Long-term outcomes following percutaneous hepatic vein recanalization for Budd-Chiari syndrome.

BACKGROUND & AIMS: A proportion of patients with Budd-Chiari Syndrome (BCS) associated with stenosis or short occlusion of the hepatic vein (HV) or upper inferior vena cava (IVC) can be treated with recanalization by percutaneous venoplasty ± HV stent insertion. We studied the long-term outcomes of this approach. METHODS: Single-centre retrospective analysis of patients referred for radiological assessment ± intervention over a 27-year period. Of 155 BCS patients, 63 patients who underwent venoplasty were studied and compared to a previously reported series treated by TIPSS (n = 59). RESULTS: Patients treated with HV interventions (32 venoplasty alone, 31 endovascular stents): mean age, 34.9 ± 10.9; M:F ratio 27:36; median follow-up, 113.0 months; 62% of patients had ≥1 haematological risk factor. Technical success was 100%, with symptom resolution in 73%. Cumulative secondary patency at 1, 5, 10 years was 92%, 79%, 79% and 69%, 69%, 64% in the stenting and venoplasty groups respectively. Where long-term patency was not achieved, 10 patients required TIPSS, and 8 underwent surgery. Actuarial survival at 1, 5, 10 years was 97%, 89% and 85%. When compared to TIPSS, HV interventions resulted in similar patency and survival rates but significantly lower procedural complications (9.5% vs 27.1%) and hepatic encephalopathy (0% vs 18%). Patient age predicted survival following multivariate analysis. CONCLUSIONS: Our data support the stepwise approach to management of BCS, with very good outcomes from venoplasty combined with stenting when required. TIPSS should only be offered where HV interventions are not feasible or unsuccessful.

U.K. guidelines on the management of variceal haemorrhage in cirrhotic patients.

These updated guidelines on the management of variceal haemorrhage have been commissioned by the Clinical Services and Standards Committee (CSSC) of the British Society of Gastroenterology (BSG) under the auspices of the liver section of the BSG. The original guidelines which this document supersedes were written in 2000 and have undergone extensive revision by 13 members of the Guidelines Development Group (GDG). The GDG comprises elected members of the BSG liver section, representation from British Association for the Study of the Liver (BASL) and Liver QuEST, a nursing representative and a patient representative. The quality of evidence and grading of recommendations was appraised using the AGREE II tool.The nature of variceal haemorrhage in cirrhotic patients with its complex range of complications makes rigid guidelines inappropriate. These guidelines deal specifically with the management of varices in patients with cirrhosis under the following subheadings: (1) primary prophylaxis; (2) acute variceal haemorrhage; (3) secondary prophylaxis of variceal haemorrhage; and (4) gastric varices. They are not designed to deal with (1) the management of the underlying liver disease; (2) the management of variceal haemorrhage in children; or (3) variceal haemorrhage from other aetiological conditions.

A reaudit of specialist-managed liver trauma after establishment of regional referral and management guidelines.

BACKGROUND: An earlier liver trauma audit (52 patients) noted that 50% were surgically managed at referring hospitals with a high morbidity and mortality, after which a regional referral and management algorithm was implemented in 2001. This study aims to reaudit specialist-managed liver trauma outcomes. METHODS: Prospective analysis of 99 patients (68 male) treated for liver injury (LI) between 2001 and 2008. Patient characteristics, management, and outcome results of these were compared with the results of previous audit. LI severity was determined by computed tomography, operative findings, and classified according to liver Organ Injury Scale. RESULTS: As implementation of guidelines, referrals increased from 5.2 patients/yr to 14.1 patients/yr, while LI profile was unchanged. Fewer patients were managed surgically with lower surgical intervention at referring hospitals (26 of 52 [50%] vs. 29 of 77 [38%]; p = 0.2). There has been a decrease in liver resection rates (14 of 26 [54%] vs. 3 of 37 [8%]; p = 0.0001]), overall mortality rate (12 of 52 [23%] vs. 11 of 99 [11%]; p = 0.059), and postoperative deaths. CONCLUSION: This reaudit confirms the role of nonoperative management of liver trauma. Early use of computed tomography scan with specialist discussion, selective use of perihepatic packing, and transfer to a specialist unit should be standard practice in the management of complex liver trauma.

Induction of hepatic 11beta-hydroxysteroid dehydrogenase type 1 in patients with alcoholic liver disease.

BACKGROUND AND AIM: The alcohol-induced pseudo-Cushing's syndrome is an important differential diagnosis of hypercortisolism that is poorly understood. Two isozymes of 11beta-hydroxysteroid dehydrogenase (11beta-HSD) interconvert hormonally active cortisol (F) and inactive cortisone (E). Previously we have shown higher urinary F:E metabolite ratios (a reflection of global 11beta-HSD activity) in patients with alcoholic liver disease (ALD) compared to patients with chronic liver disease (CLD) of other aetiologies, suggesting that the phenotype of alcoholic pseudo-Cushing's may relate to altered metabolism of F. SUBJECTS AND METHODS: We performed selective venous sampling of the hepatic, renal and peripheral veins measuring F and E concentrations (using in-house radioimmunoassay) in 20 patients with histologically confirmed ALD and 19 patients with CLD. Six patients who also had selective venous sampling for investigation of suspected hyperaldosteronism were used as 'normal' controls. RESULTS: There was a significant difference in the hepatic F gradient (mean +/- SEM) between groups, indicating increased F production in the liver in patients with ALD (34.5 +/- 21.7 nmol/l) compared to those with CLD (-21.0 +/- 18.5 nmol/l) (P < 0.05) and normals (-19.7 +/- 17.2 nmol/l) (P < 0.05). 11beta-HSD1 mRNA expression was increased fivefold in the ALD group compared with normal controls (P < 0.01). CONCLUSIONS: These results indicate significant induction of HSD11B1 gene expression and activity in patients with ALD during short- and long-term abstinence from alcohol. The mechanism is unknown but might be explained on the basis of alcohol-induced changes in intracellular redox potential or as a protective mechanism to limit liver inflammation and injury. Selective 11beta-HSD1 inhibitors may offer a novel therapeutic approach to treat alcoholic pseudo-Cushing's.

Transjugular intrahepatic portosystemic shunt in the management of Budd Chiari syndrome.

Budd Chiari syndrome presents with a wide range of severity and duration of symptoms. Transjugular intrahepatic portosystemic shunt has been used to treat selected Budd Chiari syndrome patients for several years. The technique of transjugular intrahepatic portosystemic shunt may be more challenging than in cirrhosis because of hepatic vein occlusion. Covered transjugular intrahepatic portosystemic shunt stents have reduced the requirement for follow-up interventions. Transjugular intrahepatic portosystemic shunt has been a successful bridge to liver transplant for Budd Chiari syndrome but is the definitive treatment in many cases. Patient selection is important to determine who will benefit from transjugular intrahepatic portosystemic shunt or other treatments such as hepatic vein recanalization or liver transplant.

Biliary dilatation and strictures after composite liver-small bowel transplantation in children: defining a newly recognized complication.

BACKGROUND: Biliary dilatation and strictures (BDS) are well recognized after liver transplantation but not reported after composite liver-small bowel transplantation (CLSBT). We aimed to describe and propose a classification of BDS in children undergoing CLSBT and analyze the potential risk factors. METHODS: Biliary complications of 47 consecutive children undergoing CLSBT were reviewed and classified according to presentation, location, and intervention required. The following variables were studied: small recipient (weight, <10 kg), donor-recipient weight ratio, liver/bowel graft reduction/not, partial/full pancreas, liver/bowel rejection, and median cold ischemia time (>454 min). RESULTS: Twenty-one (45%) children developed BDS at median 190 days (22 [7-138] months follow-up). Five distinct biliary lesions were identified. Most of the BDS (14/21; 67%) consisted of sphincter dysfunction-related bile duct dilatation (type I), whereas others (7/21; 33%) comprised extrahepatic bile duct (type II; n=3), hilar (type III; n=1), segmental (type IV; n=1), and diffuse (type V; n=2) intrahepatic strictures. None of the graft reduction strategies or other variables studied demonstrated a significant association with BDS. Therapeutic intervention was required in 1 of 14 type I and four of seven type II to V BDS in the form of percutaneous biliary dilatation with or without drainage. CONCLUSION: This article identifies BDS after CLSBT as a frequent late complication after CLSBT, which has a benign outcome in most cases. The natural history of children with extrahepatic and intrahepatic strictures is not yet clear and will need multicenter prospective studies.

Polyarteritis nodosa, presenting as life-threatening gastrointestinal hemorrhage in a liver transplant recipient.

This unique case reports the first recorded episode in the medical literature of vasculitis post-liver transplantation, presenting as life-threatening gastrointestinal hemorrhage. A 52-year-old Caucasian woman underwent orthotopic liver transplantation (OLT) for autoimmune cirrhosis complicated by hepatoma and portal vein thrombosis. Late hepatic artery thrombosis led to a second liver graft. Following recovery from an episode of acute rejection, the patient presented with large volume hematemesis, melena, and hemochezia (passage of fresh blood from the rectum). Following upper and lower gastrointestinal endoscopy and surgery, angiography illustrated the presence of polyarteritis nodosa (PAN), which was successfully treated with high-dose steroid therapy. Gastrointestinal hemorrhage is an unusual presentation of vasculitis, especially PAN. The occurrence of this phenomenon post-OLT, in the presence of immunosuppression is previously unreported.

Results of a retrospective multicenter trial of the Viatorr expanded polytetrafluoroethylene-covered stent-graft for transjugular intrahepatic portosystemic shunt creation.

PURPOSE: To report the results of a multicenter experience with the Viatorr expanded polytetrafluoroethylene-covered stent-graft for transjugular intrahepatic portosystemic shunt (TIPS) creation in which patency and clinical outcome were evaluated. MATERIALS AND METHODS: One hundred consecutive patients with portal hypertension, with a mean age of 52 years (range, 22-86 years), underwent implantation of the Viatorr TIPS stent-graft at one of three hospital centers. The indications for TIPS creation were variceal bleeding (n = 81) and refractory ascites (n = 19). Twenty patients had Child-Pugh class A disease, 46 had class B disease, and 34 had class C disease. Eighty-seven patients underwent de novo TIPS placements, with 13 treated for recurrent TIPS stenosis. Sixty-two patients were available for follow-up portal venography and portosystemic pressure gradient (PSG) measurement commencing 6 months after Viatorr stent-graft placement. RESULTS: The technical success rate was 100%. TIPS creation resulted in an immediate decrease in mean PSG (+/-SD) from 21 mm Hg +/- 6 to 7 mm Hg +/- 3. Acute repeat intervention (within 30 days) was required for portal vein thrombosis (n = 1), continued bleeding (n = 3), and encephalopathy (n = 1). The all-cause 30-day mortality rate was 12%. Two patients developed acute severe refractory encephalopathy, which led to death in one case. New or worsening encephalopathy was identified in 14% of patients. The incidence of recurrent bleeding was 8%. The cumulative survival rate at 1 year was 65%. Sixty-two patients available for venographic follow-up had a mean PSG of 9 mm Hg +/- 5 at a mean interval of 343 days (range, 56-967 days). There were four stent-graft occlusions (6%) and seven hemodynamically significant stenoses (11%), four within the stent-graft and three in the non-stent-implanted hepatic vein. The primary patency rate at 1 year by Kaplan-Meier analysis was 84%. CONCLUSIONS: This retrospective multicenter experience with the Viatorr stent-graft confirms the preliminary findings of other investigators of good technical results and improved patency compared with bare stents. Early mortality and symptomatic recurrence rates are low by historical standards. The theoretical increase in TIPS-related encephalopathy was not demonstrated. Longer-term follow-up will be required to determine whether the additional cost of the Viatorr stent-graft will be offset by reduced surveillance and repeat intervention.