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The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
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Epigastric abdominal pain is a common indication for consultation. In the majority of cases, medical history, clinical examination and routine biological exams allow for an easy diagnosis. Sometimes the symptomatology is unusual, in which case it is essential to perform a complete clinical examination and to use various imaging techniques to search for eventual atypical causes. Membranous obstruction of inferior vena cava is a rare cause of such a phenomenon. We describe a Budd-Chiari syndrome caused by membranous obstruction of inferior vena cava in a 66-year-old woman with no medical history as a rare cause of epigastric abdominal pain. We will describe this clinical experience in the light of the literature and point out the contribution of radiological imaging in the diagnosis of this rare pathology.
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Background: Transjugular intrahepatic portosystemic shunt (TIPS) relieves hepatic venous obstruction in Budd-Chiari syndrome (BCS), but the effect on liver function is unclear, particularly outside the immediate post-treatment period. This study aims to evaluate the long-term impact of TIPS on liver function and outcomes in BCS patients. Methods: Twenty patients with BCS who underwent TIPS from 1999 to 2018 were included. Demographic data and clinical data at the time of TIPS procedure, 6 months, 12 months, 2 years, 5 years, and 10 years post-TIPS were collected. Results: There were 13 (13/20, 65%) women and 7 (7/20, 35%) men with a mean age at the time of TIPS of 42.6 ± 12.8 years. The median time from BCS diagnosis to TIPS was 41 (IQR: 4-165) days. The number of patients with severe ascites decreased significantly from 10/17 (58.8%) at the time of TIPS, to 1/16 (7.7%), 1/13 (7.7%), 2/16 (12.5%), 1/14 (7.1%), and 0/8 (0%) at 6 months, 12 months, 2 years, 5 years and 10 years post-TIPS, respectively. 4/20 (20%) patients developed new hepatic encephalopathy post-TIPS procedure. Child-Pugh score significantly decreased from a score of 9.4 ± 1.8 pre-TIPS to 7.6 ± 1.8 at 6 months, 7.4 ± 1.5 at 12 months, 7.3 ± 1.6 at 2 years, 6.8 ± 1.5 at 5 years, and 6.4 ± 0.7 at 10 years post-TIPS. Fifteen (15/20, 75%) patients required TIPS revision including 4 (4/15, 26.7%) within 30 days, 2 (2/15, 13.3% within 1 month to 1 year, and 9 (9/15, 60%) at more than 1 year. Eight (8/20, 40%) patients underwent liver transplantation (LT) at median time of 7.3 (IQR 3.2-12.9) years after TIPS. Conclusion: TIPS placement for BCS results in sustained resolution of symptoms and improved liver function. Despite the frequent need for revisions, the long-term durability of TIPS can forgo the need for LT in the majority of patients.
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Background and aims: Increasing incidence of hepatocellular carcinoma (HCC) in India is a matter of concern and need for adequate profiling and streamlining management strategies cannot be over-emphasized. Methods: This is a prospective multi-centric observational cohort study comprising of an oncology center, one university tertiary hospital with specialized hepatology service, one public hospital with gastroenterology service, and a private liver transplant center located within a 3-km radius. The demographic and clinical parameters were recorded on a prospectively maintained database. The clinical profile, demographics, characteristics of HCC and the allocated treatment were noted and compared among the four centers. Results: In total, 672 patients were enrolled from June 2016 till January 2020. Abdominal pain (64.3%) and weight loss (47.3%) were the most common symptoms. Most common identified etiology was hepatitis B (39%). The cancer center received lesser patients with hepatitis C and those with advanced stage of HCC. The private transplant center reported the highest proportion of NASH, which was also significantly higher in those belonging to higher socioeconomic strata, and lowest proportion of alcoholic cirrhosis. Metastasis was seen in almost one-fifth (19%) cases at diagnosis. Portal vein thrombosis was evident in 40%. Adherence to treatment guidelines was seen in three-fourth cases (76%). Conclusions: Hepatitis B is the most common underlying cause for HCC, whereas other causes like NASH are on the rise. Etiologic profile may vary with selective specialization of centers catering to patients with HCC. Adherence to guideline while allocating treatment was high among all centers with highest non-adherence in BCLC A.
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Patients with cirrhosis of the liver are at high risk of developing portal vein thrombosis (PVT), which has a complex, multifactorial cause. The condition may present with a myriad of symptoms and can occasionally cause severe complications. Contrast-enhanced computed tomography (CT) is the gold standard for the diagnosis of PVT. There are uncertainties regarding the effect on PVT and its treatment outcome in patients with cirrhosis. The main challenge for managing PVT in cirrhosis is analyzing the risk of hemorrhage compared to the risk of thrombus extension leading to complications. All current knowledge regarding non-tumor PVT in cirrhosis, including epidemiology, risk factors, classification, clinical presentation, diagnosis, impact on natural history, and treatment, is discussed in the present article.
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BACKGROUND: Natural portosystemic shunt ligation practices in liver transplant vary widely across transplant centres and are frequently undertaken to prevent the serious consequence of portal steal phenomenon. No concrete indications have so far been convincingly identified for their management in living donor liver transplant. METHODS: We retrospectively studied the outcome of 89 cirrhotic patients who either did (n = 63) or did not (n = 25) undergo shunt ligation during living donor liver transplantation between 2017 and 2020. RESULTS: The incidence of early allograft dysfunction/nonfunction (P = 1.0) and portal venous complications (P = 0.555) were similar between the two groups. Although overall complications, biliary complications, and the composite of Grade III and IV complications were significantly higher in the nonligated group (P = 0.015, 0.052 and 0.035), 1- year graft and patient survival were comparable between them (P = 0.524). CONCLUSION: We conclude that shunt ligation in living donor liver transplantation may not always be necessary if adequate portal flow, good vascular reconstruction, and good graft quality have been ensured.
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OBJECTIVES: To compare the clinical outcomes in terms of structure and function between the insertion of a transjugular intrahepatic portosystemic shunt (TIPS) created with the Viabahn ePTFE covered stent/bare metal stent (BMS) combination and the Fluency ePTFE covered stent/BMS combination. METHODS: A total of 101 consecutive patients who received a TIPS from February 2016 to August 2018 in our center were retrospectively analyzed. Sixty-four subjects were enrolled in the Viabahn group and 37 were enrolled in the Fluency group. The geometry characteristics of the TIPS were calculated, and the associated occurrence of shunt dysfunction, survival, overt hepatic encephalopathy, and variceal rebleeding were evaluated. RESULTS: The technical success rate was 100%. After the insertion of the TIPS, the rate of shunt dysfunction during the first 3 months was significantly different between the Viabahn and Fluency groups (1.6% and 13.5%, respectively; p â= â0.024). Multivariate analysis indicated that the angle of portal venous inflow (α) was the only independent risk factor for shunt dysfunction (hazard ratio â= â1.060, 95% confidence interval â= â1.009-1.112, p â= â0.020). In addition, 3 months after the TIPS insertion, the α angle distinctly increased from 20.9° â± â14.3°-26.9° â± â20.1° (p â= â0.005) in the Fluency group but did not change significantly in the Viabahn group (from 21.9° â± â15.1°-22.9° â± â17.6°, p â= â0.798). CONCLUSIONS: Shunt dysfunction was related to the α angle owing to the slight effect on the α angle after the implantation of the TIPS. The Viabahn ePTFE covered stent/BMS combination was more stable in structure and promised higher short-term stent patency compared with the Fluency ePTFE covered stent/BMS combination.
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A 34-year-old woman visited our hospital because she had had abdominal bloating for 2 months. She had been diagnosed with invasive thymoma (WHO pathological type B2), for which she had undergone chemotherapy and total thymectomy 10 years previously. Six years previously, pleural dissemination was diagnosed and she had undergone right extra-pleural pneumonectomy. On presentation to our hospital, abdominal computed tomography and ultrasound scans revealed abundant ascites and a huge liver lesion, likely a metastasis from her thymoma, obstructing the inferior vena cava. The serum-ascites albumin gradient was high at 1.4 g/dL, which indicated portal hypertension. We diagnosed Budd-Chiari syndrome caused by liver metastasis from a previous thymoma. Steroid therapy resulted in shrinkage of her liver tumor and a marked decrease in her ascites. Although rare, Budd-Chiari syndrome caused by liver metastasis from a thymoma is a possible serious complication of advanced invasive thymoma.
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OBJECTIVE: The Budd-Chiari Syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction. The primary objectives of our study were to assess temporal trends in the prevalence of BCS among hospitalized patients in the United States using the National Inpatient Sample (NIS) database and to evaluate demographics, risk factors, and common presentation of BCS. METHODS: Data were extracted from the NIS to identify patients >18 years of age using all listed diagnosis of BCS from 1998 to 2017 and analyzed. RESULTS: Between 1998 and 2017, we identified a total of 8435 hospitalizations related to BCS. Over the 19-year period, the hospitalization rate for BCS increased consistently from 4.96 per 1,000,000 US population in 1998 to 10.44 per 1,000,000 in 2017, with an annual percentage change increase of 4.41% (95% confidence interval [CI]: 4.23%-4.59%, P < 0.0001). The most common risk factor (7.75%) was myeloproliferative disorder (essential thrombocythemia, polycythemia vera, myelofibrosis, chronic myeloid leukemia) followed (7.32%) by a hypercoagulable state (primary thrombophilia, protein C deficiency, factor V Leiden mutation, antiphospholipid antibody syndrome or prothrombin gene mutation) and paroxysmal nocturnal hemoglobinuria (1.63%). Cirrhosis was present in 18.7%, Portal vein thrombosis in 7.9%, and inferior vena cava thrombosis in 6.4%. The most common manifestations of BCS were ascites (29.9%) or acute kidney injury (18.8%) followed by hepatic encephalopathy (9.6%) and acute liver failure (5.6%). CONCLUSION: This large population-based study from the United States showed increasing hospitalizations related to BCS. Common presentation was ascites and acute kidney injury.
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INTRODUCTION: Celiac disease (CD) has been linked to portal hypertension (PHT) of varied etiology, but the causality association has never been proved. We aim to study the prevalence of CD in patients of PHT of different etiology. METHODS: A prospective observational study was conducted from June 2017 to December 2018 involving all the cases of PHT of varied etiology. Consecutive patients of PHT with chronic liver disease (CLD) of defined etiology like ethanol, viral hepatitis (B or C), Budd-Chiari syndrome (BCS), autoimmune-related cirrhosis, and cryptogenic CLD (cCLD) (group A) and those with noncirrhotic PHT (NCPHT), which included noncirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO) (group B), were screened for CD by IgA anti-tTG antibody followed by duodenal biopsy in serology-positive patients. RESULTS: Out of a total of 464 patients, group A constituted 382 patients, CLD related to ethanol (155), cCLD (147), hepatitis B (42), hepatitis C (21), autoimmune (10), and BCS (7), whereas 82 patients were in group B with NCPF (64) and EHPVO (18). Total 29 patients were diagnosed with CD in both groups, 17 in group A (4.5%) and 12 in group B (14.6%). In group A, 13 patients with cCLD, two with HBV-related CLD, one with BCS, and one with autoimmune-related CLD were concomitantly diagnosed as CD. In group B, CD was diagnosed in 12 patients of NCPF (11) and EHPVO (1). Liver histology showed chronic hepatitis in two patients and was normal in three patients. CONCLUSION: CD is common in PHT of different etiology, especially in cCLD, NCPH and autoimmune hepatitis; however, the etiological basis for this association is still to be defined. The likelihood of CD is higher in liver disease than the general population, and these patients should be screened for CD.
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Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.
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BACKGROUND & AIMS: Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS. METHODS: This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019. MR images were reviewed by 2 radiologists blinded to the diagnosis of the lesions. Patient and lesion characteristics were recorded, focusing on HBP imaging features. RESULTS: Twenty-six patients (mean 35 ± 11 years old [13-65]; 21 women [81%] 35 ± 12 years old [13-65]; 5 men [19%] 36 ± 10 years old [19-44]) with 99 benign liver lesions and 12 hepatocellular carcinomas (HCCs) were analyzed. Patients with HCC were significantly older than those with benign lesions (mean 50 ± 10 vs. 33 ± 9 years old, p = 0.003), with higher alpha-fetoprotein (AFP) levels (3/4 [75%] vs. 1/22 [5%] with AFP >15 ng/ml, p <0.001). Homogeneous hypointense signals were identified on HBP in 14 lesions, including 12/12 (100%) HCCs, and 2/99 (2%) benign lesions (p <0.001). Most benign liver lesions showed either peripheral (n = 52/99 [53%]) or homogeneous hyperintensity (n = 23/99 [23%]) on HBP. Lesions with signal hypointensity on HBP in patients with AFP serum levels >15 ng/ml were all HCCs. CONCLUSION: Most benign lesions showed homogeneous or peripheral hyperintensity on HBP images while all HCCs were homogeneously hypointense. HBP images are helpful to differentiate between benign lesions and HCCs and outperform other sequences. They should be systematically acquired for the characterization of focal lesions in patients with BCS. LAY SUMMARY: Hepatobiliary phase imaging is an approach that has recently been shown to discriminate between benign and malignant lesions in the liver. However, it was not known whether this imaging approach could be used effectively in patients with Budd-Chiari syndrome. Herein, we have shown that hepatobiliary phase imaging appears to be useful for differentiating between benign and malignant liver lesions in patients with Budd-Chiari syndrome.
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BACKGROUND & AIMS: Budd-Chiari Syndrome (BCS) is considered a thrombophilic state, and most patients with BCS have thrombophilic disorder. Liver dysfunction-related coagulopathy makes coagulation function unpredictable in BCS. Thromboelastography (TEG) assesses the dynamics, strength, and stability of clot formation. We conducted a pilot study using TEG to evaluate coagulation status in patients with BCS. METHODS: Fifty-one patients with newly diagnosed BCS (age 32.3 [10.7] years; 23 men) underwent TEG (TEG®5000 Hemostasis Analyzer®, USA), and its components were analyzed and correlated with clinical profile and thrombophilic disorders. Patients who had received anticoagulation, antiplatelet drugs, or radiological intervention were excluded. RESULTS: Twenty-nine patients had normal TEG, 11 had procoagulant TEG, and 11 had hypocoagulant TEG. Among patients with hypocoagulant TEG, Coagulation Index (CI) was < -3 in 11 patients, R was >8 min in 6 patients, K was >3 min in 9 patients, alpha <55 in 9 patients, and MA <51 in 7 patients; among those with hypercoagulant TEG, CI was >3 in 3 patients, R < 2 min in 2 patients, K <1 min in 2 patients, alpha >78 in none, and MA >69 mm in 7 patients. TEG findings were similar in patients with and without thrombophilic disorder. The mean platelet count (1.75, 2.22, and 1.79 × 105/mm3; P = 0.13) and international normalized ratio (1.27, 1.34, and 1.28, P = 0.69) were similar in those with procoagulant, normal, and hypocoagulant TEG. Two patients in Rotterdam class-III had abnormal LY30. Other clinical parameters did not correlate with TEG findings. CONCLUSION: Patients with BCS are heterogeneous with respect to coagulation status, with one-fifth of patients are hypocoagulant on TEG. Patients with advanced disease may have accelerated fibrinolysis.
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Liver diseases occurring during pregnancy can be serious and can progress rapidly, affecting outcomes for both the mother and fetus. They are a common cause of concern to an obstetrician and an important reason for referral to a hepatologist, gastroenterologist, or physician. Liver diseases during pregnancy can be divided into disorders unique to pregnancy, those coincidental with pregnancy, and preexisting liver diseases exacerbated by pregnancy. A rapid differential diagnosis between liver diseases related or unrelated to pregnancy is required so that specialist and urgent management of these conditions can be carried out. Specific Indian guidelines for the management of these patients are lacking. The Indian National Association for the Study of the Liver (INASL) in association with the Federation of Obstetric and Gynaecological Societies of India (FOGSI) had set up a taskforce for development of consensus guidelines for management of patients with liver diseases during pregnancy, relevant to India. For development of these guidelines, a two-day roundtable meeting was held on 26-27 May 2018 in New Delhi, to discuss, debate, and finalize the consensus statements. Only those statements that were unanimously approved by most members of the taskforce were accepted. The primary objective of this review is to present the consensus statements approved jointly by the INASL and FOGSI for diagnosing and managing pregnant women with liver diseases. This article provides an overview of liver diseases occurring in pregnancy, an update on the key mechanisms involved in its pathogenesis, and the recommended treatment options.
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Intra-hepatic portal-venous collaterals are characteristic of Budd-Chiari syndrome (BCS) and are usually of small caliber and seen on Doppler. Creation of large portal-systemic shunt, either radiologically (Transjugular intrahepatic porto-systemic shunt) or surgically results in excellent long term outcomes in BCS. Here, we report a series of three rare cases of asymptomatic BCS, who had spontaneous large intra-hepatic portal-systemic shunts.
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BACKGROUND: There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center. METHODS: This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma. RESULTS: Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. There were 11 deaths over the follow-up period - 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS. CONCLUSION: Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.
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Portal vein thrombosis, once considered as a contraindication to transjugular intrahepatic porto-systemic shunt (TIPS) is now considered as an indication. We report a case with clinical and technical success in a patient with Budd Chiari syndrome and acute portal venous thrombosis. Though it is a well-established option, with the best of our knowledge, we could not find a report from India.
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Vascular disorders of the liver frequently affect women of childbearing age. Pregnancy and the postpartum are prothrombotic states. Pregnancy seems to be a trigger for Budd-Chiari syndrome in patients with an underlying prothrombotic disorder. Whether pregnancy is a risk factor for other vascular liver disorders is unknown. In women with a known vascular liver disorder and a desire for pregnancy, stabilisation of the liver disease, including the use of a portal decompressive procedure when indicated, should be reached prior to conception. The presence of esophageal varices should be screened and adequate prophylaxis of bleeding applied in a manner similar to what is recommended for patients with cirrhosis. Most women likely benefit from anticoagulation during pregnancy and the postpartum. Labor and delivery are best managed by a multidisciplinary team with experience in this situation. Assisted vaginal delivery is the preferred mode of delivery. Although the risk of miscarriage and premature birth is heightened, current management of these diseases makes it very likely to see the birth of a live baby when pregnancy reaches 20 weeks of gestation.
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Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion.