2 There are regional differences in the BCS type [CLASS C1] in the Asia-Pacific region. Patients with splenomegaly and a normal or high platelet count should be tested for a CALR mutation in the absence of a JAK2 gene mutation. Such benign regenerative nodules are observed only in patients with chronic BCS, which is associated with long disease duration and the extent of fibrosis in non-nodular livers [78, 81].
Risk, surveillance strategy, and management of HCC
Resection of HCC in patients with BCS has been seen to be associated with increased morbidity and mortality in some studies, while other authors report good outcomes after surgical treatment of BCS-HCC [ 109 , 110 ]. Management of HCC in a patient with BCS should be in accordance with standard guidelines for HCC in patients with cirrhosis.
Assessment of coagulation and anticoagulation
In addition, thrombotic events almost secondary to the underlying thrombotic disease are common, resulting in BCS recurrence in 5–11% of cases [115, 116]. Thus, the consensus is in favor of maintaining and initiating anticoagulant therapy immediately after LT, unless the underlying disease is cured by transplantation [117]. Therefore, the outcome of BCS-HCC is better if the tumor is diagnosed early and liver function is preserved.
The decision about appropriate treatment for a patient with BCS-HCC should be made by a multidisciplinary team, including gastroenterologist, surgeon, and interventional radiologist. Presence of ascites does not always indicate cirrhosis and should not preclude the use of liver-directed therapies in a patient with HCC-BCS.
Principles of management and outcomes Treatment strategy and long‑term outcome
Although the hemodynamics of portal hypertension in patients with BCS may differ from that in cirrhotic patients, [135]. Favorable long-term results after recanalization have been reported in large series from the West and Asia [140, 141]. With less invasiveness, good efficacy and thus lower morbidity, TIPS is the predominant choice of bypass treatment and recent studies have reported a good long-term outcome with TIPS alone in patients from different regions, with 5-year survival rates ranging from 72 to .
Nevertheless, it should be noted that even with PTFE-covered stents, primary patency rate in BCS patients [152] seems lower than in cirrhotic patients. The recurrence rate of BCS after liver transplantation is low, probably due to routine long-term anticoagulation after liver transplantation [163]. Data regarding long-term survival of patients with BCS are scarce with a small sample size of patients.
More long-term data with larger sample size are required to study the long-term survival of patients with BCS. GRADE A1] Routine stenting at the initial procedure should be considered to reduce reintervention and improve long-term patency.
Radiological interventions TIPS
Angioplasty recanalization is the preferred invasive treatment, especially in patients with short-length thrombosis or ostial stenosis. For patients with primary BCS, thrombophilia screening is additionally performed to determine the etiology of the prothrombotic state. Patients with BCS need assessment of liver parenchymal and venous anatomy when planning a TIPS procedure.
TIPS may be challenging in patients with BCS due to the lack of an open hepatic vein. Clinical Success TIPS has proven successful in reducing portal hypertension in the vast majority of patients with. They concluded that balloon dilatation alone might be the optimal treatment for patients with MOVC.
24] reported a retrospective study on 93 patients with BCS who had hepatic venous obstruction (91 had membranous obstruction) with or without IVC obstruction, of which only balloon dilation was treated. Most of the patients with BCS have underlying thrombotic disorders and hypercoagulable state, therefore it is important to maintain post-intervention INR between 2 and 3.
Pathogenesis and management
Transjugular access is the preferred access for HV cannulation, with the transhepatic route reserved for difficult cases. Stepwise approach with initial balloon dilatation followed by stents in cases with residual stenosis or significant pressure gradient across stenosis. For IVC or HV stenosis or long segment occlusion, balloon dilatation with primary stent is preferred.
Bridging therapy with low-molecular-weight heparin and warfarin is used immediately after the procedure. Periodic Doppler ultrasound imaging at 1, 3, 6 months and then 6-month follow-up is recommended for all patients.
Criteria and assessment of response to therapy
In recent years, the role of liver stiffness measurement (LSM) to assess and monitor response to BCS treatment has been investigated [233, 236]. The rapid reduction in liver stiffness values reflected a reduction in liver congestion rather than an improvement in liver fibrosis. Interestingly, findings in another case series of 7 BCS patients treated with TIPS placement suggest that LSM and SSM values are associated with the occurrence and severity of BCS and can potentially guide the optimal initial treatment for each patient after diagnosis of BCS [ 236].
It is unknown whether LSM and SSM can be used to monitor response to treatment in patients treated with anticoagulant alone. MRE proved to be a useful tool for monitoring treatment response in another cohort of 15 BCS patients who. In this study, the magnitude of changes in liver stiffness was significantly correlated with changes in hepatic venous pressure gradient (r = 0.651, p = 0.009).
In patients treated with endovascular stenting, the metal stent may create imaging artifacts and may be heated by the radiofrequency electromagnetic pulse. A decrease in liver stiffness after endovascular therapy indicates an improvement in hepatic congestion.
Liver transplantation in Budd–Chiari syndrome
In contrast to the above study, correlation between the changes in LSM and the changes in pressure gradient across the treated hepatic venous outflows was not observed in this study. Patients with acute non-fulminant presentation had markedly high values of both LSM and SSM, i.e. 75 kPa, which represents severe hepatic overload and portal hypertension. Therefore, the LSM and SSM values at presentation could potentially help stratify the severity of BCS and predict the need for invasive treatment.
Due to the relatively small number of patients and the short follow-up period (range: 3 days-6 months) in these studies, a larger cohort with different clinical presentations and long-term follow-up is needed to validate the promising results. advantages of LSM and SSM as a non-invasive tool for determining treatment outcome before implementation in practice. There are some limitations in performing TE in BCS patients who are obese, have gross ascites, or narrow intercostal spaces. Further studies to determine the utility of LSM in predicting the long-term outcome of BCS patients are required.
The combination of liver stiffness and spleen stiffness measurement can classify disease severity and guide optimal therapy. In some cases, the IVC can be used as an anastomotic site after appropriate thrombectomy.
Special situation: acute liver failure and acute‑on‑chronic liver failure
The hepatic veins of the liver graft must be anastomosed with an open outflow tract from the recipient. In other cases, the vena cava may be sclerotic due to the effects of inflammation, making it unsuitable for anastomosis. If the sclerotic changes in the IVC are limited, the sclerotic section can be replaced with a vein graft, which is then anastomosed with an intact vena cava.
Since patients with BCS are often in a prothrombotic state, long-term anticoagulation therapy should be maintained after liver transplantation [242]. Patients with type A and type C, BCS-ACLF should undergo urgent radiological intervention such as TIPS, HV stenting or thrombolysis. Patients with BCS-ACLF unresponsive to or ineligible for radiological/medical therapy should be considered for liver transplantation.
Special situation: pregnancy, pediatric, adolescents
For example, in a child with portal hypertension, the presence of engorged veins over the mid back suggests venous collaterals formed in response to IVC obstruction. Imaging tests (Doppler scan, CT or MRI scan) are done to detect and characterize obstruction of the hepatic outflow tract. In patients with BCS, tests for an underlying prothrombotic condition should also be performed [257].
Treatment consists of two strategies: decompression of the blocked hepatic venous outflow tract and anticoagulation. Balloon angioplasty of the occluded veins alone is the preferred strategy in infants and younger children due to a lack of appropriately sized stents. IVC angioplasty and stenting of blocked vessels is performed in a single session using uncovered self-expanding metal stents, which enlarge to a degree with the age and size of the vein.
TIPS/DIPS in children is limited by the size of the stents; hence a strategy of "covered stent in an uncovered stent" is used, where the length of the uncovered portion at the end of the hepatic vein is determined by the size of the pediatric liver [248]. In women known to have BCS, it is preferable to perform endovascular treatment to decompress the blocked hepatic venous outflow and initiate anticoagulation prior to planning pregnancy.
Future research areas
Because children are more likely to play contact sports, there is an increased risk of bleeding when treated with anticoagulants. Monitoring the adequacy of anticoagulation in children with increasing age and weight with repeated blood tests can also be challenging. Although there is limited data specifically on BCS in adolescence, a study of 43 patients found that response to therapy was as good as that seen in adults.
About one third of adolescents with BCS present with hepatomegaly without ascites and have less frequent thrombophilic disorders [36]. In women, BCS causes primary infertility and successful treatment of BCS increases the chance of conception. It is important that the clinician and radiologist have a high index of suspicion and consider the diagnosis of BCS in any child or adolescent with unexplained portal hypertension.
Long-term results of transjugular intrahepatic portosystemic shunt in Indian patients with Budd-Chiari syndrome.
