Date Published: February 23, 2018
Publisher: Public Library of Science
Author(s): Marek Szymczak, Piotr Kaliciński, Grzegorz Kowalewski, Dorota Broniszczak, Małgorzata Markiewicz-Kijewska, Hor Ismail, Marek Stefanowicz, Adam Kowalski, Joanna Teisseyre, Irena Jankowska, Waldemar Patkowski, Stanislaw Stepkowski.
Living donor liver transplantation (LDLT) in patients with acute liver failure (ALF) has become an acceptable alternative to transplantation from deceased donors (DDLT). The aim of this study was to analyze outcomes of LDLT in pediatric patients with ALF based on our center’s experience.
We enrolled 63 children (at our institution) with ALF who underwent liver transplantation between 1997 and 2016. Among them 24 (38%) underwent a LDLT and 39 (62%) received a DDLT. Retrospectively analyzed patient clinical data included: time lapse between qualification for transplantation and transplant surgery, graft characteristics, postoperative complications, long-term results post-transplantation, and living donor morbidity. Overall, we have made a comparison of clinical results between LDLT and DDLT groups.
Follow-up periods ranged from 12 to 182 months (median 109 months) for LDLT patients and 12 to 183 months (median 72 months) for DDLT patients. The median waiting time for a transplant was shorter in LDLT group than in DDLT group. There was not a single case of primary non-function (PNF) in the LDLT group and 20 out of 24 patients (83.3%) had good early graft function; 3 patients (12.5%) in the LDLT group died within 2 months of transplantation but there was no late mortality. In comparison, 4 out of 39 patients (10.2%) had PNF in DDLT group while 20 patients (51.2%) had good early graft function; 8 patients (20.5%) died early within 2 months and 2 patients (5.1%) died late after transplantation. The LDLT group had a shorter cold ischemia time (CIT) of 4 hours in comparison to 9.2 hours in the DDLT group (p<0.0001). LDLT is a lifesaving procedure for pediatric patients with ALF. Our experience showed that it may be performed with very good results, and with very low morbidity and no mortality among living donors when performed by experienced teams following strict procedures.
Although the first reported case of acute liver failure (ALF) was described in 1946, the definition was introduced in 1970 by Trey and Davidson . Indeed, ALF is usually defined as a clinical syndrome characterized by an abrupt onset of jaundice and hepatic encephalopathy within 8 weeks after first clinical symptoms, often in the absence of any liver disease . However, this generally accepted definition does not fully apply to ALF in children. Encephalopathy in children, particularly in infancy, may occur much later or not at all during ALF development. Even when encephalopathy symptoms are present, clinical ALF is often difficult to diagnose. The first definition of ALF in children was introduced by Bhaduri and Mieli-Vergani in 1996 . According to this definition, it is a multisystem disorder in which there is a severe impairment of the liver function with or without encephalopathy, but with hepatocellular necrosis in children who did not have any symptoms of chronic liver disease. Overall, the outcome of liver transplantation (LT) for ALF is worse in children than in adults and especially worse when compared to the results of LT for chronic liver diseases; it is also associated with high mortality [3, 4]. Indeed, the survival of liver transplant recipients for ALF depends mainly on the urgent (hours or days) availability of a suitable donor. This is particularly difficult in pediatric patients as the possibility of harvesting a matching liver from a deceased donor (DD) is especially unpredictable.
Between 1990 and 2016 there were 689 LTs performed in pediatric patients in Children’s Memorial Health Institute (CMHI), including 312 LDLTs (45%). Among 689 LT patients, 63 were children transplanted for ALF (9.1%) between 1997 and 2016: 24 (38%) underwent LDLT and 39 (62%) were transplanted with a graft from a deceased donor (DDLT). Patients were qualified for LT based on the King’s College three criteria, or specific criteria in a case of Wilson’s disease.
In recent years, there has been significant progress in the treatment of ALF. This progress is mainly attributed to dramatic improvements in the quality of medical care, especially in intensive care techniques, including multiple excellent extracorporeal procedures such as hemodialysis and albumin-dialysis. Despite these undisputable successes in patients’ management, the emergency LT (LDLT or DDLT) remains as the only life-saving procedure for patients with ALF. In fact, all of the improved emergency level procedures only help to prolong patients’ temporary survival while awaiting LT . ALF almost always requires urgent LT as these patients deteriorate very rapidly and often end up with MOF. The clinical management of ALF patients requires knowledge, experience, extraordinary accuracy, consistency, and the ability to make the right decisions under pressure to save patients’ lives. Since ALF is a multiorgan disease with very unpredictable outcomes the deterioration of patients’ condition can occur rapidly in a matter of hours or days and therefore the waiting time for LT is limited . This is especially important while facing organ shortage in a pediatric population.  Independently of the best efforts, many patients with ALF die shortly before LT or are disqualified from LT because of existing contraindications . Despite improvements in organ allocation, the mortality of patients with ALF who are waiting for LT is significantly higher than among patients who are waiting for a donor for any other reason. Moreover, the long-term results of LT are worse for recipients with ALF in comparison to recipients with any chronic liver insufficiency, despite clear progress in the intensive care management prior and after LT [7,11,12].