Clinical examination revealed low saturation, telangiectasias, digital clubbing, and collateral venous circulation in the thorax. Imaging confirmed the diagnosis of Abernethy syndrome.