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Available and emerging robotic systems

Introduction Since the initial development of the Green Telepresence system robotic platform in 1986, robotic-assisted surgery has dramatically transformed the landscape of surgical care. With the evolution of minimally invasive surgical approaches, robotic-assisted surgery improved upon the ergonomic and visualization limitations of laparoscopy while maintaining the advantages of this less invasive approach. It has been rapidly adopted within general surgery and the surgical sub-specialties, and it…

The history of robotic surgery

A full anthology of the development of surgical robotics would require a complete textbook. Perhaps we could start the story with Leonardo da Vinci and his genius with a 16th century drawing of a mechanical knight ( Fig. 1.1 ). The Intuitive Surgical Company recognized the genius of Leonardo by naming their robotic systems in his honor, including the current and most well-known surgical robot, the…

Future of Liver Transplantation in Children

Introduction Liver transplantation was a dream only a century or so ago. The idea of replacing a diseased organ with a new one was a matter of science fiction. However, innovations in surgery, the development of intensive care and anesthesia, progress in the understanding of how the immune system works, and the introduction of effective immunosuppressive medication have led to the advent of clinical programs of…

Challenges for Young People With Liver Disease Including Transition to Adult Services

Long-term outcomes following liver transplantation (LT) are inferior for adolescents and young adults in comparison with younger children and adults. Adolescence is a transitional stage of psychosocial development and a time of rapid physical growth and change. This developmental period includes sexual maturation, cognitive development, emotional changes, and the transition from total social and economic dependence to relative independence. Insight into the unique neurodevelopmental changes that…

Prospects for Immune Tolerance

Introduction The extraordinary success of liver transplantation (LT) as a treatment for end-stage liver diseases has resulted in the need to redirect research priorities and clinical resources toward improving the health and well-being of what are now thousands of long-term surviving LT recipients. This requires the optimization of pharmacological immunosuppression to prevent immune-mediated liver damage while minimizing toxicity. The routine management of immunosuppressive drugs currently relies…

Chronic Graft Injury

Introduction Patient and graft survival after pediatric liver transplantation (LT) has improved significantly: children undergoing LT have a patient survival of 79% and graft survival of 63% 10 years post-transplant, according to the European Liver Transplant Registry data . In 2018, a single-center study reported patient and graft survival rates of 83% and 73% at 10 years and 79% and 64% at 20 years after pediatric…

Psychosocial Outcomes After Pediatric Liver Transplantation

Introduction Liver transplantation (LT) is a lifesaving treatment for children with end-stage liver disease (ESLD). As immunosuppressive and surgical techniques have improved over time, long-term patient and graft survival rates have continued to increase. With recent 5-year patient survival rates reported as being greater than 85% in the United States, it is important to look beyond patient and graft survival rates and biomedical (e.g., laboratory or…

Pediatric Liver Transplantation in South America: Incidence and Indications

Introduction South America currently has approximately 430,485,637 inhabitants, 16.2% of whom are under the age of 18. Brazil is the most populous South American country (213,106,000 people), followed by Colombia and Argentina (48,106,000 and 47,106,000 people, respectively). Economic resources vary widely, with most South Americans having a low or mid-range income. As an example of the disparity, the gross domestic product at purchasing power parity per…

Pediatric Liver Transplantation in Asia

Introduction The history of surgery begins with traditional medicine in India (Ayurveda since 2000 BCE) and China (Yin-Yang since 3000 BCE). Surgery began with the first attempt to control bleeding from a vessel. A compelling body of evidence indicates that Sushruta (600 BCE), a surgeon in ancient India, first performed ligation of blood vessels. Sushruta was the first surgeon of antiquity, and his monumental treatise was…

Liver Retransplantation

Incidence and Indications—Early and Late Liver Retransplantation The incidence of graft failure after pediatric liver transplantation has been decreasing with surgical and medical progress, but still reaches 10% to 15% in the first year, about 20% within 5 years, and up to 30% within 10 years after transplantation. Retransplantations are usually differentiated into early retransplantations (within 30 days) and late retransplantations. The main indications for retransplantation,…

Metabolic Conditions

An inherited metabolic disorder is an increasingly common indication for liver transplantation (LT), accounting for more than 15% of pediatric cases worldwide and up to 30% in some specialist centers. Urea cycle disorders are now the most common indication in the United States, followed by alpha-1 antitrypsin deficiency. In an individual child and family, the therapeutic decision has to incorporate the transplant center’s results and experience,…

Liver Transplantation for Liver Tumors in Children

Introduction Primary liver tumors in the pediatric population are rare entities. Based on UK children’s cancer registry data (0–16 years old), in the period 2006 to 2008, a total of 55 cases of hepatic tumors (hepatoblastoma and hepatocellular carcinoma) were registered, equating to an annual incidence of approximately 2.0 per million children. The Surveillance, Epidemiology, and End Results (SEER) data from the United States describe the…

Auxiliary Liver Transplantation for Acute Liver Failure in Children

Introduction Acute liver failure (ALF) is defined as the acute onset of severe hepatitis with loss of hepatic function in patients with no known underlying liver disease. ALF can result from various hepatic insults including viral infections, drug-induced liver injury, toxin exposures, and autoimmune diseases ( Fig. 37.1 ). Non-A, non-B hepatitis, an unexplained cause for ALF, remains the most common scenario. ALF in children is…

Liver Transplantation for Acute Liver Failure in Children

Etiology of Pediatric Acute Liver Failure The Pediatric Acute Liver Failure (PALF) Study Group, a multicenter and multinational consortium, suggested the following definition: (1) no known evidence of chronic liver disease, (2) biochemical evidence of liver injury, and (3) hepatic-based coagulopathy defined by international normalized ratio (INR) over 1.5 not corrected by vitamin K administration in the presence of hepatic encephalopathy (HE) or INR over 2.0…

Alagille Syndrome

Introduction Alagille syndrome (ALGS) is a rare multisystem disorder and one of the most frequent inherited causes of cholestatic liver disease in children. Traditionally, ALGS has been characterized by the presence of at least three of the following five principal clinical features: bile duct paucity and/or cholestasis, cardiac involvement (typically peripheral pulmonary artery stenosis), skeletal anomalies (often butterfly vertebrae), ophthalmological involvement (usually posterior embryotoxon), and/or characteristic…

Biliary Atresia

Introduction Biliary atresia is a uniquely perinatal disease that 30 years ago would have been universally fatal. With the development of the palliative Kasai portoenterostomy (Morio Kasai, 1922–2008), along with advances in pediatric liver transplantation (first performed by Thomas Starzl, 1926–2017), survival into adulthood and long-term quality of life are excellent. Biliary atresia is the main indication for liver transplantation in pediatrics. Biliary atresia is a…

Gene Therapy

Introduction In recent years, the number of gene therapy (GT) trials targeting the liver have grown significantly, particularly when it comes to gene addition strategies (delivery of cDNA for a whole gene to the target organ/cells, which then exists in addition to the host genome with the mutated gene) to rescue monogenic diseases. Although several gene delivery approaches have been tested in preclinical animal models, including…

Cell Transplantation

Introduction For numerous liver diseases of childhood, liver transplantation (LT) is a lifesaving procedure. However, it requires scarce organs, a highly experienced team to manage the surgical procedure, complications, and follow-up and lifelong immunosuppression for the recipient. Living donor organs and split livers gave us the proof of concept that a partial organ is sufficient to restore liver metabolic functions. Liver cell therapy (LCT), where cells…

Liver Assist Systems in Pediatric Liver Failure

Introduction Currently, the standard medical treatment (SMT) for pediatric liver failure incorporates optimal supportive care with hemodynamic and respiratory support, infection control, and avoidance of gastrointestinal bleeding, aiming to bridge patients to recovery or to liver transplantation (LT). LT is the only definitive treatment for liver failure. Since the 1980s, emerging literature, within the adult setting, has studied the efficacy of extracorporeal liver support system (ELSS)…

Role of Biomarkers in Monitoring Liver Allograft Function

Introduction Over the past decades, survival rates after pediatric liver transplantation have increased from about 76% to over 90% more than 10 years after transplantation (Tx). Follow-up care of liver-transplanted children includes both monitoring of graft functioning and monitoring for associated comorbidities. Monitoring the allograft is comprised of a number of features, ranging from the innate hepatic metabolic and synthetic functions to late surgical complications, such…