Congenital diseases with multi-organ expression as an indication for liver transplantation in children during the first years of life: Integrative approach to improve the outcomes

Abstract

Pediatric liver transplantation has evolved since the first transplant was performed by Dr. Starzl more than 50 years ago. Innovations in surgical techniques, immunosuppression, and infection control have significantly improved patient outcomes, graft survival, and quality of life. However, further progress depends not only on the discovery of new treatment option but should rely upon the identification of the factors in the perioperative care which contribute to successful outcomes and guide our decision-making process.

Indications for pediatric liver transplantation can be divided into three major groups: cholestatic liver conditions, liver based metabolic disorders, and others represented by acute liver failure of different etiologies, tumors, inherited autoimmune diseases, and multiple infectious causes.

Many of these conditions manifest themselves with multi organ pathologic expression, requiring earlier interventions to treat acute liver failure or chronic cholestasis leading to failure to thrive. Containment of patient pathology and maintenance of optimal neurologic and extra-hepatic visceral organ function is paramount during the pre - transplant period.

Multidisciplinary management of comorbid conditions in complex pediatric liver transplant candidates during the perioperative period with the emphases on optimization and patient selection has never been summarized in the literature. Thant is why this review focuses on a comprehensive, multidisciplinary preoperative evaluation of pediatric patients in whom progressive liver disfunction manifests early in life and eventually requires liver transplantation. We will address multi-organ system involvement, preoperative optimization in anticipation of the complexity of intraoperative care, as well as short- and long-term postoperative management.