We recommend aggressive, definitive surgery for thoracic esophageal perforations, whether diagnosed early or late. A variety of options are discussed with regard to complicated presentations.

No abstract

Peri-operative transfusion of blood or blood products is associated with increased morbidity and mortality after cardiac surgery. However, excessive hemodilution as a result of avoiding the use of homologous blood products can also lead to decreased oxygen delivery to vital end organs and dilutional coagulopathy. This is particularly challenging in pediatric cardiac surgery where there is a large discrepancy between the patient circulating blood volume and the priming volume of the cardiopulmonary bypass (CPB) circuit. Strategies to avoid the use of homologous blood products during pediatric cardiac surgery must also incorporate miniaturization of the CPB circuit and other bypass techniques in order to avoid problems with excessive hemodilution. We report a 5.9 kg male infant who underwent successful surgical correction of a ventricular septal defect without the use of homologous blood transfusion. Our strategies included the pre-operative administration of erythropoietin and iron to increase red blood cell mass, acute normovolemic hemodilution (ANH) before the institution of CPB, retrograde autologous priming (RAP), cell salvage, continuous ultrafiltration, vacuum-assisted venous drainage to minimize the circuit size and priming volume, and the use of near infrared spectroscopy (NIRS) to monitor the patient during the entire procedure. The utilization of these strategies is now standard for our entire pediatric cardiac surgical population.

Multifocal atrial tachycardia has certain electrocardiographic similarities to atrial fibrillation. The mechanism of atrial fibrillation is heterogenous but in some cases may arise from a single ectopic driver with fibrillatory conduction to the rest of the atria. This has led to the speculation that multifocal atrial tachycardia may have a similar mechanistic unifocal site that disperses through the atrium in a fibrillatory pattern. Ivabradine has been reported to be efficacious in an adult with paroxysmal atrial fibrillation as well as in children with junctional or ectopic atrial tachycardias. This is the first report of successfully using ivabradine, a novel anti-arrhythmic If blocking agent, to convert multifocal atrial tachycardia in a 5-month-old critically ill infant to a pattern indicating a single ectopic atrial focus. This allowed the patient’s single atrial focus to be ablated with return to sinus rhythm and decannulation from ventriculoarterial extracorporeal membrane oxygenation. This case suggests that multifocal atrial tachycardia may arise from a single automatic focus with downstream fibrillatory conduction to the atria.

Processing of nouns and action verbs can be differentially compromised following lesions to posterior and anterior/motor brain regions, respectively. However, little is known about how these deficits progress in the course of neurodegeneration. To address this issue, we assessed productive lexical skills in a patient with posterior cortical atrophy (PCA) at two different stages of his pathology. On both occasions, he underwent a structural brain imaging protocol and completed semantic fluency tasks requiring retrieval of animals (nouns) and actions (verbs). Imaging results were compared with those of controls via voxel-based morphometry (VBM), whereas fluency performance was compared to age-matched norms through Crawford’s t-tests. In the first assessment, the patient exhibited atrophy of more posterior regions supporting multimodal semantics (medial temporal and lingual gyri), together with a selective deficit in noun fluency. Then, by the second assessment, the patient’s atrophy had progressed mainly toward fronto-motor regions (rolandic operculum, inferior and superior frontal gyri) and subcortical motor hubs (cerebellum, thalamus), and his fluency impairments had extended to action verbs. These results offer unprecedented evidence of the specificity of the pathways related to noun and action-verb impairments in the course of neurodegeneration, highlighting the latter’s critical dependence on damage to regions supporting motor functions, as opposed to multimodal semantic processes.