Contemporary Pediatric Liver Transplantation: Complex Challenges, Evolving Solutions

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Excerpt

Just a quarter century ago, children with life-threatening acute and chronic end-stage liver disease faced an often insurmountable list of barriers to successful liver transplantation (LTx). The incidence of death while waiting for LTx was exceedingly high, especially in infants and toddlers, because of an insufficient number of size-matched liver donors. To make matters worse, posttransplant early graft survival remained below 70% until the early 1990s. It is a testament to the collaborative efforts of innovative surgeons, hepatologists, intensivists, and anesthesiologists that LTx now affords these children with ready access to this ultimate of therapies with an expectation of early (1 year) patient survival reaching and even exceeding 90% according to some registries.1,2 LTx physicians, emboldened by these outcomes, are now increasingly willing to offer transplant as an option even in patients with complexities once thought to be prohibitive. Numerous challenges that often arise in the perioperative period remain as potential impediments to the full benefits afforded by successful LTx. Arbitrarily these can be categorized as: (1) disease-specific abnormalities such as the splenic malformation syndrome and various hepatic-based metabolic derangements; (2) pulmonary disease related to liver dysfunction; (3) the allograft itself, most notably when it is of an incompatible blood type (iABO) or is procured from a donor after cardiac death; and (4) the inherent size conundrum inherent to infants and small recipients (<5 kg).
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