Pediatric Leigh Syndrome: Neuroimaging Features and Genetic Correlations.

The neurodiagnostic criteria of Leigh syndrome have not yet been clearly redefined based on the expanding of molecular etiologies. We aimed to analyze 20 years of clinical, genetic, and magnetic resonance studies from our Leigh syndrome cohort to provide a detailed description of central nervous system lesions in Leigh syndrome and their biological evolution in view of their genetic and clinical findings. Our study adds new neurodiagnostic insights to the current knowledge of Leigh syndrome, including association with overlapping syndromes, and the correlation of pathogenic genetic variants with neuroimaging phenotypes. ANN NEUROL 2020;88:218-232.

Quantitative contrast-enhanced ultrasound of the brain on twin fetal lambs maintained by the extrauterine environment for neonatal development (EXTEND): initial experience.

BACKGROUND: With the development of an artificial environment to support the extremely premature infant, advanced imaging techniques tested in this extrauterine system might be beneficial to evaluate the fetal brain. OBJECTIVE: We evaluated the feasibility of (a) performing contrast-enhanced ultrasound (CEUS) and (b) quantifying normal and decreased brain perfusion in fetal lambs maintained on the extrauterine environment for neonatal development (EXTEND) system. MATERIALS AND METHODS: Twin premature fetal lambs (102 days of gestational age) were transferred to the EXTEND system. Twin B was subjected to sub-physiological flows (152 mL/kg/min) and oxygen delivery (15.9 mL/kg/min), while Twin A was maintained at physiological levels. We administered Lumason contrast agent into the oxygenator circuit and performed serial CEUS examinations. We quantified perfusion parameters and generated parametric maps. We also recorded hemodynamic parameters, serum blood analysis, and measurements across the oxygenator. Postmortem MRIs were performed. RESULTS: No significant changes in hemodynamic variables were attributable to CEUS examinations. On gray-scale images, Twin B demonstrated ventriculomegaly and progressive parenchymal volume loss culminating in hydranencephaly. By CEUS, Twin B demonstrated decreased peak enhancement and decreased overall parenchymal perfusion when compared to Twin A by perfusion parameters and parametric maps. Changes in perfusion parameters were detected immediately following blood transfusion. Postmortem MRI confirmed ultrasonographic findings in Twin B. CONCLUSION: In this preliminary experience, we show that CEUS of the brain is feasible in fetal lambs maintained on the EXTEND system and that changes in perfusion can be quantified, which is promising for the application of CEUS in this extrauterine system supporting the premature infant.

The Peritoneum: Beyond the Tissue - A Review.

Background: Despite its complexity, the peritoneum is usually underestimated in classical medical texts simply as the surrounding tissue (serous membrane) of the gut. Novel findings on physiology and morphology of the peritoneum and mesothelial cell exist but they are usually focused or limited to Continuous Ambulatory Peritoneal Dialysis research and practice. This review aims to expose, describe and analyze the most recent evidence on the peritoneum's morphology, embryology and physiology. Materials and Methods: A literature review was performed on Pubmed and MEDLINE. With no limit of publication date, original papers and literature reviews about the peritoneum, the peritoneal cavity, peritoneal fluid, and mesothelial cells were included (n = 72). Results: Peritoneum develops in close relationship to the gut from an early period in embryogenesis. Analyzing together the development of the primitive gut and the surrounding mesothelium helps understanding that the peritoneal cavity, the mesenteries and other structures can be considered parts of the peritoneum. However, some authors consider that structures like the mesenteries are different to the peritoneum. The mesothelial cell has a complex ultrastructural organization with intercellular junctions and apical microvilli. This complexity is further proven by the large array of functions like selective fluid and cell transport; physiological protective barrier; immune induction, modulation, and inhibition; tissue repair and scarring; preventing adhesion and tumoral dissemination; cellular migration; and the epithelial-mesenchymal transition capacity. Conclusion: Recent evidence on the anatomy, histology, and physiology of the peritoneum, shows that this structure is more complex than a simple serous membrane. These results call for a new conceptualization of peritoneum, and highlight the need of adequate research for identifying clinical relevance of this knowledge.

Low values of central venous oxygen saturation (ScvO2) during surgery and anastomotic leak of abdominal trauma patients.

BACKGROUND: There is a well known relationship between hypoperfusion and postoperative complications like anastomotic leak. No studies have been done addressing this relationship in the context of abdominal trauma surgery. Central venous oxygen saturation is an important hypoperfusion marker of potential use in abdominal trauma surgery for identifying the risk of anastomotic leak development. The purpose of this study was to identify the relationship between low values of central venous oxygen saturation and anastomotic leak of gastrointestinal sutures in the postoperative period in abdominal trauma surgery. METHODS: A cross-sectional prospective study was performed. Patients over 14 years old who required surgical gastrointestinal repair secondary to abdominal trauma were included. Anastomotic leak diagnosis was confirmed through clinical manifestations and diagnostic images or secondary surgery when needed. Central venous oxygen blood saturation was measured at the beginning of surgery through a central catheter. Demographic data, trauma mechanism, anatomic site of trauma, hemoglobin levels, abdominal trauma index, and comorbidities were assessed as secondary variables. RESULTS: Patients who developed anastomotic leak showed lower mean central venous oxygen saturation levels (60.0% ± 2.94%) than those who did not (69.89% ± 7.21%) (p = 0.010). CONCLUSIONS: Central venous oxygen saturation <65% was associated with the development of gastrointestinal leak during postoperative time of patients who underwent surgery secondary to abdominal trauma.