Anaesthetic considerations for liver transplantation in propionic acidemia

Propionic acidemia (PA) is an autosomal recessive disorder of metabolism due to deficiency of the enzyme propionyl-CoA carboxylase (PCC) that converts propionyl-CoA to methylmalonyl-CoA with the help of the cofactor biotin inside the mitochondria. The resultant accumulation of propionyl-CoA causes severe hyperammonaemia and life-threatening metabolic acidosis. Based on the positive outcomes, liver transplantation is now recommended for individuals with recurrent episodes of hyperammonaemia or acidosis that is not adequately controlled with appropriate medical therapies. We report anaesthetic management of two children with PA for liver transplantation at our institution. It is essential for the anaesthesiologist, caring for these individuals to be familiar with the manifestations of the disease, the triggers for decompensation and management of an acute episode

Anaesthesia and intensive care for simultaneous liver-kidney transplantation: A single-centre experience with 12 recipients

Background and Aims: The perioperative management of patients presenting for simultaneous liver and kidney transplantation (SLKT) is a complex process. We analysed SLKTs performed in our institution to identify preoperative,intraoperative and post-operative challenges encountered in the management. Methods: We retrospectively studied the case records of 12 patients who underwent SLKT between 2009 and 2014 and analysed details of pre-operative evaluation and optimisation, intraoperative anaesthetic management and the implications of use of perioperative continuous renal replacement therapy (CRRT) and the post-operative course of these patients. Results: Of the total 12 cases, 4 were under 16 years of age. The indications for SLKT were primary hyperoxaluria (5), congenital hepatic fibrosis with polycystic kidney disease (2), ethanol-related end-stage liver disease (ESLD) with hepatorenal syndrome type 1 (1). Four patients had ESLD with end-stage renal disease due to other causes. Six recipients received live donor grafts and 6 patients received cadaveric grafts. Seven patients received intraoperative CRRT. Mean duration of surgery was 12.5 h. Cardiac output monitors used were trans-oesophageal echocardiogram (2), pulmonary artery catheter (1) and pulse contour cardiac output monitor (3). There was 1 sepsis-related mortality on 7th post-operative day. Conclusion: A thorough pre-operative evaluation and optimisation, knowledge and anticipation of potential problems, and meticulous intraoperative fluid management guided by appropriate monitoring and use of CRRT when needed can help in achieving successful outcomes

An international, multicenter, survey-based analysis of practice and management of acute liver failure

International audienceINTRODUCTION: Acute liver failure (ALF) is an acute liver dysfunction with coagulopathy and hepatic encephalopathy in a patient with no known liver disease. As ALF is rare and large clinical trials are lacking, the level of evidence regarding its management is low-moderate, favoring heterogeneous clinical practice. In this international multicenter survey study, we aimed to investigate the current practice and management of patients with ALF. METHODS: An online survey targeting physicians who care for patients with ALF was developed by the International Liver Transplantation Society ALF Special-Interest Group. The survey focused on management and liver transplantation (LT) practices of ALF. Survey questions were summarized overall and by geographic region. RESULTS: A total of 267 physicians completed the survey with a survey response rate of 21.36%. Centers from all continents were represented. More than 90% of physicians were specialized in either transplant hepatology/surgery or anesthesiology/critical care. Two hundred and fifty-two (94.4%) respondents’ institutions offered LT. A total of 76.8% of respondents’ centers had a dedicated liver- or transplant- intensive care unit (p<0.001). Median time to LT was within 48 hours in 12.7% of respondents’ centers, 72 hours in 35.6%, one week in 37.6%, and more than one week in 9.6% (p<0.001). Deceased-donor liver graft (49.6%) was the most common type of graft offered. For consideration of LT, 84.8% of physicians used King’s College Criteria and 41.6% used Clichy Criteria. Significant differences were observed between Asia, Europe, and North America for offering LT, number of LTs performed, volume of ALF patients, admission to a dedicated intensive care unit, median time to LT, type of liver graft, monitoring hepatic encephalopathy and intracranial pressure, management of coagulopathy, and utilization of different criteria for LT. DISCUSSION: In our study, we observed significant geographic differences in the practice and management of ALF. As ALF is rare multicenter studies are valuable to identify global practice