Silvia Buratti

Granular cell tumor of the esophagus: report of a pediatric case and literature review.

Granular cell tumors (GCT) are submucosal or subcutaneous tumors most commonly found in skin, tongue, and breast. Tumors involving the gastrointestinal tract account for 8% of all GCT (1), and only 2% are found in the esophagus (2,3). In 1926, Abrikossoff reported the first five cases of GCT of the tongue, designating them as “myoblastomas,” and in 1931 he reported the first GCT of the esophagus (4). By 1998, approximately 270 cases of GCT of the esophagus had been reported. Esophageal GCT are more common in women and black patients, and most occur in the fourth, fifth, and sixth decade (5–7). To our knowledge no cases of GCT of the esophagus have been reported in children. We report the first GCT of the esophagus in a pediatric patient and review the literature concerning clinicopathologic aspects and management of these uncommon tumors.

Drugs and the liver: advances in metabolism, toxicity, and therapeutics.

Biotransformation of drugs is one of the major functions of liver. Hepatic drug metabolism develops early in organogenesis and continues in postnatal life through puberty. Genetic and developmental studies on hepatic drug metabolism show that immaturity, polymorphisms, and altered balance of different hepatic enzymatic activities affect pharmacologic inactivation and alter the risk of toxic effects of drugs on the hepatic parenchyma. Although drug-induced liver disease is less common in children, several reports of hepatotoxicity are published every year. Furthermore, the increasing use of nonregulated remedies (eg, herbal preparations or recreational drugs) increases the risk of unpredictable and potentially severe reactions. Many significant advances in the treatment of hepatic diseases have been achieved recently. However, differences in clinical features, natural history, and response to treatment between children and adults require evaluation of new therapeutic options in focused pediatric clinical trials.

Severe Neonatal Legionella Pneumonia: Full Recovery After Extracorporeal Life Support.

Legionella pneumophila is responsible for hospital or community-acquired pneumonia. Neonatal legionellosis is associated with rapidly severe clinical course and high mortality rates. We describe a case of hospital-acquired Legionella pneumonia in a newborn with undiagnosed tracheoesophageal fistula and acute respiratory failure requiring venovenous extracorporeal membrane oxygenation support before fistula repair. Standardized multiplex polymerase chain reaction assay allowed early diagnosis. Extracorporeal life support associated with appropriate antibiotic therapy, surfactant, and steroid therapy was effective in achieving complete recovery. This is the first report of successful neonatal extracorporeal life support for respiratory failure secondary to L pneumophila.

Emergency percutaneous, bicaval double-lumen, ECMO cannulation in neonates and infants: Insights from three consecutive cases

Background Veno-venous extracorporeal membrane oxygenation (ECMO) is probably the preferable configuration to assist children with respiratory failure who do not respond to maximized conventional therapies. The single-vessel, double-lumen approach through the internal jugular vein is extremely advantageous, especially in infants, where femoral access presents limitations related to the small dimensions of the veins. In case of emergencies, ECMO might need to be started at the bedside, without the availability of fluoroscopic guidance. To our knowledge, a completely percutaneous approach has not been reported before in children younger than 1 year and weighing less than 5 kg. Methods We describe 3 cases of emergency bedside, percutaneous, bicaval double-lumen cannulation under real-time transthoracic ultrasound control in 2 neonates and 1 infant. Results In our experience, this approach proved to be safe, effective and time saving, while minimizing bleeding from the cannula insertion site. Cannulation times, from decision making to the beginning of ECMO flow, were 30, 28, 25 minutes respectively, from patient 1 to 3. We do not report any cannula-related injury to vessels and heart structures. Conclusions Our preliminary data suggest that, with the described precautions, percutaneous, echo-guided, bicaval double-lumen cannulation in neonates and infants could be effective and free from major complications. Further evaluation should be warranted in the neonatal population.

Venovenous ECMO for congenital diaphragmatic hernia: Role of ductal patency and lung recruitment

We report a case of antenatally diagnosed left-sided congenital diaphragmatic hernia, managed on venovenous extracorporeal membrane oxygenation with an hemodynamic and ventilation strategy aimed at preventing left and right ventricular dysfunction. Keeping the ductus arteriosus open with prostaglandin infusion and optimizing lung recruitment were effective in achieving hemodynamic stabilization and an ideal systemic oxygen delivery. The patient was discharged from the hospital and had normal development at 1 year of age. The combination of ductal patency and lung recruitment has not been previously reported as a strategy to stabilize congenital diaphragmatic hernia patients undergoing venovenous extracorporeal membrane oxygenation. We believe that this approach may deserve further evaluation in prospective studies.

Congenital diaphragmatic hernia repair during whole body hypothermia for neonatal hypoxic ischemic encephalopathy.

Major malformations, surgery and persistent pulmonary hypertension (PHT) have been considered contraindications to therapeutic hypothermia (TH) in newborns with hypoxic-ischemic encephalopathy (HIE). We report two patients with undiagnosed congenital diaphragmatic hernia (CDH) who developed HIE after birth. Diagnosis of moderate HIE was formulated based on clinical, laboratory and electroencephalographic criteria. The patients were treated with whole body hypothermia (33.5 °C) for 72 h. During hypothermia the patients underwent surgical repair with regular perioperative course. Ventilatory support with high-frequency oscillatory ventilation, oxygen requirements and inotropic support remained stable during hypothermia. Serial echocardiographic evaluations did not demonstrate any change in pulmonary pressure values. In our experience TH did not increase the risk of hemodynamic instability, PHT or bleeding. Hypothermia may be considered in patients with HIE and CDH or other surgical conditions with favorable prognosis.