Coats Disease in 9 Patients: A Hispanic Case Series.

BACKGROUND/AIM: To describe demographic and clinical characteristics, treatment, and visual prognosis of Coats disease in Hispanic patients. METHODS: A retrospective chart review was performed on nine patients (ten eyes) diagnosed with Coats disease in our two clinical centrers from 2004 - 2017. RESULTS: Mean age at diagnosis was 5.5 years (range 1 - 12 years) and mean follow-up time was 48 months (range 9 - 108 months). Eight patients (89%) were male and had unilateral disease and one (11%) female patient had bilateral disease. In 40% of the cases, patients were asymptomatic. Visual acuity at first presentation was worse than hand motion in 60% of the eyes. Half of the eyes (5/10 eyes, 50%) had exudative retinal detachment (≥ stage IIIA). Vascular ablation with cryotherapy combined with retinal photocoagulation was the most frequent therapeutic approach (40%). Despite anatomical success at 6 months in 100% of the treated eyes, visual outcome at 1 year of treatment was poor (worse than 20/200) in 70% of the cases. CONCLUSIONS: In our case series, patients were mostly asymptomatic on presentation, with severe stages of Coats disease. Even with anatomical success after surgical treatment in all treated cases, long-term visual prognosis remained very limited.

The subcommissural organ and the Reissner fiber: old friends revisited.

The subcommissural organ (SCO) is an ancient and conserved brain gland secreting into cerebrospinal fluid (CSF) glycoproteins that form the Reissner fiber (RF). The present investigation was designed to further investigate the dynamic of the biosynthetic process of RF glycoproteins prior and after their release into the CSF, to identify the RF proteome and N-glycome and to clarify the mechanism of assembly of RF glycoproteins. Various methodological approaches were used: biosynthetic labelling injecting 35S-cysteine and 3H-galactose into the CSF, injection of antibodies against galectin-1 into the cerebrospinal fluid, light and electron microscopical methods; isolated bovine RF was used for proteome analyses by mass spectrometry and glycome analysis by xCGE-LIF. The biosynthetic labelling study further supported that a small pool of SCO-spondin molecules rapidly enter the secretory pathways after its synthesis, while most of the SCO-spondin molecules are stored in the rough endoplasmic reticulum for hours or days before entering the secretory pathway and being released to assemble into RF. The proteomic analysis of RF revealed clusterin and galectin-1 as partners of SCO-spondin; the in vivo use of anti-galectin-1 showed that this lectin is essential for the assembly of RF. Galectin-1 is not secreted by the SCO but evidence was obtained that it would be secreted by multiciliated ependymal cells lying close to the SCO. Further, a surprising variety and complexity of glycan structures were identified in the RF N-glycome that further expands the potential functions of RF to a level not previously envisaged. A model of the macromolecular organization of Reissner fiber is proposed.

Differences in gene expression profiling and biomarkers between histological colorectal carcinoma subsets from the serrated pathway.

AIMS: To discern the differences in expression profiling of two histological subtypes of colorectal carcinoma (CRC) arising from the serrated route (serrated adenocarcinoma (SAC) and CRC showing histological and molecular features of a high level of microsatellite instability (hmMSI-H) both sharing common features (female gender, right-sided location, mucinous histology, and altered CpG methylation), but dramatically differing in terms of prognosis, development of an immune response, and treatment options. METHODS AND RESULTS: Molecular signatures of SAC and hmMSI-H were obtained by the use of transcriptomic arrays; quantitative polymerase chain reaction (qPCR) and immunohistochemistry (IHC) were used to validate differentially expressed genes. An over-representation of innate immunity functions (granulomonocytic recruitment, chemokine production, Toll-like receptor signalling, and antigen processing and presentation) was obtained from this comparison, and intercellular cell adhesion molecule-1 (ICAM1) was more highly expressed in hmMSI-H, whereas two genes [those encoding calcitonin gene-related peptide-receptor component protein and C-X-C motif chemokine ligand 14 (CXCL14)] were more highly expressed in SAC. These array results were subsequently validated by qPCR, and by IHC for CXCL14 and ICAM1. Information retrieved from public databanks confirmed our findings. CONCLUSIONS: Our findings highlight specific functions and genes that provide a better understanding of the role of the immune response in the serrated pathological route and may be of help in identifying actionable molecules.

The value of early and comprehensive diagnoses in a human fetus with hydrocephalus and progressive obliteration of the aqueduct of Sylvius: Case Report.

BACKGROUND: Mutant rodent models have highlighted the importance of the ventricular ependymal cells and the subcommissural organ (a brain gland secreting glycoproteins into the cerebrospinal fluid) in the development of fetal onset hydrocephalus. Evidence indicates that communicating and non-communicating hydrocephalus can be two sequential phases of a single pathological phenomenon triggered by ependymal disruption and/or abnormal function of the subcommissural organ. We have hypothesized that a similar phenomenon may occur in human cases with fetal onset hydrocephalus. CASE PRESENTATION: We report here on a case of human fetal communicating hydrocephalus with no central nervous system abnormalities other than stenosis of the aqueduct of Sylvius (SA) that became non-communicating hydrocephalus during the first postnatal week due to obliteration of the cerebral aqueduct. The case was followed closely by a team of basic and clinic investigators allowing an early diagnosis and prediction of the evolving pathophysiology. This information prompted neurosurgeons to perform a third ventriculostomy at postnatal day 14. The fetus was monitored by ultrasound, computerized axial tomography and magnetic resonance imaging (MRI). After birth, the follow up was by MRI, electroencephalography and neurological and neurocognitive assessments. Cerebrospinal fluid (CSF) collected at surgery showed abnormalities in the subcommissural organ proteins and the membrane proteins L1-neural cell adhesion molecule and aquaporin-4. The neurological and neurocognitive assessments at 3 and 6 years of age showed neurological impairments (epilepsy and cognitive deficits). CONCLUSIONS: (1) In a hydrocephalic fetus, a stenosed SA can become obliterated at perinatal stages. (2) In the case reported, a close follow up of a communicating hydrocephalus detected in utero allowed a prompt postnatal surgery aiming to avoid as much brain damage as possible. (3) The clinical and pathological evolution of this patient supports the possibility that the progressive stenosis of the SA initiated during the embryonic period may have resulted from ependymal disruption of the cerebral aqueduct and dysfunction of the subcommissural organ. The analysis of subcommissural organ glycoproteins present in the CSF may be a valuable diagnostic tool for the pathogenesis of congenital hydrocephalus.

Alcohol-Focused Behavioral Couple Therapy.

Alcohol Behavioral Couple Therapy (ABCT) has emerged over the last 30 years as a highly efficacious treatment for those with alcohol use disorders. This review highlights the historical and conceptual underpinnings of ABCT, as well as the specific treatment elements and structure. Proposed active ingredients, moderators, and mediators of treatment outcome are discussed. Efficacy is evaluated for reductions in identified patient drinking, improved relationship functioning, and reductions in intimate partner violence. Adaptations of ABCT for substances other than alcohol are described. Other adaptations, including brief interventions, interventions addressing PTSD and TBI along with alcohol use, and interventions deliverable via technology platforms are described. Additional cost-benefit and cost-effectiveness findings supporting the economic value of ABCT are noted. Future directions for research in this area include possible adaptations for female identified patients, nontraditional couples, LGBT partners and dyads involving nonintimate partner relationships. The development of more flexible models and enhanced dissemination strategies may improve clinical uptake and utility as well as increasing the feasibility of this treatment for integrated healthcare settings.

Assessing Treatment Integrity in Alcohol Behavioral Couple Therapy.

OBJECTIVES: Alcohol Behavioral Couple Therapy (ABCT) is an efficacious treatment for alcohol use disorders. Coding treatment integrity can shed light on the active ingredients of ABCT, but there are no published studies of treatment integrity instruments for ABCT. The present study describes the development and initial reliability of the Treatment Integrity Rating System - Couples Version (C-TIRS) for ABCT. METHODS: The C-TIRS was used to rate 284 first- and mid-treatment ABCT sessions of 188 couples in four randomized clinical trials. RESULTS: Average inter-rater reliability for distinguishing ratings between C-TIRS items was fair-to-good for quantity items (intraclass correlation [ICC] = 0.64) and poor-to-fair for quality items (ICC = 0.41). Five C-TIRS subscales were defined a priori to measure treatment components involving cognitive-behavioral therapy, spouse involvement, couple therapy, common therapeutic factors, and overall adherence to the treatment protocol and had adequate internal reliability (α = 0.74-0.89). Inter-rater reliability was fair to good on seven of ten scales but poor on three scales (ICC range = 0.17-0.72). CONCLUSIONS: The C-TIRS was designed to provide information about quantity and quality of the delivery of ABCT components; however, further refinement of the C-TIRS is warranted before it should be used in frontline practice. Clinical implications and recommendations for future research are discussed.

3-Year follow-up after Lasik: assessing the risk factors for retreatment.

The purpose of this study was to evaluate the correlation of important risk factors for LASIK retreatment and the retreatment rate. A retrospective cohort study was conducted. Records of patients who underwent LASIK between January 2011 and January 2012 at the Zambrano-Hellion Medical Center, Tec de Monterrey (México), and posteriorly underwent LASIK retreatment were identified and risk factors to receive retreatment were assessed using relative risk. Main outcomes were retreatment rate, risk factors for retreatment, and uncorrected distance visual acuity (UDVA). 482 eyes from 241 patients were available for a 36-month follow-up analysis. 68.5 % had primary myopic LASIK; 37 % were ≤ 2 diopters (D), 52 % were > 2 and < 6 D, and 11 % were ≥ 6 D of myopia. 31.5 % of the eyes had hyperopic LASIK. Retreatment was performed in 6.85 % eyes. Myopia > 6 D (RR 4.13), hyperopic refraction (RR 3.18), and age > 40 (RR 3.07) were the most important risk factors for retreatment (P = 0.004, P = 0.007, P = 0.006, respectively). UDVA was ≥ 20/40 in 92.1 % and ≥ 20/20 in 81.6 % of the retreated eyes and 82 % of the eyes within ± 0.50 D of target refraction. Increasing degrees of myopia, followed by hyperopic refraction, and age were the most important associated factors to retreatment. LASIK retreatment was safe and effective.

Absolute configuration of (13R)- and (13S)-labdane diterpenes coexisting in Ageratina jocotepecana.

Chemical investigation of the hexanes extracts of Ageratina jocotepecana afforded (-)-(5S,9S,10S,13S)-labd-7-en-15-oic acid (1), methyl (-)-(5S,9S,10S,13S)-labd-7-en-15-oate (2), (+)-(5S,8R,9R,10S,13R)-8-hydroxylabdan-15-oic acid (3), and (-)-(5S,9S,10S,13Z)-labda-7,13-dien-15-oic acid (5). The coexistence of (13R)- and (13S)-labdanes in this member of the Asteraceae family was demonstrated by vibration circular dichroism measurements of ester 2 and methyl (+)-(5S,8R,9R,10S,13R)-8-hydroxylabdan-15-oate (4) in comparison to the DFT B3LYP/DGDZVP-calculated spectra. In addition, transformation of 1 and 3 with HClO4 in MeOH yielded epimeric methyl (+)-(5S,10S,13S)-labd-8-en-15-oate (6) and methyl (+)-(5S,10S,13R)-labd-8-en-15-oate (7), respectively, confirming the presence of C-13 epimers in this plant. Diterpene 1 showed remarkable antibacterial activity against Bacillus subtilis (MIC 0.15 mg/mL) and Staphylococcus aureus (MIC 0.78 mg/mL), while diterpene 3 exhibited moderate activities against the same organisms.

Expression profiling shows differential molecular pathways and provides potential new diagnostic biomarkers for colorectal serrated adenocarcinoma.

Serrated adenocarcinoma (SAC) is a recently recognized colorectal cancer (CRC) subtype accounting for 7.5 to 8.7% of CRCs. It has been shown that SAC has a poorer prognosis and has different molecular and immunohistochemical features compared with conventional carcinoma (CC) but, to date, only one previous study has analyzed its mRNA expression profile by microarray. Using a different microarray platform, we have studied the molecular signature of 11 SACs and compared it with that of 15 matched CC with the aim of discerning the functions which characterize SAC biology and validating, at the mRNA and protein level, the most differentially expressed genes which were also tested using a validation set of 70 SACs and 70 CCs to assess their diagnostic and prognostic values. Microarray data showed a higher representation of morphogenesis-, hypoxia-, cytoskeleton- and vesicle transport-related functions and also an overexpression of fascin1 (actin-bundling protein associated with invasion) and the antiapoptotic gene hippocalcin in SAC all of which were validated both by quantitative real-time PCR (qPCR) and immunohistochemistry. Fascin1 expression was statistically associated with KRAS mutation with 88.6% sensitivity and 85.7% specificity for SAC diagnosis and the positivity of fascin1 or hippocalcin was highly suggestive of SAC diagnosis (sensitivity = 100%). Evaluation of these markers in CRCs showing histological and molecular characteristics of high-level microsatellite instability (MSI-H) also helped to distinguish SACs from MSI-H CRCs. Molecular profiling demonstrates that SAC shows activation of distinct signaling pathways and that immunohistochemical fascin1 and hippocalcin expression can be reliably used for its differentiation from other CRC subtypes.

Below the surface of a touch.

How the body and brain respond to a gentle stroke dynamically changes depending on how familiar someone is with the other person.

Prescribing Psychotropic Medications for Justice-Involved Juveniles.

Psychiatric disorders are exceedingly common in justice-involved youth. Relevant justice systems are varied, evolving, complex, and underserved. The intent of this article is to highlight the research and best practices related to managing the psychiatric disorders of justice-involved youths with a particular focus on pharmacotherapy. We review relevant features of the justice system and related case law, how prescribing for these individuals varies from both community settings and for incarcerated adults, effective assessment and medication selection, informed consent, management of nonadherence and medication diversion, and applied evidence for specific psychiatric problems common in these patients. Psychiatrists caring for justice-involved youth will benefit from tools, like this article, for training and orientation to provide timely and effective treatment services and consultation in these settings.

Summer and spring elbow rashes is a variant of polymorphous light eruption: confirmation by photoprovocation and histopathology in a series of five cases.

Background: Summer and spring eruptions on the elbows are a variant of polymorphous light eruption described on clinical and histopathological grounds; however, to our knowledge, they have not been confirmed by photobiological studies. Objective: Based on photobiological studies, this study aimed to demonstrate the involvement of ultraviolet-A (UVA) radiation in this variant of polymorphous light eruption occurring exclusively on the elbows. Methods: A series of five patients with polymorphous light eruption lesions on the elbows were included in our study. All patients underwent phototesting and photoprovocation of the skin lesions after exposure to a UVA light source [Philips UVA HPA lamp (400 W)]. All patients underwent punch biopsy and histopathological and immunohistochemical studies with anti-CD123. Results: In all the cases, UVA irradiation caused the appearance of skin lesions on the elbows with characteristic polymorphous light eruption. Histological data showed edema in the superficial dermis and a perivascular lymphocytic infiltrate compatible with polymorphous light eruption. Immunohistochemical staining for CD1-23 showed negative results. Conclusions: For the first time, photobiological photoprovocation studies demonstrated that repeated exposure to UVA radiation leads to the generation of skin lesions on the elbows, which are clinically and histologically consistent with summer and spring eruptions, confirming that elbow rash is a variant of polymorphous light eruption.

A cell junction pathology of neural stem cells leads to abnormal neurogenesis and hydrocephalus.

Most cells of the developing mammalian brain derive from the ventricular (VZ) and the subventricular (SVZ) zones. The VZ is formed by the multipotent radial glia/neural stem cells (NSCs) while the SVZ harbors the rapidly proliferative neural precursor cells (NPCs). Evidence from human and animal models indicates that the common history of hydrocephalus and brain maldevelopment starts early in embryonic life with disruption of the VZ and SVZ. We propose that a "cell junction pathology" involving adherent and gap junctions is a final common outcome of a wide range of gene mutations resulting in proteins abnormally expressed by the VZ cells undergoing disruption. Disruption of the VZ during fetal development implies the loss of NSCs whereas VZ disruption during the perinatal period implies the loss of ependyma. The process of disruption occurs in specific regions of the ventricular system and at specific stages of brain development. This explains why only certain brain structures have an abnormal development, which in turn results in a specific neurological impairment of the newborn. Disruption of the VZ of the Sylvian aqueduct (SA) leads to aqueductal stenosis and hydrocephalus, while disruption of the VZ of telencephalon impairs neurogenesis. We are currently investigating whether grafting of NSCs/neurospheres from normal rats into the CSF of hydrocephalic mutants helps to diminish/repair the outcomes of VZ disruption.

Efficacy and safety of cinacalcet for the treatment of secondary hyperparathyroidism in patients with advanced chronic kidney disease before initiation of regular dialysis.

AIM: To evaluate the compassionate use of cinacalcet for the management of secondary hyperparathyroidism in patients who are not on dialysis. METHODS: Patients with stage 4-5 chronic kidney disease (CKD) who were not on dialysis, had an intact parathyroid hormone (iPTH) level greater than 300 pg/mL, and had not responded satisfactorily to treatment with phosphate binders and vitamin D were prospectively studied. Patients received 6 months of compassionate treatment with cinacalcet, which was initiated at a dose of 30 mg/day orally and flexibly dosed thereafter based on iPTH levels. RESULTS: Twenty-six patients with a mean age±standard deviation (SD) of 58.8±16.1 years were enrolled in the study and included in the statistical analysis. The mean percentage change in iPTH levels from baseline after 6 months of treatment was -67.9±17.0%, with 92.3% (95% confidence interval (CI), 75.9-97.9) of patients showing an iPTH level within the limits recommended by Kidney Disease Outcomes Quality Initiative (K/DOQI) guidelines. The mean serum calcium concentrations had decreased significantly at the end of the study (-8.0±6.9%), while the mean serum phosphorus concentration had significantly increased (+8.3±17.0%). CONCLUSION: Our results suggest that cinacalcet may be a useful alternative for the treatment of secondary hyperparathyroidism in pre-dialysis patients who are unresponsive to other treatments. The hypocalcemia and hyperphosphatemia reported in previous studies may not occur if a moderate dose of calcimimetics is used in patients with marginal glomerular filtration rates, especially if combined with vitamin D analogues and calcium-based phosphate binders.

"I'm not one of them": barriers to help-seeking among American Indians with alcohol dependence.

The majority of people with alcohol use disorders do not seek formal treatment. Research on barriers to help-seeking have only recently focused on ethnic minority populations. The present study investigated the extent to which an adult American Indian (AI) sample experienced similar and/or unique barriers to help-seeking as have been reported in the literature. Using both qualitative and quantitative methods, 56 (54% male) AIs with lifetime alcohol dependence completed a semistructured face-to-face interview and a self-administered written survey. Interviews were tape recorded, transcribed, and coded for four major themes: personal barriers, pragmatic barriers, concerns about seeking help, and social network barriers. Quantitative data provided percentage endorsing each survey item and strength of each barrier, which were categorized according to the four major themes. In previous research, most barriers questionnaires have not queried for cultural concerns or how the specific type of help may be a mismatch from the client's perspective. Given the rapidly changing racial/ethnic demography in the United States, further research addressing cultural and spiritual concerns as well as more common barriers is indicated. (PsycINFO Database Record (c) 2012 APA, all rights reserved).

Impact of an Antimicrobial Stewardship Program on the Incidence of Carbapenem Resistant Gram-Negative Bacilli: An Interrupted Time-Series Analysis.

Carbapenem-resistant Gram-negative bacilli (CR-GNB) are a critical public health threat, and carbapenem use contributes to their spread. Antimicrobial stewardship programs (ASPs) have proven successful in reducing antimicrobial use. However, evidence on the impact of carbapenem resistance remains unclear. We evaluated the impact of a multifaceted ASP on carbapenem use and incidence of CR-GNB in a high-endemic hospital. An interrupted time-series analysis was conducted one year before and two years after starting the ASP to assess carbapenem consumption, CR-GNB incidence, death rates of sentinel events, and other variables potentially related to CR-GNB incidence. An intense reduction in carbapenem consumption occurred after starting the intervention and was sustained two years later (relative effect -83.51%; 95% CI -87.23 to -79.79). The incidence density of CR-GNB decreased by -0.915 cases per 1000 occupied bed days (95% CI -1.743 to -0.087). This effect was especially marked in CR-Klebsiella pneumoniae and CR-Escherichia coli, reversing the pre-intervention upward trend and leading to a relative reduction of -91.15% (95% CI -105.53 to -76.76) and -89.93% (95% CI -107.03 to -72.83), respectively, two years after starting the program. Death rates did not change. This ASP contributed to decreasing CR-GNB incidence through a sustained reduction in antibiotic use without increasing mortality rates.

Anaphylaxis to wheat flour-derived foodstuffs and the lipid transfer protein syndrome: a potential role of wheat lipid transfer protein Tri a 14.

BACKGROUND: Food allergy to wheat-derived foodstuffs is on the rise. Tri a 14, a wheat flour lipid transfer protein (LTP) allergen, has been described as a major allergen associated with baker's asthma and wheat food allergy. Cross-reactivity among LTP allergens leads to the so-called 'LTP syndrome'. METHODS: Eight adult patients showing anaphylaxis after ingestion of wheat-derived foodstuffs were selected. A homemade wheat extract, purified natural (n) and recombinant (r) Tri a 14, and peach fruit and Artemisia pollen LTP allergens Pru p 3 and Art v 3 were subjected to skin prick test, specific IgE determination (ELISA) and IgE immunodetection assays. RESULTS: All tests were positive in the 8 selected patients with the homemade extract. Positive skin prick test responses to nTri a 14, Pru p 3 and Art v 3 were found in 5/8, 6/8 and 4/4 patients, respectively. Specific IgE determined by ELISA assays was detected in 6 to nTri a 14 and rTri a 14, in 4 to Pru p 3 and in 3 to Art v 3 out of 8 individual sera tested, whereas all these sera showed IgE binding to nTri a 14 and Pru p 3 in immunodetection after SDS-PAGE separation. CONCLUSIONS: Tri a 14 seems to be a relevant allergen in patients with anaphylaxis after ingestion of wheat flour foodstuffs, according to in vitro and in vivo results. Clinical history of the analyzed patients, together with sensitization to peach Pru p 3 and Artemisia pollen Art v 3, suggests that 6 of them suffer from LTP syndrome.

Publisher Correction: α-SNAP is expressed in mouse ovarian granulosa cells and plays a key role in folliculogenesis and female fertility.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Assessing Consumer Behavior in the Wine Industry and Its Consequences for Wineries: A Case Study of a Spanish Company.

The widening gap between production and domestic consumption has led Spain to become the country with the largest volume of wine exports, of which it has a global market share of 20.5%. Wineries in Spain have, in recent years, undergone important transformation and modernization processes, while their customers have simultaneously acquired more power than ever before owing to both the availability of information and the technological means required to access that information. The objectives of this paper are the following. We first wish to analyze current wine consumer behavior in an attempt to discover indicators associated with exploratory behavior, i.e., the willingness to try new and innovative products for the first time. We also attempt to understand the extent of wine consumers' demands in this new digital and technological world. The second goal is to analyze the changes that have taken place in the wine industry, which have come about as a result of the evolution of consumers' expectations and demands. The customer accordingly now plays a new role in this new quick change scenario, which involves aspects such as the intensive use of technology, advances in communication, digital transformation, etc. We consider that this could affect some aspects of business and have, therefore, chosen to analyze one of the most advanced Spanish wineries in order to examine its entrepreneurial orientation, strategy and level of technological and digital transformation when linked to adaptation to this new consumer behavior. The results and conclusions obtained will allow us to apply our findings in future research, during which we intend to expand our studies to wineries from other regions and countries.

Pathogenesis of cerebral malformations in human fetuses with meningomyelocele.

BACKGROUND: Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation) may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events.The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. METHODS: Seven fetuses and 1 neonate (16-40 week gestational age, GA) with MMC and 6 fetuses with normal cerebral development (22-41 week GA) were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US). After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. RESULTS: At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21-34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37-40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. CONCLUSION: In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation and/or hydrocephalus. Since denuded areas cannot re-establish cell function, neuro-developmental consequences could induce permanent cerebral pathology.