Simon Olliff

TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients.

BACKGROUND & AIMS Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder secondary to hepatic venous outflow obstruction. Small series of BCS patients indicate that transjugular intrahepatic portosystemic shunt (TIPS) may be useful. However, the influence of TIPS on patient survival and factors that predict the outcome of TIPS in BCS patients remain unknown. METHODS One hundred twenty-four consecutive BCS patients treated with TIPS in 6 European centers between July 1993 and March 2006 were followed until death, orthotopic liver transplantation (OLT), or last clinical evaluation. RESULTS Prior to treatment with TIPS, BCS patients had a high Model of End Stage Liver Disease and high Rotterdam BCS prognostic index (98% of patients at intermediate or high risk) indicating severity of liver dysfunction. However, 1- and 5-year OLT-free survival were 88% and 78%, respectively. In the high-risk patients, 5-year OLT-free survival was much better than that estimated by the Rotterdam BCS index (71% vs 42%, respectively). In the whole population, bilirubin, age, and international normalized ratio for prothrombin time independently predicted 1-year OLT-free survival. A prognostic score with a good discriminative capacity (area under the curve, 0.86) was developed from these variables. Seven out of 8 patients with a score >7 died or underwent transplantation vs 5 out of 114 patients with a score <7. CONCLUSIONS Long-term outcome for patients with severe BCS treated with TIPS is excellent even in high-risk patients, suggesting that TIPS may improve survival. Furthermore, we identified a small subgroup of BCS patients with poor prognosis despite TIPS who might benefit from early OLT.

A re‐evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts

Previously, we have found that the absence of the colon after liver transplantation (LT) protects the patient from recurrent primary sclerosing cholangitis (rPSC). As our previous observation has not been confirmed in other series, we have reviewed our cohort of patients grafted for primary sclerosing cholangitis (PSC) with greater numbers and longer follow‐up to reassess the rate, consequences, and risk factors for rPSC. We collected data on patients who underwent LT for PSC between January 1986 and April 2006. Data were collected for cytomegalovirus status, inflammatory bowel disease status, time of colectomy, type of colectomy, donor‐recipient gender mismatch, recipient sex, extended donor criteria (EDC), and donor risk index. Accepted criteria were used to diagnose rPSC. Of a total of 230 consecutive adult patients, 61 (27%) underwent colectomy pre‐/peri‐LT, and 54 (23.5%) developed rPSC at a median of 4.6 (range, 0.5–12.9) years post‐LT. A total of 263 deceased donor grafts were used, and 73 were EDC grafts. A diagnosis of rPSC was made in 61 of the 263 grafts (23%). The recurrence‐free patient survival was significantly better (P < 0.05) in patients who underwent pre‐/peri‐LT colectomy and in those with non‐EDC grafts. In conclusion, in this larger cohort of 230 patients and with longer follow‐up of 82.5 (range, 0.0–238.6) months [in comparison with the previous report of 152 recipients with a follow‐up of 52.8 (range, 1–146) months], we have shown that colectomy remains a significant risk factor for rPSC and that colectomy before and during initial LT for PSC confers a protective effect against rPSC in subsequent graft(s). Moreover, we have shown that EDC grafts are also a significant risk factor for rPSC. Liver Transpl 15:330–340, 2009.

Risk factors for recurrence of primary sclerosing cholangitis of liver allograft

Primary sclerosing cholangitis (PSC) is a disease of unknown cause that effects the biliary tree and is closely associated with inflammatory bowel disease. We did a retrospective analysis of the risk factors associated with recurrence of PSC in an allograft after liver transplantation. Recurrence of disease, assessed by liver histology or imaging the biliary tree, occurred in 56 of 152 patients (37%) at a median of 36 months (range 1.4-120 months). Multivariate analysis showed that being male (relative risk 1.2, 95% CI 0.73-2.15) and an intact colon before transplantation (8.7, 1.19-64.48) were associated with recurrence. These observations could help elucidate the pathogenesis of the disease.

Presence and severity of non-alcoholic fatty liver disease in a large prospective primary care cohort.

BACKGROUND & AIMS Non-alcoholic fatty liver disease (NAFLD) is a common cause of abnormal LFTs in primary care, but there are no data defining its contribution nor reporting the range of NAFLD severity in this setting. This study seeks to calculate the range of disease severity of NAFLD in a primary care setting. METHODS Adult patients with incidental abnormal LFTs, in the absence of a previous history, or current symptoms/signs of liver disease were prospectively recruited from eight primary care practices in Birmingham. NAFLD was diagnosed as fatty liver on ultrasound, negative serological liver aetiology screen, and alcohol consumption ≤30 and ≤20 g/day in males and females, respectively. The NAFLD Fibrosis Score (NFS) was calculated to determine the presence or absence of advanced liver fibrosis in subjects identified with NAFLD. RESULTS Data from 1118 adult patients were analysed. The cause of abnormal LFTs was identified in 55% (614/1118) of subjects, with NAFLD (26.4%; 295/1118) and alcohol excess (25.3%; 282/1118) accounting for the majority. A high NFS (>0.676) suggesting the presence of advanced liver fibrosis was found in 7.6% of NAFLD subjects, whereas 57.2% of NAFLD patients had a low NFS (<-1.455) allowing advanced fibrosis to be confidently excluded. CONCLUSIONS NAFLD is the commonest cause of incidental LFT abnormalities in primary care (26.4%), of whom 7.6% have advanced fibrosis as calculated by the NFS. This study is the first of its kind to highlight the burden of NAFLD in primary care and provide data on disease severity in this setting.

Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome

Background: We report our experience with management of patients with Budd Chiari syndrome over the past two decades. In 1996 we described a novel approach involving recanalisation of hepatic veins by combined percutaneous and transvenous approaches. This was incorporated into an algorithm published in 1999 in which our preferred treatment for all cases of Budd Chiari syndrome with short segment occlusion or stenosis of the hepatic veins involves recanalisation of the hepatic veins by transvenous or combined percutaneous-transvenous approaches. In symptomatic Budd Chiari syndrome where recanalisation is not possible, we perform transjugular intrahepatic portosystemic shunts (TIPS) because TIPS decompresses the portal circulation directly in an adjustable way. In this series of patients with Budd Chiari syndrome treated with radiological interventions alone, we assess their medium term outcome using two independent objective prognostic indices. Methods: We retrospectively studied 61 patients with non-malignant Budd Chiari syndrome treated by radiological intervention alone in our centre. Results: Actuarial survival for the entire cohort at one year and five years was 94% and 87%, respectively. Survival of our patients with mild disease (according to the Murad classification) was 100% at one year and at five years, with intermediate disease severity 94% at one year and 86% at five years, and with severe disease 85% at one year and 77% at five years. Conclusion: Management of Budd Chiari syndrome by interventional radiology resulted in excellent medium term survival for patients in all categories of disease severity.

PTFE-covered stents improve TIPS patency in Budd-Chiari syndrome.

Transjugular intrahepatic portosystemic shunt (TIPS) have been shown to be an efficient portal‐systemic derivative treatment for Budd‐Chiari syndrome (BCS) patients uncontrolled by medical therapy. However, the main drawback of TIPS for this condition is a very high rate of shunt dysfunction. Recently, polytetrafluoroethylene (PTFE)‐covered stents have been shown to reduce the incidence of TIPS dysfunction in patients with cirrhosis. The aim of the study was to assess the incidence of TIPS dysfunction in 2 cohorts of BCS patients treated with bare or PTFE‐covered stents. The study included 25 TIPS procedures (16 bare stents and 9 covered stents) with a median follow‐up period of 20.4 months (range, 3.9‐124.8). Fourteen of 16 patients (87%) receiving bare stents had TIPS dysfunction compared to 3 of the 9 patients (33%) receiving PTFE‐covered stents (P = .005). The actuarial rates of primary patency in the bare‐stent group were 19% at 1 year compared with 67% in the PTFE‐covered stent group (P = .02; log‐rank test). The number of additional interventional procedures to maintain TIPS patency was significantly greater in the bare‐stent than in the PTFE‐covered stent group (1.9 ± 1.2 vs. 0.6 ± 0.9; P = .007). The number of patients with clinical relapses was greater in the bare‐stent group compared to the PTFE‐covered stent group (13 vs. 5 episodes in 9 and 3 patients, respectively). In conclusion, PTFE‐covered stents have a considerable advantage over bare stents for the TIPS treatment of BCS patients, with a lower dysfunction rate, a lower number of reinterventions, and fewer prosthesis requirements. PTFE‐covered stents are preferable in patients with Budd‐Chiari Syndrome. (HEPATOLOGY 2004;40:1197–1202.)

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.

Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome.

There have been encouraging reports on transjugular intrahepatic portosystemic stent‐shunt (TIPSS) for Budd–Chiari syndrome (BCS). Long‐term data are lacking.

Cholangiocarcinoma Complicating Primary Sclerosing Cholangitis: A 24-Year Experience

Aim: To report the prevalence and outcome of cholangiocarcinoma arising in primary sclerosing cholangitis for a British tertiary referral centre. Methods: All patients diagnosed with primary sclerosing cholangitis and concurrent cholangiocarcinoma were identified from a prospectively maintained departmental database, and the mode of presentation, management and outcome were determined. Results: Of 370 patients with primary sclerosing cholangitis, 48 patients (13%) were diagnosed with a cholangiocarcinoma within a median time of 0.51 months (range: 0–73.12) from presentation to the unit. Mode of presentation included: inoperable tumours (n = 14); incidental findings in transplant hepatectomy specimens (n = 13); primary sclerosing cholangitis follow-up (n = 9); transplant work-up (n = 5); transplant waiting list (n = 5); suspected tumour confirmed at transplant (n = 1), and incidental finding at cholecystectomy (n = 1). The diagnosis was confirmed by: radiology-guided biopsy (n = 27); MRI (n = 3); CT (n = 2); laparoscopy or laparotomy (n = 2), and frozen section at transplant (n = 1). Management consisted of: transplantation (n = 14, including 1 abandoned); hepatic resection (n = 8), and palliation through stenting (n = 26). The overall median survival of the cohort was 4.9 months (range: 0.09–104.5). Median survival ranged from 2.6 months (range: 0.09–35.3) for palliation to 7.6 months (range: 0.6–99.6) for transplantation and 52.8 months (range: 3.7–104.5) for resection. There was no difference in survival between the transplant and resection groups (p = 0.14). Conclusions: Cholangiocarcinoma is a common finding in primary sclerosing cholangitis and regular screening of this cohort of patients at referring centres is advocated to detect early tumours, as surgical treatment at an early stage offers significantly better outcomes for this cohort of patients.

Symptomatic portal biliopathy: a single centre experience from the UK.

Background and methods Biliary obstruction as a consequence of portal biliopathy, because of extrahepatic portal vein occlusion is an uncommon cause of biliary disease in the western world. We reviewed all patients presenting to the Regional Liver Transplant Unit in Birmingham, UK with symptomatic portal biliopathy between 1992 and 2005 and report the presentation, investigation, management and outcome of these complex patients. Results Thirteen patients with symptomatic portal biliopathy were followed up for a median of 2 years (range 1–18 years). Jaundice was the presenting feature in all cases and was associated with bile duct stones or debris in 77% (10 of 13) of cases. Successful treatment of biliary problems was achieved by biliary decompression in six cases (metallic stent=three, plastic stent=one, combined procedure=one and sphincterectomy=one) and portal decompression in three cases (transjugular intrahepatic portosystemic shunt=two, meso-caval shunt=one). Successful biliary drainage could not be achieved endoscopically or by portal decompression in one case that was accepted for combined liver and small bowel transplantation. Three patients had spontaneous resolution without recurrence over the follow-up period. Ten patients (77%) experienced gastrointestinal bleeding. Two deaths over the follow-up period occurred; both were associated with portal hypertensive bleeding. Conclusion Endoscopic management (sphincterectomy and stone extraction or stent insertion) is effective initial therapy for patients with symptomatic portal biliopathy. In the case of persistent biliary obstruction porto-systemic shunting (transjugular intrahepatic portosystemic shunt or surgical) should be considered, however, the extent of vascular thrombosis precludes this in most cases.