Seminars in Pediatric Surgery

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1 , 2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function...

Performance of the appropriate operation is highly important to ensure that any patient with a suspected ovarian germ cell tumor receives optimal therapy that prioritizes cure while simultaneoulsy minimizing risk of short and long-term toxicities of treatment. The following critical elements of any operative procedure performed for a suspected pediatric or adolescent ovarian germ cell tumor are reviewed: 1. Complete resection of the tumor via ipsilateral oophorectomy while avoiding tumor rupture and spillage, and 2...

The 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...

Children with renal masses require surgical management to provide accurate surgical staging and skilled resection of the tumor. This document includes evidence-based recommendations for pediatric surgeons regarding the resection, staging, and proper nodal basin evaluation.

Children, adolescents and young adults with testicular germ cell tumors require appropriate surgical care to insure excellent outcomes. This article presents the most critical elements, and their basis in evidence, for surgery in this population. Specifically, the importance of inguinal radical orchiectomy for malignant tumors, partial orchiectomy for prepubertal tumors and normal serum tumor markers, and the appropriate use of post-chemotherapy retroperitoneal lymph node dissection in those with residual retroperitoneal masses...

While unfractionated heparin (UFH) remains the mainstay of anticoagulation during pediatric extracorporeal life support, direct thrombin inhibitors (DTIs) are increasingly used. In this article, we will review most recent evidence regarding utilization of both UFH and DTIs and compare their known advantages and disadvantages. We will present anticoagulation monitoring strategies during ECMO and outline the most recent Extracorporeal Life Support Organization's anticoagulation guidelines, however with the caveat that there are no true consensus recommendations for anticoagulation management in pediatric ECMO...

Extracorporeal membrane oxygenation (ECMO) is an invaluable resource in the treatment of critically ill children with cardiopulmonary failure.  To date, over 36,000 children have been placed on ECMO and the utilization of this life saving treatment continues to expand with advances in ECMO technology.  This article offers a review of pediatric ECMO including modes and sites of ECMO cannulation, indications and contraindications, and cannulation techniques.  Furthermore, it summarizes the basic principles of pediatric ECMO including circuit maintenance, nutritional support, and clinical decision making regarding weaning pediatric ECMO and decannulation...

Congenital diaphragmatic hernia (CDH) is the most common indication for ECLS in neonatal respiratory failure. The ultimate purpose of ECLS is to grant cardiopulmonary support, allowing time for operative intervention and optimization of cardiopulmonary function as the pathophysiologic processes of pulmonary hypertension, pulmonary hypoplasia, and ventricular dysfunction either improve or resolve. In CDH, ECLS plays a crucial role in the management of the most challenging patients, facilitating postnatal stabilization, allowing a ventilation strategy which minimizes barotrauma and volutrauma, and permitting treatment of and recovery from pulmonary hypertension and/or cardiac dysfunction...

Extra Corporeal Membrane Oxygenation (ECMO) has historically been reserved for refractory pulmonary and cardiac support in children and adult. Operative intervention on ECMO was traditionally contraindicated due to hemorrhagic complications exacerbated by critical illness and anticoagulation needs. With advancements in ECMO circuitry and anticoagulation strategies operative procedures during ECMO have become feasible with minimal hemorrhagic risks. Here we review anticoagulation and operative intervention considerations in the pediatric population during ECMO cannulation...

ECMO remains an important support tool in the treatment of neonates with reversible congenital cardiopulmonary diseases. There are specific circumstances that call for either venoarterial (VA) or venovenous (VV) ECMO in neonates. While limited by the infant's the size and gestational age, ECMO can confer exceptional survival rates to a number of neonates who can often develop without devastating complications. However, it remains a labor and time intensive endeavor, which may be impractical or unattainable in resource-limited environments...

Since the early use of extracorporeal life support (ECLS), new innovations and technological advancements have augmented the ability to use this technology in children and neonates. Cannulae have been re-designed to maintain structure and allow for single cannula venovenous (VV) ECLS in smaller patients. Circuit technology, including pumps and tubing, has evolved to permit smaller priming volumes and lower flow rates with fewer thrombotic or hemolytic complications. New oxygenator developments also improve efficiency of gas exchange...

Extracorporeal membrane oxygenation (ECMO) represents a lifesaving therapy utilized in in the most critically ill neonates and children with reversible cardiopulmonary failure. As a result of the severity of their critical illness these patients are among the highest risk populations for developing acute kidney injury (AKI) and disorders of fluid balance including the pathologic state of fluid overload (FO). In multiple studies AKI has been shown to occur commonly in 60-80% children treated with ECMO and is associated with adverse outcomes...

ECMO for neonatal and pediatric respiratory failure provides gas exchange to allow lung recovery from reversible pulmonary ailments. This is a comprehensive discussion on the various strategies and advances utilized by pediatric ECLS specialists today. ECMO patients require continual monitoring, serial gasses and radiographs, near-infrared spectroscopy (NIRS - to monitor oxygen delivery to regional tissue beds), and more quality ECLS directed care. As the foundation to lung recovery, good EMCO closely monitors ECLS flow rates, sweep gasses, and membrane lung function...

Necrotizing Enterocolitis (NEC) is a devastating intestinal inflammatory disease with significant morbidity and mortality. Numerous predictors have been implicated in the development of NEC, with a relatively less emphasis on maternal factors. Pregnancy drives women into a new stage of life that increases their susceptibility to biological and psychological stress. Additionally, maternal stress during pregnancy has been linked to various complications that can negatively impact both the mother and the developing fetus...

Necrotizing enterocolitis (NEC) is an infectious and inflammatory intestinal disease that is the most common surgical emergency in the premature patient population. Although the etiology of the disease is multifactorial, intestinal dysbiosis is a hallmark of this disease. Based on this, probiotics may play a therapeutic role in NEC by introducing beneficial bacteria with immunomodulating, antimicrobial, and anti-inflammatory functions into the gastrointestinal tract. Currently, there is no Food and Drug Administration (FDA)-approved probiotic for the prevention and treatment of NEC...

Necrotizing enterocolitis (NEC) is a devastating intestinal inflammatory disorder, most prevalent in premature infants, and associated with a high mortality rate that has remained unchanged in the past two decades. NEC is characterized by inflammation, ischemia, and impaired microcirculation in the intestine. Preclinical studies by our group have led to the discovery of remote ischemic conditioning (RIC) as a promising non-invasive intervention in protecting the intestine against ischemia-induced damage during early-stage NEC...

Antibiotic therapy remains a cornerstone of treatment of both medical and surgical presentations of necrotizing enterocolitis (NEC). However, guidelines regarding the administration of antibiotics for the treatment of NEC are lacking and practices vary amongst clinicians. Although the pathogenesis of NEC is unknown, there is consensus that the infant gastrointestinal microbiome contributes to the disease. The presumed connection between dysbiosis and NEC has prompted some to study whether early prophylactic enteral antibiotics can prevent NEC...

Necrotizing enterocolitis (NEC) is a devastating gastrointestinal disorder in premature infants that causes significant morbidity and mortality. Research efforts into the pathogenesis of NEC have discovered a pivotal role for the gram-negative bacterial receptor, Toll-like receptor 4 (TLR4), in its development. TLR4 is activated by dysbiotic microbes within the intestinal lumen, which leads to an exaggerated inflammatory response within the developing intestine, resulting in mucosal injury. More recently, studies have identified that the impaired intestinal motility that occurs early in NEC has a causative role in disease development, as strategies to enhance intestinal motility can reverse NEC in preclinical models...

Necrotizing enterocolitis (NEC) is a devastating gastrointestinal disease that affects premature neonates. It frequently results in significant morbidity and mortality for those affected. Years of research into the pathophysiology of NEC have revealed it to be a variable and multifactorial disease. However, there are risk factors associated with NEC including low birth weight, prematurity, intestinal immaturity, alterations in microbial colonization, and history of rapid or formula based enteral feeds (Fig...

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