INTRACAVAL LIVER WITH CARDIAC EXTENSION. A NEW DEVELOPMENTAL ANOMALY?

Inferior vena cava (IVC) obstruction is uncommon in children. We report a patient with liver within a IVC extending to the right atrium who underwent successful surgical resection. A 12-year-old boy with an Arnold Chiari malformation was admitted for seizures. Premature ventricular contractions prompted an echocardiogram. This revealed a pedunculated mass in the right atrium and an IVC producing turbulent flow. He underwent a mass excision that was continuous with the liver. Histology demonstrated normal liver parenchyma. Based on the embryologic intimacy between the caudate lobe and the IVC, we postulate that the ectopic hepatic nodule was due to aberrant migration of hepatocytes into the IVC during embryogenesis

Twin Reversed Arterial Perfusion Sequence (TRAPS): An Illustrative Series of 13 Cases

Twin reversed-arterial-perfusion sequence (TRAPS) is a rare and severe complication of monochorionic twin pregnancies. It usually occurs in the setting of monochorionic placentation, when the heart of a normal appearing twin serves as the pump for one or more dysmorphic twins whose head, thoracic organs, and upper extremities do not fully develop or do not develop at all and thus lack cardiac activity. Anomalous vascular placental architecture causes a shift in arterial flow towards the acardiac twin(s). The exact physiopathologic mechanisms that lead to this devastating phenomenon are not well known. We reviewed the maternal history and the surgical pathology reports of the fetuses and placentas of 13 different cases of TRAPS that were collected in a 23-year study period at a single institution. Herein we summarize the characteristic findings and illustrate specific mechanical feto-placental circulation issues that appear to be instrumental in the development of TRAPS

Differential Changes in TGF-β/BMP Signaling Pathway in the Right Ventricular Myocardium of Newborns With Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left ventricle (LV) and increased biomechanical stress on the right ventricle (RV) from single ventricle physiology. Despite the clinical significance, the signaling pathways active during RV remodeling and disease progression are not known. To address this, we examined differential changes in expression of genes associated with transforming growth factor-β (TGF-β)/bone morphogenetic protein (BMP) signaling in RV tissue isolated from HLHS patients relative to RV and LV tissue from control subjects. Quantitative real-time polymerase chain reaction was used to detect changes in expression of 84 genes involved in TGF-β/BMP-mediated cardiac development, cell growth, and differentiation in RV tissue collected from 6 neonates with HLHS undergoing stage 1 Norwood procedure (age, 1-7 days; mean, 4 days) and RV and LV tissue obtained from 5 infants with noncardiac pathology (age range, 1-135 days: mean, 85 days) that served as controls. Analysis of gene expression profiles between control-LV and control-RV revealed significant depression of TGF-β/BMP signaling in RV compared with LV. Of the 84 genes analyzed, 38 were differentially expressed between HLHS-RV and control-RV, whereas only 22 compared with control-LV. Significant changes were observed in: tissue remodeling genes including Activin receptor type IIA ( ACVR2A) (+2.13) and Activin receptor-like kinase 1 ( ACVRL1) (+2.22); and cell survival, growth, and differentiation genes including CDC25A (+2.18), p21 (−3.64), p15 (+2.15), BMP5 (+4.58), BMP3 (+2.16), GDF3 (+8.59), NODAL (+2.32), and BMP binding endothelial regulator (BMPER) (+4.58). The most significant changes common to HLHS-RV versus control-RV and control-LV sample groups is observed for Anti müllerian hormone receptor 2 ( AMHR2) (+18.79 control-RV, +3.38 control-LV), and the BMP antagonist Inhibin alpha ( INHA) (+11.47 control-RV, +5.73 control-LV). Although this descriptive study does not allow cause-effect inferences, our results suggest changes in cardiac development pathways and upregulation of genes associated with cell growth and differentiation in the neonatal RV of children with HLHS. These molecular profiles are more closely related to those observed in the normal LV rather than normal RV at similar maturational age. This work provides the basis for future mechanistic studies to elucidate the molecular mechanisms regulating RV remodeling in HLHS

Thrombotic cutaneous gangrene with autoamputation of the penis: a rare extracolonic manifestation of ulcerative colitis in a child

We present a pediatric patient with ulcerative colitis who developed thrombotic cutaneous gangrene involving skin of the lower chest, abdomen, back, bilateral buttocks, bilateral thighs, perineum, and genitalia, ultimately resulting in autoamputation of the glans penis. After an extensive review of the literature, we describe the diagnosis and management of this devastating condition

Bridging the Gaps Between the Histopathological and Demographic Risk Factors of Preterm Birth in a Unique Miami Inner-City Population

We aim to identify the link between placental histological findings and obstetric reports to determine possible risk factors of spontaneous preterm birth (SPTB). We prospectively ascertained birth records and outcomes from all deliveries in our hospital in 1 year. Records were used to determine and stratify for either full-term or preterm [spontaneous or indicated (I)] deliveries. We analyzed for risk factor association using χ(2) tests and common odds ratio estimates (SPSS v21.0). Our cohort totaled 6088 deliveries: 236 IPTB, 43 SPTB, and 5809 term births. Largely Hispanic, we determined race, parity, prenatal care access, preeclampsia, gestational diabetes, and BMI to be highly associated with SPTB (p < 0.01). Histologically, placentas of women with SPTB were twice as likely to have chronic villitis. We found that chronic villitis is associated with SPTB. Results of this study can be used in increasing the understanding of SPTB