Single-Staged Management of Pediatric Neuropathic Scoliosis with Intradural-Extramedullary Schwannoma and Improvement in Intraoperative Neuromonitoring: A Case Report.

CASE: A 16-year-old girl with lumbar prominence presented to our outpatient clinic complaining of sporadic back pain without paresthesia. Radiographic investigation revealed a 68° left thoracolumbar curve with the apex at L1. Preoperative magnetic resonance imaging identified a mass at T10-11, subsequently confirmed by pathology as a schwannoma. She was treated surgically with resection and posterior spinal fusion in a single-staged procedure under neuromonitoring guidance. Intraoperative improvement in motor evoked potentials after resection informed the decision to perform simultaneous deformity correction. CONCLUSION: We discuss the unusual coincidence of a schwannoma with scoliosis and our management algorithm based on operative changes in neuromonitoring.

Novel Use of Subcostal Polyethylene Bands to Manage Tumor-Related Scoliosis Requiring Serial Imaging: A Case Report.

CASE: A 16-year-old male patient with severe kyphoscoliosis, paraplegia, and neurogenic bowel/bladder caused by a juvenile pilocytic astrocytoma was treated surgically using a hybrid fusion construct with polyethylene bands after neoplasm resection. Owing to the necessity of serial postoperative magnetic resonance imaging studies to evaluate the recurrence of pathology and known effect of metal artifact from spinal instrumentation, preservation of radiographic resolution was critical. CONCLUSION: We describe the novel utility of polyethylene bands placed around the ribs as a safe and effective form of hybrid construct for reducing radiographic metal artifact in spinal deformity cases requiring serial imaging.

A Provocative Molecular Link between Mammographic Density and BRCA1-loss associated TNBC.

Triple-negative breast cancer (TNBC) is a highly aggressive form of breast cancer that has a high mortality rate and disproportionately affects young African American (AA) women who carry mutations in the BRCA1 gene. Approximately 80% of breast cancers which develop in BRCA1-mutant carriers will have TNBC and the molecular mechanism facilitating tumor development is unclear. Our earlier work suggested Ubc9 to play a critical role in BRCA1 loss mediated TNBC cell migration and metastasis. Collagen is one of the major components of the stromal extracellular matrix (ECM) network that influences tissue density. Its re-organization act as a scaffold aiding cancer cells to migrate causing metastasis. Ubc9 is known to increase the production of collagen, a key component of fibroglandular breast tissue, as well as tumorigenesis. Our work is based on the hypothesis that loss of BRCA1 in women with high breast density causes abnormal Ubc9 levels which upregulates collagen, fibronectin and inhibits SIRT1, ß-catenin expression facilitating TNBC. We tested this hypothesis by studying the expression of total collagen, fibronectin, Ubc9, SIRT1, ß-catenin in BRCA1 mutant TNBC cells and tumor sample derived from patient with dense breasts using immunofluorescence, immunohistochemistry, and collagen assay. Our results suggest for the first time that mutation or loss of BRCA1 function in women with fibrocystic breasts can lead to over expression of Ubc9, induction of collagen and; fibronectin, inhibition of SIRT1 and nuclear accumulation of ß-catenin which could contribute to TNBC development. This network will aid not only in the identification of potential mechanism-based biomarkers that could detect disease early, but also enforce preventive measures that could reduce the risk for TNBC in women with high MD thus reducing the mortality associated with these cancers to achieve health equity.

Surveillance of ventricular septal defects in Delaware.

BACKGROUND: The prevalence of ventricular septal defects (VSDs), a birth defect in which there is an opening in the wall that separates the left and right ventricles of the heart, seemed to be substantially higher in Delaware compared with the National Birth Defects Prevention Network (NBDPN). The Delaware Birth Defects Registry (BDR) noted their high prevalence of VSDs in comparison with other states. METHODS: A subset of children with a VSD born in 2007 through 2010 was identified from the complete reportable statewide defect list that the BDR creates each year. VSDs were categorized by type of VSD (muscular, perimembranous, conotruncal, or atrioventricular septal defect), by either isolated or complex, and then by spontaneously closed, surgically closed, open but clinically insignificant, lost to follow-up, fetal or neonatal death. RESULTS: The BDR team found a prevalence of VSD of 83.4 per 10,000 including fetal/neonatal deaths. Excluding fetal and neonatal deaths the prevalence was 78.7 per 10,000 live births. Excluding small muscular VSDs, the prevalence in Delaware falls to 25.7 per 10,000. CONCLUSION: The BDR team chose to include all babies with all types of VSDs. Using these criteria Delaware's prevalence of 78.7 was higher than that reported by other states (whose prevalence ranges from 1.6 to 70.0 per 10,000 live births) (National Birth Defects Prevention Network, ). Delaware's prevalence is similar to other states when small muscular VSDs are excluded. Birth Defects Research (Part A) 106:888-893, 2016. © 2016 Wiley Periodicals, Inc.