European J Pediatr Surg Rep
January 2024
Background: Congenital portosystemic shunts (CPSS) are rare congenital abnormalities causing abnormal blood flow between the portal vein and systemic circulation. This study reports on the peri-operative anticoagulation management of CPSS patients post closure, focusing on the incidence of thrombotic and bleeding complications.
Methods: This is a single-center retrospective analysis of CPSS patients who underwent surgery or endovascular intervention between 2005 and 2021.
Multi-organ transplantation involves the transplant of two or more organs from a single donor into a single recipient; in most cases, one of these organs is a kidney. Multi-organ transplantation is uncommon in pediatric transplantation but can be life-saving or significantly life-improving for children with rare diseases, including primary heart, liver, pancreas, or intestinal failure with secondary kidney failure, metabolic disorders, and genetic conditions causing multi-organ dysfunction. This manuscript reviews the current state of pediatric multi-organ transplantation that includes a kidney, with a focus on indications, evaluation, and key differences in management compared to kidney-alone transplantation.
View Article and Find Full Text PDFThe appropriate management of pediatric liver malignancies, primarily hepatoblastoma and hepatocellular carcinoma, requires an in depth understanding of contemporary preoperative risk stratification, experience with advanced hepatobiliary surgery, and a good relationship with one's local or regional liver transplant center. While chemotherapy regimens have become more effective, operative indications more well-defined, and overall survival improved, the complexity of liver surgery in small children provides ample opportunity for protocol violation, inadequate resection, and iatrogenic morbidity. These guidelines represent the distillation of contemporary literature and expert opinion as a means to provide a framework for preoperative planning and intraoperative decision-making for the pediatric surgeon.
View Article and Find Full Text PDFIntroduction: Umbilical vein catheterization (UVC) can cause portal venous thrombosis, leading to the development of extrahepatic portal venous obstruction (EHPVO) and portal hypertension (PHT). The feasibility of the Meso-Rex bypass (MRB) for the treatment of EHPVO in patients with a history of UVC has been questioned. We compared the feasibility of performing an MRB in patients with or without a history of previous UVC.
View Article and Find Full Text PDFBackground: ABO incompatible (ABOi) liver transplantation (LT) was initially associated with a higher incidence of vascular, biliary, and rejection complications and a lower survival than ABO compatible (ABOc) LT. Various protocols have been proposed to manage anti-isohemagglutinin antibodies and hyperacute rejection. We present our experience with a simplified protocol using only plasmapheresis.
View Article and Find Full Text PDFBackground: Pediatric liver surgery is a complex and challenging procedure and can be associated with major complications, including mortality. Best practices are not established. The aims of this study were to evaluate surgeons' individual and institutional practices in pediatric liver surgery and make recommendations applicable to the management of children who require liver surgery.
View Article and Find Full Text PDFBackground: Positive fluid balance (FB) is associated with poor outcomes in critically ill children but has not been studied in pediatric liver transplant (LT) recipients. Our goal is to investigate the relationship between postoperative FB and outcomes in pediatric LT recipients.
Methods: We performed a retrospective cohort study of first-time pediatric LT recipients at a quaternary care children's hospital.
Background: It is impossible to predict which patients with biliary atresia (BA) will fail after Kasai portoenterostomy (KPE). We evaluated the predictive nature of pre-KPE clinical and histological factors on transplant-free survival (TFS) and jaundice clearance. Methods: A retrospective review of patients who received a KPE at our institution (1997−2018) was performed.
View Article and Find Full Text PDFBackground: Systemic anticoagulation after pediatric liver transplantation (pLT) is believed to reduce the incidence of vascular thrombosis, but it may also cause an increase in hemorrhagic complications.
Procedure: A 5-year retrospective review of pLT done at our institution was performed (2014-2018). The occurrence of early hemorrhagic and thrombotic complications was compared when using low-dose or high-dose anticoagulation after transplant (p < .
Kidney transplantation is the treatment of choice for pediatric patients with end-stage kidney disease. Unlike adult recipients undergoing transplantation, special considerations must be taken when transplanting children based on the underlying etiology of kidney disease, previous surgical procedures, anatomical limitations and necessary technical adjustments. Additionally, the choice of donor must be measured to ensure optimal graft survival given a longer post-transplant life expectancy.
View Article and Find Full Text PDFIntroduction: Primary liver transplants (pLT) in patients with biliary atresia (BA) are infrequent, since most babies with BA undergo a prior Kasai portoenterostomy (KPE). This study compared transplant outcomes in children with BA with or without a prior KPE. We hypothesized that pLT have less morbidity and better outcomes compared to those done after a failed KPE.
View Article and Find Full Text PDFBackground & Aims: Biliary atresia (BA) is the commonest single etiology indication for liver replacement in children. As timely access to liver transplantation (LT) remains challenging for small BA children (with prolonged waiting time being associated with clinical deterioration leading to both preventable pre- and post-transplant morbidity and mortality), the care pathway of BA children in need of LT was analyzed—from diagnosis to LT—with particular attention to referral patterns, timing of referral, waiting list dynamics and need for medical assistance before LT. Methods: International multicentric retrospective study.
View Article and Find Full Text PDFAm J Transplant
May 2022
Purpose: Standardized protocols have been shown to improve outcomes in several pediatric surgical conditions. We implemented a multi-disciplinary gastroschisis practice bundle at our institution in 2013. We sought to evaluate its impact on closure type and early clinical outcomes.
View Article and Find Full Text PDFSolid organ transplantation is now an accepted therapeutic modality for children and teenagers suffering from a wide variety of complex medical conditions. Unfortunately, patients continue to die while on the organ waiting list as there remains an imbalance between the number of recipients listed for transplantation and the number of donors available. The organ allocation process continues to generate ethical questions and debates.
View Article and Find Full Text PDFNiemann-Pick type C disease is a rare autosomal recessive lysosomal disorder that leads to the accumulation of lipids in cellular organelles. Affected infants are often cholestatic with hepatosplenomegaly, developmental delay and may present in acute liver failure. Medical therapy has shown some promise in long-term studies, in patients with milder phenotypes of the disease.
View Article and Find Full Text PDFBackground: Early hepatic artery thrombosis (HAT) after liver transplantation is a serious complication that frequently results in graft loss and the need for retransplantation. Although studies have reported on various operative and endovascular treatment approaches, pharmacologic strategies for the prevention or management of HAT are not well defined. Patients with blood clotting disorders, those with a contraindication to heparin, and those who have previously developed HAT represent unique challenges in management.
View Article and Find Full Text PDFBackground: The utility of repeat serial transverse enteroplasty (reSTEP) has been questioned after patients failed to achieve enteral autonomy. We compared the outcomes after reSTEP to one lifetime STEP (oneSTEP), and tried to identify patients at risk for reSTEP failure.
Methods: All STEPs done at our institution were reviewed.