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Background: The guide for monitoring and treatment of congenital hepatic hemangiomas (CHH) will depend on the subtype and the postnatal clinical behavior. Our aim is to present a series of CHH and characterize its clinical, histologic and genetic correlation, compared to cutaneous congenital hemangiomas (CCH)., Material and Methods: A retrospective review of CHH patients diagnosed between 1991 and 2018 was performed. Clinical, morphological and histological data were analyzed and deep high-throughput sequencing was performed., Main Results: Sixteen patients with CHH were included. Five patients were followed up with serial ultrasounds while pharmacological treatment (corticosteroids and propranolol) was decided in five. Surgical resection was performed in five owing to hemorrhage and suspicion of malignancy, and the last patient underwent embolization. Histologic analysis was available in 7 patients and confirmed CHH, showing two different histological patterns that could be associated with the presence of somatic pathogenic variants in GNAQ and/or PIK3CA detected in the genetic testing. Review of 7 samples of CCH revealed some histologic differences compared to CHH., Conclusion: CHH resemble its cutaneous homonym with similar clinical behavior. Histologic analysis can differentiate two subgroups while genetic testing can confirm mutations in GNAQ and in PIK3CA in a subset of CHH., Type of Study: Treatment study., Level of Evidence: IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study is to describe the malformations of cortical development detected in a model of cerebrospinal fluid (CSF) leakage and the influence of surgical closure technique on developmental outcome., Methods: Using a surgically induced model of myelomeningocele (MMC) in sheep, we studied the effects of different repair methods upon the development of hydrocephalus, the presence of the Arnold-Chiari II (AC-II) hindbrain malformation, and cerebral cortex developmental anomalies using gross and histologic (hematoxylin and eosin and Nissl staining) study techniques., Results: A malformed cerebral cortex, including 2 anomalous cortical folding patterns, and lower brain weights were observed in the untreated animals. Hydrocephalus and AC-II malformations were also found in this group. These malformations were mostly prevented with prenatal 2-layer closure., Conclusions: Cerebral cortical malformations and hydrocephalus, in addition to the AC-II hindbrain malformation, are disorders caused by fetal CSF leakage. These malformations were prevented with the technique of MMC closure currently used in humans. Both observations magnify the importance of the second hit associated with chronic CSF leakage, in addition to the primary defect causing the MMC, in the development of the malformation complex., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/aim: GLUT1 is an erythrocyte-type glucose transporter protein typically expressed in cutaneous proliferating hemangioma. Immunostaining for GLUT1 is becoming valuable for predicting the outcome of vascular skin tumors. Liver vascular tumors (LVTs) are a serious challenge for pediatric surgeons because of their often severe and sometimes unpredictable clinical course. To improve therapeutic strategies, we designed this study in which we tested in pathology specimens of LVT the hypothesis that GLUT1 expression could be useful to understand and classify LVT., Material and Methods: In the last 10 years, we treated 20 children with LVT. Pathology specimens from biopsy, excision, or autopsy were available in 11 of them. The paraffin sections were immunostained for GLUT1 and also for Ki-67 to assess the proportion of proliferating cells. Patients were divided into 2 groups: GLUT1-positive (n = 4) and GLUT1-negative (n = 7) that were compared for age at diagnosis, survival, and proportion of proliferating cells. Nonparametric and chi 2 tests were used for statistical analysis as appropriate., Results: Mean age at diagnosis was higher in GLUT1-positive group, although not statistically significant in comparison with GLUT1-negative (308 +/- 515 vs 70 +/- 51 days, respectively). Three of 4 children in GLUT1-positive group died versus 1 of 7 in the GLUT1-negative group (not significant). All GLUT1-positive tumors were multicentric hemangiomata without central necrosis and only 1 with large vessels. Among GLUT1-negative tumors, 5 were solitary masses and 2 were multicentric (the value of the last 2 specimens was uncertain), 2 had central necrosis, and 2 had large vessels. Proliferation index was 18% +/- 1.42% and 1.42% +/- 0.97%, respectively, in each group ( P < .05)., Conclusions: GLUT1-positive tumors have significantly higher proliferation rates than negative ones. Mortality tended to be higher in children with GLUT1-positive tumors. Positive GLUT1 immunostaining is likely specific for proliferating hemangioma, and it predicts the typical course of proliferation followed by involution.

Background/purpose: Blood drainage of the graft into the recipient portal vein reestablishes the physiological venous outflow after small bowel transplantation (SBT). However, although this approach is likely beneficial for the host, it may be technically more demanding making portocaval venous drainage the preferred arrangement during human SBT. The aim of this study was to examine in a syngeneic model of SBT the possible benefits of portoportal anastomosis (PPA) vs portocaval anastomosis (PCA) in terms of body and organ weights and bacterial translocation., Methods: Syngeneic SBT was carried out in 25 Brown-Norway male rats weighing 249 +/- 17.5 g using either PPA (n = 13) or PCA (n = 12). Half the animals in each group were killed, respectively, on postoperative day 2 or 7. Liver, spleen, and lungs were weighed and under sterile conditions the regional lymph nodes were excised. The nodes and venous samples from the cava and portal veins were cultured for aerobes and anaerobes. Bacterial components were detected in blood by polymerase chain reaction. The findings in both groups were compared by chi2 or Mann-Whitney U tests., Results: Mean postoperative body weight change was -3.6% +/- 1.5% in PPA and -6.0% +/- 1.2% in PCA animals (ns) on day 2 and -6.5% +/- 2.6% and -8.0% +/- 5.0% (ns) on day 7. Liver, spleen, and lung weights were not significantly different between both groups on either end point. Gram-negative enteric bacteria were found in 3 of 7 PCA animals and 2 of 6 PPA animals at day 2 (ns) and in 1 of 6 and 4 of 6 on day 7 (ns). Aerobic gram-positive bacteria were found in 1 of 7 and 1 of 6 (ns), 3 of 6 and 3 of 6 (ns), respectively, in the 4 groups. Most positive cultures corresponded to portal blood and lymph node samples. There were no anaerobic growths., Conclusions: -No body or organ weight change suggesting significant functional advantages of one technical alternative over the other could be demonstrated. -Bacterial translocation in the absence of rejection was frequent after SBT independently of the variety of venous outflow used. No difference in bacterial translocation between both anastomosis could be demonstrated. -Orthotopic venous drainage did not seem to be advantageous in the present experimental setting.