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Introduction
Around 500,000 children are born every year in Italy; and since it has been estimated that two children out of 10,000 newborns have liver diseases which will require a liver transplant, this means that in Italy the need for a pediatric transplant translates into approximately 100 cases per year. 2. Transplant assessment and preparation 3. Transplant surgery techniques Liver transplant is the therapy of choice for a number of liver diseases in the pediatric age that develop into end-stage liver failure. Cholestatic diseases and metabolic congenital defects are the two groups of diseases that on the whole require a transplant in more than 90% of children. Other liver diseases that do not bring about end-stage liver disease can also lead to the need for a liver transplant. These are: Non-progressive liver diseases with greater morbility than in liver transplants
Cystic fibrosis
Children eligible for a liver transplant should undergo an assessment as soon as possible to outline effective need for transplant and identify the clinical conditions that can be treated by a medical therapy. At the end of the pre-transplant assessment, it is important to define as accurately as possible the severity rate of the disease in order to establish the urgency level of the single patient within the waiting list, as compared to the other candidates. At the end of the assessment process, provided the medical conditions allow for it, the patient is sent home after having contacted the referring pediatrician, who is requested to inform the transplant center about any significant variation in the child's clinical situation.
In whole-liver transplants,
the whole liver is procured from a cadaveric donor whose weight is similar to
the recipient's. Usually the weight ratio between donor and recipient is 1: 2.
Reduced-liver transplant. With this kind of surgery, the whole liver is procured from an adult cadaveric donor and a procedure similar to liver resection is performed on it on the backtable. Though using adult livers for children solves the problem of the lack of adequate-sized organs for pediatric recipients, in practice this technique has shifted the issue from pediatric to adult waiting lists (much longer).Living donor liver transplant. In 1988, we find the first description of a surgery in which the II and the III segment has been procured from a living donor (mother) and transplanted into a child with biliary atresia. Living donor liver transplants have been widely discussed with reference to the ethics of having a healthy person undergo major surgery, such as left hepatectomy, with all related risks. The legitimacy of this procedure has been recognized, and more than 1,200 surgeries of this kind have been performed worldwide, with a mortality and morbility for the donors of approximately 0.2% and 10% respectively. To minimize the risks associated with the operation, potential donors undergo extremely accurate tests to assess their psychophysical eligibility. For pediatric recipients, living donor transplants in most cases produce excellent results, due mainly to the possibility of transplanting children before their clinical conditions worsen. The main goals of long-term follow-up of pediatric patients after liver transplant are: Monitor the graft's functions. Even months or years after the transplant, some changes in liver function levels may occur, which need to be correctly interpreted and treated. Monitor side effects and drug interactions. The medications used after a liver transplant have a number of side effects which should be adequately monitored and, if possible, prevented. With children, one of the main goals of medium- and long-term follow-up is to foster their growth, often seriously compromised by the liver disease. It has been proved that adequate growth rarely occurs when cortisone is chronically administered. Every-other-day administration and steroid suspension are goals to be strenuously pursued. Both cyclosporine and tacrolimus are nephrotoxic drugs. It seems almost certain that the most severe kidney damage occurs in the first weeks following the transplant, when the levels of these drugs are at their highest. Psycological Support. A number of research studies have proved that, even in the long term, serious liver diseases in pediatric patients negatively affect the development of the central nervous system and of the cognitive faculties. Also, given the period of psychological stress for both patients and families, liver transplants may leave emotional scars. Pediatric transplant recipients should be adequately assisted from a psychological standpoint at least for the first months or years after the transplant. Psychological support may often be significant for the whole family, which is sometimes seriously affected. Thanks to modern immunosuppressants, new surgical techniques which have virtually eliminated mortality from pediatric waiting lists, and improved knowledge of long-term issues regarding transplanted patients, transplanted children can grow and live an almost ordinary life. However, liver transplants, like other the organ transplant procedures are not finished products, and there is large room for improvement, which will in large part be made possible thanks to research. Recent advances in surgical techniques, particularly the split liver, have virtually eliminated mortality from pediatric waiting lists, and after five years the expected survival rate is nowadays approximately 85%. It is feasible for transplanted children to become adults and live an almost normal social life except for the tests they will continually have to undergo to assess the correct functioning of the transplant and monitor the risks of chronic immunosuppression. At the moment, accurate long-term follow-up is the most powerful tool to safeguard the health of transplanted children. |
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Since January 2004, a pediatric liver transplant program has been implemented
at ISMETT in Palermo with the goal of meeting pediatric needs of central
and southern Italy.
Surgeries
currently performed for pediatric liver transplants are:
