Frederick Chen

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.

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

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.

Outcomes of pregnancy in patients with known Budd-Chiari syndrome

AIM To analyse the risk of pregnancy (a prothrombotic state) in patients with Budd-Chiari Syndrome (BCS). METHODS Retrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015. RESULTS Out of 53 females with BCS, 7 women had 16 pregnancies. Median age at diagnosis of BCS in these women was 25 years (range 21-34 years). At least one causal factor for BCS was identified in 6 women (86%). Six women had undergone radiological decompressive treatment. All patients had anticoagulation. Six fetuses were lost before 20 wk gestation in 2 women. There were 9 deliveries over 32 wk gestation and one delivery at 27 wk. All infants did well. Seven babies were born by emergency caesarean section. There were no cases of thrombosis. Two patients had notable vaginal (PV) bleeding in 3 pregnancies. None of the patients had variceal haemorrhage. Two patients were diagnosed with pulmonary hypertension, one during pregnancy and the other in the post-partum period. There was no maternal mortality. CONCLUSION Maternal outcomes in patients with treated BCS are favourable and fetal outcomes beyond 20 wk gestation are good. There has been increased rate of caesarean section. Pulmonary hypertension is an important finding that needs further validation. These patients should be managed in centers experienced in treating high-risk pregnancies.

Is it time to revisit contraindications to organ donation from donors with a JAK-2 mutation? Safe use of a liver allograft from a donor with essential thrombocythaemia

Transplantation can cure end‐stage liver disease and hepatocellular carcinoma. However, the balance of organ demand and provision is heavily tipped to the detriment of patients. Patients awaiting transplantation rely on the greater use of marginal donors that may carry a risk to the recipient. UK authorities have decreed donor haematological malignancy an absolute contraindication. The authors describe the first report of a patient being safely transplanted with a liver from a donor who suffered from JAK2 V617F mutation‐driven essential thrombocythaemia to a patient with a critical burden of hepatocellular carcinoma. A year after transplantation, the patient has neither evidence of acquisition of the donors pathology, nor evidence of carcinoma recurrence. The case highlights the responsibility of the recipient team to maximize the use of organs by expert risk assessment. Dissemination of experience should inform future decisions, benefit patients and bolster utility in an era of growing waiting‐list mortality.

Case Report: A Rare and Unusual Cause of Acute Liver Failure

Budd-Chiari syndrome (BCS) is a rare condition caused by hepatic outflow obstruction. Typical clinical features include right upper quadrant pain, ascites and hepatosplenomegaly. We report a case of a 26 year old female who presented with jaundice, worsening abdominal swelling and abdominal pain. She subsequently developed acute liver failure and spontaneous bacterial peritonitis (SBP) with significant renal impairment requiring organ support. Computed tomography (CT) scan showed congested liver and occlusion of three hepatic veins consistent with Budd- Chiari Syndrome. Transjugular intra hepatic portosystemic stent/shunt (TIPSS) was inserted which resulted in complete resolution liver and renal function. She was subsequently found to be positive for the JAK-2 V617F mutation suggesting an underlying myeloproliferative disorder. This is a rare case in which a patient developed spontaneous bacterial peritonitis (SBP) in the context of BCS. The patient had excellent outcome from TIPSS procedure, which rescued her from undergoing liver transplantation.

Commentary: TIPSS for Budd-Chiari syndrome - authors' reply

gle-handedly by two experienced physicians, cases had been carefully selected (only 68 of the 104 patients actually underwent TIPSS) and all patients were maintained on full anticoagulation throughout. It would have been helpful to know what criteria were used for patient selection to generalise these findings, and whether the improved survival was a function of effective anticoagulation. Owing to the lack of randomised controlled evidence and the relative rarity of BCS, the management of BCS will likely continue to be based on expert opinions and local expertise. In general, recanalisation attempts are advocated as the first line, and TIPSS as the rescue step when the former is unsuitable or ineffective. While angioplasty is common in Asia and surgical shunts are favoured in certain US centres, 6 TIPSS has become increasingly popular in Europe. 7 Given the feasibility, safety, efficacy profile and excellent long-term survival, the authors add to the growing body of evidence regarding the importance of TIPSS as a viable option for Budd-Chiari syndrome. ACKNOWLEDGEMENT Declaration of personal and funding interests: None.