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Role of Radiology and Interventional Radiology After Pediatric Liver Transplantation

Diagnosis of Complications: Role of Imaging Modalities Systematic Follow-up Ultrasonography with Doppler (US Doppler) is the best imaging modality for systematic follow-up of the liver allograft and for the detection and diagnosis of complications. Grayscale mode allows for the analysis of graft parenchyma, vessels, and bile ducts and the detection of fluid collections ( Fig. 29.1 ). Color and pulsed Doppler modes allow the precise analysis…

Post-transplant Lymphoproliferative Disease

Introduction Post-transplant lymphoproliferative diseases (PTLDs) are the most frequent malignant disorders after solid-organ transplantation in children. Although morphologically often indistinguishable from de novo lymphomatous diseases, per definition, every lymphoproliferation arising after transplantation is considered a PTLD. Because pathogenesis in the context of immunosuppression, viral infection, and treatment differ from lymphomas in immunocompetent patients, PTLD needs to be considered as its own disease entity. Thus the World…

Immunological and Other Late Complications

There are several late medical complications that may jeopardize long-term survival after pediatric liver transplantation (LT). A significant number of recipients develop subclinical but progressive graft fibrosis, as increasingly evidenced in protocol liver biopsies performed 1 to 10 years post-LT, presumably related to chronic autoimmune/alloimmune injury of the allograft, possibly as a result of under-immunosuppression. On the other hand, chronic over-immunosuppression exposes the patient to the…

Recurrence of Disease After Liver Transplantation

The recurrence of underlying disease is relatively low in cases of pediatric liver transplantation (LT) compared with adults. The underlying liver disease does not recur in biliary atresia or in most forms of metabolic liver disease. The most important indications that can be complicated by disease recurrence after transplantation at pediatric age are the following: 1. Autoimmune liver disease 2. Familial cholestatic syndromes, particularly bile salt…

Surgical Complications

Introduction Long-term outcomes after pediatric liver transplantation are nowadays characterized by their excellence. Remarkably, when compared with older age group results, graft and patient midterm survival rates are higher, with a remarkably low loss in the longer term (for both the grafts and the patients). The survival curves are also close to a stable line after the second year. This relates to the fact that most…

Role of Histology Following Pediatric Liver Transplantation

Introduction This chapter will briefly consider the examination of the recipient’s liver, removed at transplantation, and then focus on the role of the pathologist in the interpretation of the liver allograft biopsy. Complications following liver transplantation have been described in other chapters of the text, as summarized in Fig. 24.1 . Biopsy manifestations of these complications are comprehensively addressed elsewhere and will be described here with…

Hematologic Complications After Liver Transplant

Introduction Solid organ transplants can be associated with a variety of hematologic abnormalities ranging from minor to life-threatening. When they occur, they may lead to prolonged admissions, reduced physical capacity, infectious complications, risk of bleeding, and overall impact the child’s return to normal life. Some of the common and earliest hematologic complications after liver transplantation are single- or multilineage cytopenias of different etiologies, including infectious, inflammatory,…

General Aspects of Early Management

Introduction Liver transplantation (LT) should not be considered a procedure that resolves the problem of a native liver at once but rather a “long-term care plan.” Indeed, the surgical procedure is only the first step of a complex management program that is almost invariably characterized by complications of various types and severity. Post-transplant complications are commonly classified as “surgical” or “medical,” but there is commonly no…

Biliary Complications in Pediatric Liver Transplantation

Introduction Pediatric liver transplantation (LT) has expanded since 1980 because of the broad utilization of segmental grafts including split, living donor, and, occasionally, also reduced grafts. This currently allows achievement of a timely transplant in virtually all the children on the waiting list. As a drawback, the pediatric population is exposed to the specific morbidity related to these kinds of grafts and particularly to biliary complications…

Early Vascular Complications

This chapter treats early vascular complications after pediatric liver transplantation (PLT), which herein will be defined as complications that occur during the first 4 weeks after liver transplantation (LT). General Considerations PLT is characterized by coagulation disorders. Preoperatively, routine coagulation tests fail to detect the subtle interactions between a developing coagulation balance, the various etiologies of chronic liver disease, and acute liver failure. Post-operatively, hemostasis is…

Anti-infection Strategies

Anti-infection Strategies Infections are the cause of significant morbidity and mortality after transplantation, both in the immediate post-operative period and beyond ( Fig. 19.1 ). Anti-infection strategies include preventive measures, such as antimicrobial prophylaxis or vaccination (see Chapter 6 ), and also effective therapy. However, pharmacokinetics, interactions, lack of evidence-based data in this population (transplant and/or pediatric), and availability of pediatric-compatible formulations make treatment options often…

Anti-rejection Strategies

Introduction Although the liver is known to be a somewhat immunotolerant organ, pediatric liver transplantation (LT) would not have reached current success without efficient immunosuppressive drugs. Standard protocols include calcineurin inhibitors (CNIs), most often tacrolimus (TAC) and sometimes cyclosporine A (CsA). Additional induction with anti-interleukin 2 receptor antibodies (anti-IL2r) or a short corticosteroids (CS) course further decrease the risk of rejection. Specific situations may require immunosuppression…

Types of Rejection

Immunological Mechanisms of Rejection Transplantation of an allograft induces a formidable immunological response by the recipient designed to destroy the foreign organ efficiently and quickly. Successfully controlling this complex multifaceted cascade is the critical element required for graft survival. It is beyond the scope of this chapter to explore fully our evolving understanding of the immunological mechanisms of rejection. The interested reader is referred to excellent…

Post-Operative Care After Liver Transplantation

Introduction Liver transplantation (LT) is a major surgical procedure, with recipients presenting with end-stage liver disease and the complications thereof. It follows that post-operative care is complex and requires continuous anticipation of potential co-morbidities and complications. Although the immediate post-operative period is characterized by a multi-organ dysfunction syndrome, standard supportive care usually leads to a progressive improvement within the first few days post-operatively. In addition to…

Anesthetic Aspects

Introduction Anesthesia for pediatric liver transplantation is complex, diverse, and challenging. The indications for transplantation and the physiology of children vary from the adult population, making the pediatric service unique. The role of the anesthetist must begin with the preassessment of potential candidates, optimization while on the waiting list, and, finally, anesthesia for the transplant procedure. Knowledge of the plethora of liver conditions and the multifaceted…

The Transplant Procedure: Surgical Aspects and Techniques

Introduction Pediatric liver transplantation (PLT) sits at the pinnacle of pediatric surgical endeavors. In the early years, long-term survival rates after PLT were 11% to 39% and, since then, have improved to up to 90% with long-term graft survival rates of more than 80%. The need for PLT is estimated to be one to two per million population. With continuing improvements in surgical and interventional techniques…

Combined Liver and Small Bowel Transplantation

Introduction Intestinal transplantation (ITx) was first attempted in the 1970s in children with intestinal failure (IF) for whom intravenous nutrition was not available. The early efforts did not meet with long-term success because of lack of adequate immunosuppression. The first successful adult combined liver and small bowel transplant was performed in Canada in 1988; the first successful isolated ITx was in 1989 in Paris in a…

Combined Transplantation Liver-Heart, Lung, and/or Pancreas

Liver-Heart Indication Combined heart and liver transplantation (CHLT) is still an evolving procedure, representing only 0.3% of all cardiac transplantation in a Unified Network for Organ Sharing (UNOS) database study spanning 28 years. The most common indications are cirrhosis secondary to cardiac disease, usually restrictive cardiomyopathy (most commonly from amyloidosis), and either palliated univentricular congenital heart disease or cardiomyopathy. As such, most patients are in the…

Combined Liver and Kidney Transplantation

Whereas isolated liver and kidney transplantations (LTs and KTs, respectively) have become routine procedures for isolated organ failure, combined liver and kidney transplantation (CLKT) is relatively rarely performed worldwide, and the number of centers undertaking this procedure is limited. In 2008 and 2012, there have been consensus conferences on CLKT to establish guidelines for evaluation, listing, and transplantation of adult patients with combined liver disease and…

Liver and Hematopoietic Stem Cell Transplantation

Liver and hematopoietic stem cell transplantation (HSCT) have established their places in the ever-increasing number of individual indications in their respective fields of modern medicine. However, there is a group of overlapping conditions in immunology, hematology, metabolic medicine, and oncology where a combination of these two procedures could occasionally be considered as a potential cure. Some of these combined clinical scenarios requiring both procedures are urgent,…