Spontaneous porto-systemic shunts in liver cirrhosis:Clinical and therapeutical aspects

Spontaneous porto-systemic shunts(SPSS)are frequent in liver cirrhosis and their prevalence increases as liver function deteriorates,probably as a consequence of worsening portal hypertension,but without achieving an effective protection against cirrhosis'complications.Several types of SPSS have been described in the literature,each one associated with different clinical manifestations.In particular,recurrent or persistent hepatic encephalopathy is more frequent in patients with splenorenal shunt,while the presence of gastric varices and consequently the incidence of variceal bleeding is more common in gastrorenal shunt.In the advanced stage,the presence of large SPSS can lead to the so called“portosystemic shunt syndrome”,characterized by a progressive deterioration of hepatic function,hepatic encephalopathy and,sometimes,portal vein thrombosis.The detection of SPSS in patients with liver cirrhosis is recommended in order to prevent or treat recurrent hepatic encephalopathy or variceal bleeding.

Budd-Chiari syndrome in children:Challenges and outcome

Budd-Chiari syndrome(BCS)is an uncommon disease of the liver,characterised by obstruction of the hepatic venous outflow tract.The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe.Compared to adults with BCS,children have primary BCS as the predominant etiology,earlier clinical presentation,and hence better treatment outcome.Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS,though work-up for the same is often unyielding in children.Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield.In recent years,advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS.Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response.Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness.This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.

Transjugular intrahepatic porto 全身的分流在老: 为门高血压的复杂并发症的减轻

AIM:To present a dedicated series of transjugular intrahepatic porto-systemic shunts(TIPS) in the elderly since data is sparse on this population group.METHODS:A retrospective review was performed of patients at least 65 years of age who underwent TIPS at our institutions between 1997 and 2010.Twentyfive patients were referred for TIPS.We deemed that 2 patients were not considered appropriate candidates due to their markedly advanced liver disease.Of the 23 patients suitable for TIPS,the indications for TIPS placement was portal hypertension complicated by refractory ascites alone(n = 9),hepatic hydrothorax alone(n = 2),refractory ascites and hydrothorax(n = 1),gastrointestinal bleeding alone(n = 8),gastrointestinal bleeding and ascites(n = 3).RESULTS:Of these 23 attempted TIPS procedure patients,21 patients had technically successful TIPS procedures.A total of 29 out of 32 TIPS procedures including revisions were successful in 21 patients with a mean age of 72.1 years(range 65-82 years).Three of the procedures were unsuccessful attempts at TIPS and 8 procedures were successful revisions of our existing TIPS.Sixteen of 21 patients who underwent successful TIPS(excluding 5 patients lost to follow-up) were followed for a mean of 14.7 mo.Ascites and/or hydrothorax was controlled following technically successful procedures in 12 of 13 patients.Bleeding was controlled following technically successful procedures in 10 out of 11 patients.CONCLUSION:We have demonstrated that TIPS is an effective procedure to control refractory complications of portal hypertension in elderly patients.

Body composition changes after transjugular intrahepatic portosystemic shunt in patients with cirrhosis

AIM:To investigate the effect of transjugular intra-hepatic porto-systemic shunt (TIPS) on malnutrition in portal hypertensive cirrhotic patients.METHODS: Twenty-one patients with liver cirrhosis and clinical indications for TIPS insertion were investigated before and 1, 4, 12, 52 wk after TIPS. For each patient we assayed body composition parameters [dry lean mass, fat mass, total body water (TBW)], routine liver and kidney function tests, and free fatty acids (FFA). Glucose and insulin were measured for the calculation of the homeostasis model assessment insulin resistance (HOMA-IR); liver function was measured by the galactose elimination capacity (GEC); the severity of liver disease was graded by model for end-stage liver disease (MELD).RESULTS: Porto-systemic gradient decreased after TIPS (6.0±2.1 mmHg vs 15.8±4.8 mmHg, P<0.001). Patients were divided in two groups according to initial body mass index. After TIPS, normal weight patients had an increase in dry lean mass (from 10.9±5.9 kg to 12.7±5.6 kg, P=0.031) and TBW (from 34.5±7.6 L to 40.2±10.8 L,P=0.007), as well as insulin (from 88.9±49.2 pmol/L to 164.7±107.0 pmol/L,P=0.009) and HOMA-IR (from 3.36%±2.18% to 6.18%±4.82%,P=0.023). In overweight patients only FFA increased significantly (from 0.59±0.24 mmol/L to 0.93±0.34 mmol/L, P=0.023).CONCLUSION: TIPS procedure is effective in lowering portal pressure in patients with portal hypertension and improves body composition without significant changes in metabolic parameters.

Duodenal variceal bleeding secondary to idiopathic portal hypertension treated with transjugular intra-hepatic portosystemic shunt plus embolization: A case report

BACKGROUND Duodenal varices are a lesser-known complication with non-cirrhotic portal hypertension. We report a circuitous route from missed diagnosis of duodenal varices to correction. An extremely rare case of duodenal variceal bleeding secondary to idiopathic portal hypertension(IPH) is expounded in this study, which was controlled by transjugular intra-hepatic porto-systemic shunt(TIPS) plus embolization. CASE SUMMARY A 46-year-old woman with anemia for two years was frequently admitted to the local hospital. Upon examination, anemia was attributed to gastrointestinal tract bleeding, which resulted from duodenal variceal bleeding detected by repeated esophagogastroduodenoscopy. At the end of a complete workup, IPH leadingto duodenal varices was diagnosed. Portal venography revealed that the remarked duodenal varices originated from the proximal superior mesenteric vein. TIPS plus embolization with coils and Histoacryl was performed to obliterate the rupture of duodenal varices. The anemia resolved, and the duodenal varices completely vanished by 2 mo after the initial operation. CONCLUSION TIPS plus embolization may be more appropriate to treat the bleeding of large duodenal varices.

Small diameter H-graft porta-caval shunt performed at different stages of liver disease

BACKGROUND: Partial porto-systemic shunts have been popularized because of reported low rate of mortality and morbidity (especially encephalopathy, liver failure and oc- clusion). To further investigate these assumptions, we ret- rospectively reviewed the results of partial porta-caval shunts performed at different stages of liver disease. METHODS: Twenty-nine cirrhotic patients underwent a partial porta-caval shunt with a ringed polytetrafluoroethy- lene interposition prosthesis of 8-mm ( 20 patients) or 10- mm (9 patients) in diameter. Pre- and post-shunt porta- caval pressure was measured in all patients. Twelve patients (41.4%) belonged to Child A, 11 Child B (37.9%), and 6 Child C (20.7%). Eleven patients (37.9%) suffered from hepatic encephalopathy preoperatively. Twelve patients (41%) were operated on in emergency/urgency. RESULTS: Porta-caval pressure gradient, reduced signifi- cantly using either 8- or 10-mm prosthesis. The overall ear- ly mortality and morbidity were 13. 8% and 48% respec- tively. The early mortality and morbidity were different be- tween patients of Child A and B when compared to those of Child C (0 vs 66.6% and 34.8% vs 66.6% respectively). No patient re-bled early from varices. The overall late mor- tality and morbidity were 40% and 64% respectively. Shunt thrombosis and stenosis took place in 16% and 8% of the two groups of patients respectively; variceal re-bleeding oc- curred in 4 patients (16%). Encephalopathy occurred post- operatively in 5 patients (20%), acute in 3 patients (12%), and chronic in 2 (8%). The actuarial survival rate at 3 and 5 years was 92% and 75% for patients of Child A, 70% and 60% for patients of Child B, and 0% for patients of Child C. CONCLUSIONS: Our results indicate that partial porta-ca- val shunt with a small diameter interposition H-graft is an effective procedure for the treatment of variceal bleeding, as well as for the prevention of re-bleeding in patients of Child A and those of Child B, as an elective or emergency/ urgency procedure, with a low rate of complications and encephalopathy. This technique could be used safely in pa- tients with good liver function but they should be moni- tored closely because of the risk of shunt occlusion.