Antenatal corticosteroids and outcomes in gastroschisis: A multicenter retrospective cohort study.

OBJECTIVE: In gastroschisis, there is evidence to suggest that gut dysfunction develops secondary to bowel inflammation; we aimed to evaluate the effect of maternal antenatal corticosteroids administered for obstetric reasons on time to full enteral feeds in a multicenter cohort study of gastroschisis infants. METHODS: A three center, retrospective cohort study (1992-2013) with linked fetal/neonatal gastroschisis data was conducted. The primary outcome measure was time to full enteral feeds (a surrogate measure for bowel function) and secondary outcome measure was length of hospital stay. Analysis included Mann-Whitney and Cox regression. RESULTS: Of 500 patients included in the study, 69 (GA at birth 34 [25-38] weeks) received antenatal corticosteroids and 431 (GA at birth 37 [31-41] weeks) did not. Antenatal corticosteroids had no effect on the rate of reaching full feeds (Hazard ratio HR 1.0 [95% CI: 0.8-1.4]). However, complex gastroschisis (HR 0.3 [95% CI: 0.2-0.4]) was associated with an increased time to reach full feeds and later GA at birth (HR 1.1 per week increase in GA [95% CI: 1.1-1.2]) was associated with a decreased time to reach full feeds. CONCLUSION: Maternal antenatal corticosteroids use, under current antenatal steroid protocols, in gastroschisis is not associated with an improvement in neonatal outcomes such as time to full enteral feeds or length of hospital stay.

Emergence of rich-club topology and coordinated dynamics in development of hippocampal functional networks in vitro.

Recent studies demonstrated that the anatomical network of the human brain shows a "rich-club" organization. This complex topological feature implies that highly connected regions, hubs of the large-scale brain network, are more densely interconnected with each other than expected by chance. Rich-club nodes were traversed by a majority of short paths between peripheral regions, underlining their potential importance for efficient global exchange of information between functionally specialized areas of the brain. Network hubs have also been described at the microscale of brain connectivity (so-called "hub neurons"). Their role in shaping synchronous dynamics and forming microcircuit wiring during development, however, is not yet fully understood. The present study aimed to investigate the role of hubs during network development, using multi-electrode arrays and functional connectivity analysis during spontaneous multi-unit activity (MUA) of dissociated primary mouse hippocampal neurons. Over the first 4 weeks in vitro, functional connectivity significantly increased in strength, density, and size, with mature networks demonstrating a robust modular and small-world topology. As expected by a "rich-get-richer" growth rule of network evolution, MUA graphs were found to form rich-clubs at an early stage in development (14 DIV). Later on, rich-club nodes were a consistent topological feature of MUA graphs, demonstrating high nodal strength, efficiency, and centrality. Rich-club nodes were also found to be crucial for MUA dynamics. They often served as broker of spontaneous activity flow, confirming that hub nodes and rich-clubs may play an important role in coordinating functional dynamics at the microcircuit level.

A comparison of computational methods for detecting bursts in neuronal spike trains and their application to human stem cell-derived neuronal networks.

Accurate identification of bursting activity is an essential element in the characterization of neuronal network activity. Despite this, no one technique for identifying bursts in spike trains has been widely adopted. Instead, many methods have been developed for the analysis of bursting activity, often on an ad hoc basis. Here we provide an unbiased assessment of the effectiveness of eight of these methods at detecting bursts in a range of spike trains. We suggest a list of features that an ideal burst detection technique should possess and use synthetic data to assess each method in regard to these properties. We further employ each of the methods to reanalyze microelectrode array (MEA) recordings from mouse retinal ganglion cells and examine their coherence with bursts detected by a human observer. We show that several common burst detection techniques perform poorly at analyzing spike trains with a variety of properties. We identify four promising burst detection techniques, which are then applied to MEA recordings of networks of human induced pluripotent stem cell-derived neurons and used to describe the ontogeny of bursting activity in these networks over several months of development. We conclude that no current method can provide "perfect" burst detection results across a range of spike trains; however, two burst detection techniques, the MaxInterval and logISI methods, outperform compared with others. We provide recommendations for the robust analysis of bursting activity in experimental recordings using current techniques.

Transanal irrigation for intractable faecal incontinence and constipation: outcomes, quality of life and predicting non-adopters.

PURPOSE: Intractable faecal incontinence (FI) and constipation is a challenging condition to manage in children. Transanal irrigation (TAI) is a non-operative treatment option. This study presents our experience with TAI with the aim of finding predictive factors of non-compliance. METHODS: This is an outcome and quality of life (QoL) study of a prospectively maintained database of patients < 17 years old commenced on TAI for intractable FI/constipation between 2008 and 2014. Outcome measures were: (1) compliance-classified as non-adopter (use of TAI stopped within 1 month after commencement) or adopter; (2) functional outcome-classified as responder (totally continent or occasional soiling) or non-responder; (3) Rintala score; and (4) QoL score (PedsQL™ 4.0 Generic Core Scale). Analysis to determine predictive factors was also performed. RESULTS: 42 patients were started on TAI [74% male, median age of commencement was 7 (3-16) years]. Underlying diagnoses were: idiopathic constipation (62%), anorectal malformation (26%), Hirschsprung disease (5%), spina bifida (5%) and gastroschisis (2%). Median follow-up period was 14 (3-78) months. 24% were non-adopters. 84% of the adopters responded to treatment. Rintala scores (mean ± SD) pre- and post-TAI were 6.7 ± 3.5 and 11.2 ± 4.8, respectively (P < 0.001). QoL scores pre- and post-TAI were 55.6 ± 24.1 and 65.5 ± 23.7, respectively (P < 0.001). Median age at which TAI was commenced in the non-adopter and adopter group were 6 (IQR 4.5-8.25) and 8 (IQR 7-12), respectively (P = 0.008). CONCLUSION: TAI is a safe and effective treatment for intractable constipation/FI in children. If tolerated, it can significantly improve quality of life. Age and underlying diagnosis are important factors when recommending TAI to children with intractable FI/constipation.

Knockdown of mental disorder susceptibility genes disrupts neuronal network physiology in vitro.

Schizophrenia and bipolar disorder are common diseases caused by multiple genes that disrupt brain circuits. While great progress has been made in identifying schizophrenia susceptibility genes, these studies have left two major unanswered mechanistic questions: is there a core biochemical mechanism that these genes regulate, and what are the electrophysiological consequences of the altered gene expression? Because clinical studies implicate abnormalities in neuronal networks, we developed a system for studying the neurophysiology of neuronal networks in vitro where the role of candidate disease genes can be rapidly assayed. Using this system we focused on three postsynaptic proteins DISC1, TNIK and PSD-93/DLG2 each of which is encoded by a schizophrenia susceptibility gene. We also examined the utility of this assay system in bipolar disorder (BD), which has a strong genetic overlap with schizophrenia, by examining the bipolar disorder susceptibility gene Dctn5. The global neuronal network firing behavior of primary cultures of mouse hippocampus neurons was examined on multi-electrode arrays (MEAs) and genes of interest were knocked down using RNAi interference. Measurement of multiple neural network parameters demonstrated phenotypes for these genes compared with controls. Moreover, the different genes disrupted network properties and showed distinct and overlapping effects. These data show multiple susceptibility genes for complex psychiatric disorders, regulate neural network physiology and demonstrate a new assay system with wide application.

Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes.

INTRODUCTION: Gastroesophageal reflux disease (GERD) is associated with accelerated decline in lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS) is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly investigated. This study aimed to determine the incidence of GERD in children with CF and to evaluate complications and outcomes of ARS. MATERIALS AND METHODS: A systematic literature-based search was conducted using various online databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s) was recorded. Data on postoperative complications and outcomes (including symptoms, lung function, and nutritional status) following ARS were analyzed. RESULTS: Ten articles (n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month-36 years). The overall incidence of GERD was 46% (range, 19-81%), derived from seven studies (n = 212 patients). Four publications (n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication (majority with gastrostomy placement). Major postoperative complications occurred in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range, 3 months-6 years); 59% showed symptom improvement, and pulmonary exacerbations and decline in lung function were reduced. Nutritional status mainly improved in milder CF cases. There were no deaths related to ARS. CONCLUSION: Approximately half of pediatric CF patients have GERD. Published data for children with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following ARS. However, ARS has shown to slow the deterioration of lung function in CF.

Optimal management of gastrojejunal tube in the ENFit era - Interventions that changed practice.

BACKGROUND: We experienced a high incidence of jejunal tube (JEJ) displacement in children who underwent percutaneous endoscopic transgastric jejunostomy (PEGJ), ever since the introduction of ENFit connector (2017). METHODS: Two interventions were introduced in 2018 - fixative suture to PEGJ ENFit connector, and conversion to balloon transgastric-jejunal feeding device (Balloon GJ) whenever possible. Children receiving PEGJ and Balloon GJ in 2.8 years were categorized into 3 eras: 2016 (pre-ENFit), 2017 (ENFit) and 2018 (interventional), for comparison of complications and sequelae. Kaplan-Meier survival curves with log-rank test (P < 0.05) were applied. RESULTS: 100 children underwent 323 JEJ insertions - PEGJ (n = 237), Balloon GJ (n = 86). Complications occurred in 188 JEJs (58%), more frequently with PEGJ than Balloon GJ (69% vs. 29%, P < 0.0005). PEGJ had higher complication/1000-tube-days (6 vs. 0, P < 0.0005). In 2018, complication rate reduced from 76% to 30% (P < 0.0005) owing to effectiveness of PEGJ connector suture application (P = 0.019), and increased utilization of Balloon GJ (16% to 44%, P = 0.005). Balloon GJ showed better JEJ survival (P = 0.019), less morbidity (emergency attendance, X-ray) and greater cost-effectiveness than PEGJ. CONCLUSIONS: Balloon GJ had better overall outcomes than PEGJ. Suture application to connector successfully reduced JEJ internal displacement in PEGJ; however, conversion to Balloon GJ should be strongly considered. LEVEL OF EVIDENCE: II.

Isolated bilateral simplex ureteric ectopia: Bladder capacity as an indicator of continence outcome.

INTRODUCTION: Isolated bilateral simplex ectopic ureters (BSEUs) are rare but pose a therapeutic challenge: ureteric reimplantation alone does not accomplish continence in all. Identifying the patients needing additional procedures for continence early could prevent multiple operations. OBJECTIVE: Potential preoperative indicators for postoperative continence are explored in eight BSEU girls without cloacal, anorectal, or spinal anomalies. STUDY DESIGN: With institutional approval, all patients with BSEU between 1985 and 2012 were retrospectively reviewed. Cystoscopy determined the site of ureteric ectopia (6 of 16 at the bladder neck [BN], 5 of 16 below the BN, and 5 of 16 in the distal urethra). Bladders were assessed by a combination of ultrasound, urodynamics, micturating cystourethrogram, cystoscopic, and intraoperative observations. Expected bladder capacity for age (EBCA) was calculated by 30 ml + (30 ml × age in years) or 38 ml + (2.5 ml × age in months) for children greater or less than 2 years, respectively. Continence outcomes were appraised at a minimum of 4 years. The small number of patients precludes credible statistical analysis and therefore raw data are presented. RESULTS: Patients underwent cross-trigonal ureteric reimplantation at 1-5.5 years, in five without BN surgery and in three with a Young-Dees-Leadbetter BN tightening. Of those without BN surgery at reimplantation, four achieved satisfactory continence for their age, but one has had multiple procedures culminating in BN closure, ileocystoplasty, and Mitrofanoff. Among the BN-tightening group, one was in nappies at 4 years, one had residual stress incontinence after two further BN injections, and one proceeded to artificial urinary sphincter after two BN injections. Five patients had significant renal impairment. DISCUSSION: Patients with satisfactory continence after reimplantation alone and those needing further procedures tended to differ in their preoperative observations of bladder capacity and apparent BN competence. This study suggests preoperative observations of an empty bladder on serial ultrasound and/or a wide-open BN with small or even moderate bladder capacity at cystoscopy to indicate the need for BN surgery. In contrast, children with bladder filling to at least 30% of expected bladder capacity for age on preoperative ultrasound or apposition of the BN at cystoscopy may achieve satisfactory continence after ureteric reimplantation alone. Bladder capacity as an indicator of BN competence can also be correlated to continence outcomes in previously published series. Polyuria associated with renal impairment can exacerbate the challenge for continence. CONCLUSION: Preoperative bladder capacity appears to be an indicator of inherent BN function and a thorough assessment of the urinary tract by cystoscopy, ultrasound, micturating cystourethrogram, and functional imaging may guide the surgeon on the need for BN surgery at the time of ureteric reimplantation. Where continence remains elusive, patients should be counselled that a further BN injection is occasionally of value although more significant BN procedures are required for most.

Canalization of genetic and pharmacological perturbations in developing primary neuronal activity patterns.

The function of the nervous system depends on the integrity of synapses and the patterning of electrical activity in brain circuits. The rapid advances in genome sequencing reveal a large number of mutations disrupting synaptic proteins, which potentially result in diseases known as synaptopathies. However, it is also evident that every normal individual carries hundreds of potentially damaging mutations. Although genetic studies in several organisms show that mutations can be masked during development by a process known as canalization, it is unknown if this occurs in the development of the electrical activity in the brain. Using longitudinal recordings of primary cultured neurons on multi-electrode arrays from mice carrying knockout mutations we report evidence of canalization in development of spontaneous activity patterns. Phenotypes in the activity patterns in young cultures from mice lacking the Gria1 subunit of the AMPA receptor were ameliorated as cultures matured. Similarly, the effects of chronic pharmacological NMDA receptor blockade diminished as cultures matured. Moreover, disturbances in activity patterns by simultaneous disruption of Gria1 and NMDA receptors were also canalized by three weeks in culture. Additional mutations and genetic variations also appeared to be canalized to varying degrees. These findings indicate that neuronal network canalization is a form of nervous system plasticity that provides resilience to developmental disruption. This article is part of the Special Issue entitled 'Synaptopathy--from Biology to Therapy'.

SynGAP regulates ERK/MAPK signaling, synaptic plasticity, and learning in the complex with postsynaptic density 95 and NMDA receptor.

At excitatory synapses, the postsynaptic scaffolding protein postsynaptic density 95 (PSD-95) couples NMDA receptors (NMDARs) to the Ras GTPase-activating protein SynGAP. The close association of SynGAP and NMDARs suggests that SynGAP may have an important role in NMDAR-dependent activation of Ras signaling pathways, such as the MAP kinase pathway, and in synaptic plasticity. To explore this issue, we examined long-term potentiation (LTP), p42 MAPK (ERK2) signaling, and spatial learning in mice with a heterozygous null mutation of the SynGAP gene (SynGAP(-/+)). In SynGAP(-/+) mutant mice, the induction of LTP in the hippocampal CA1 region was strongly reduced in the absence of any detectable alteration in basal synaptic transmission and NMDAR-mediated synaptic currents. Although basal levels of activated ERK2 were elevated in hippocampal extracts from SynGAP(-/+) mice, NMDAR stimulation still induced a robust increase in ERK activation in slices from SynGAP(-/+) mice. Thus, although SynGAP may regulate the ERK pathway, its role in LTP most likely involves additional downstream targets. Consistent with this, the amount of potentiation induced by stimulation protocols that induce an ERK-independent form of LTP were also significantly reduced in slices from SynGAP(-/+) mice. An elevation of basal phospho-ERK2 levels and LTP deficits were also observed in SynGAP(-/+)/H-Ras(-)/- double mutants, suggesting that SynGAP may normally regulate Ras isoforms other than H-Ras. A comparison of SynGAP and PSD-95 mutants suggests that PSD-95 couples NMDARs to multiple downstream signaling pathways with very different roles in LTP and learning.

Quantitative differences in developmental profiles of spontaneous activity in cortical and hippocampal cultures.

BACKGROUND: Neural circuits can spontaneously generate complex spatiotemporal firing patterns during development. This spontaneous activity is thought to help guide development of the nervous system. In this study, we had two aims. First, to characterise the changes in spontaneous activity in cultures of developing networks of either hippocampal or cortical neurons dissociated from mouse. Second, to assess whether there are any functional differences in the patterns of activity in hippocampal and cortical networks. RESULTS: We used multielectrode arrays to record the development of spontaneous activity in cultured networks of either hippocampal or cortical neurons every 2 or 3 days for the first month after plating. Within a few days of culturing, networks exhibited spontaneous activity. This activity strengthened and then stabilised typically around 21 days in vitro. We quantified the activity patterns in hippocampal and cortical networks using 11 features. Three out of 11 features showed striking differences in activity between hippocampal and cortical networks: (1) interburst intervals are less variable in spike trains from hippocampal cultures; (2) hippocampal networks have higher correlations and (3) hippocampal networks generate more robust theta-bursting patterns. Machine-learning techniques confirmed that these differences in patterning are sufficient to classify recordings reliably at any given age as either hippocampal or cortical networks. CONCLUSIONS: Although cultured networks of hippocampal and cortical networks both generate spontaneous activity that changes over time, at any given time we can reliably detect differences in the activity patterns. We anticipate that this quantitative framework could have applications in many areas, including neurotoxicity testing and for characterising the phenotype of different mutant mice. All code and data relating to this report are freely available for others to use.

Preformed silos versus traditional abdominal wall closure in gastroschisis: 163 infants at a single institution.

INTRODUCTION: The surgical management of gastroschisis (GS) is controversial. The most commonly used strategy for abdominal wall closure is surgery on day 1 of life with the aim of primary closure (PC) or construction of a surgical silo (SS) and secondary closure thereafter. The other widely used technique is application of a preformed silo (PFS) and reduction of contents over a few days before final closure. There is still a paucity of comparative outcome data. METHODS: A retrospective case note review of all infants initially treated at a single institution between October 1993 and October 2012. PFS was adopted as the technique of choice in April 2005. Infants with closed or closing GS were excluded. Data are presented as median (range). p < 0.05 were significant. RESULTS: There were 163 infants (156 complete data sets). PFSs were applied in 67 infants and PC/SS were applied in 89 infants of whom 19 infants required a SS. There was no statistical difference between gestational age (p = 0.8), birth weight (p = 0.7), time to first (p = 0.07) and full enteral feeding (p = 0.08), length of hospital stay (p = 0.17), or necrotizing enterocolitis (p = 0.4) and mortality (p = 0.4). Infants treated with PC + SS were closed on day 0 (range, 0-11 days) versus day 6 (range, 2-22 days) of life (p < 0.001). PC + SS were ventilated for day 5 (range, 1-22 days) versus day 3.5 (range, 0-20 days) days (p = 0.01). CONCLUSION: Infants treated with PFS required less ventilation than those treated by PC + SS. There was no difference in time to full feeds, length of hospital stay mortality or morbidity.

Is early delivery beneficial in gastroschisis?

PURPOSE: Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at <37weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD). METHODS: A retrospective review of gastroschisis (1992-2012) linked fetal/neonatal data at 2 tertiary referral centers was performed. Primary outcomes were ENT and length of hospital stay (LOS). Data (median [range]) were analyzed using parametric/non-parametric tests, positive/negative predictive values, and regression analysis. RESULTS: Two hundred forty-six patients were included. Thirty-two were complex (atresia/necrosis/perforation/stenosis). ENT (p<0.0001) and LOS (p<0.0001) were reduced with increasing gestational age. IABD persisted to last scan in 92 patients, 68 (74%) simple (intact/uncompromised bowel), 24 (26%) complex. IABD or EABD diameter in complex patients was not significantly greater than simple gastroschisis. Combined IABD/EABD was present in 22 patients (14 simple, 8 complex). When present at <30weeks, the positive predictive value for complex gastroschisis was 75%. Two patients with necrosis and one atresia had IABD and collapsed extra-abdominal bowel from <30weeks. CONCLUSION: Early delivery is associated with prolonged ENT/LOS, suggesting elective delivery at <37weeks is not beneficial. Combined IABD/EABD or IABD/collapsed extra-abdominal bowel is suggestive of complex gastroschisis.

Dynamic MRI (dMRI) as a guide to therapy in children and adolescents with persistent full thickness rectal prolapse: a single centre review.

INTRODUCTION: Full thickness rectal prolapse (FTRP) tends to be self-limiting in children and is usually managed expectantly. However, it may persist and therefore requires surgical correction. There is no consensus upon operative management, and no one procedure has uniformly good outcomes. The aim of this study was to determine whether pre-operative diagnostic dMRI findings might help identify the operative approach best suited to the anatomical abnormality of the individual child. METHODS: A retrospective review of ten children with persistent FTRP who had been evaluated pre-operatively with dMRI between 2002 and 2010 was performed. In this preliminary work, MRI findings were not used to direct surgical management. Data collected included: age at presentation, underlying medical conditions, timing and findings of dMRI (specifically, descent of rectum from pubococcygeal (PC) line on straining), timing and type of surgery, surgical outcomes, and length of follow-up. RESULTS: Ten children (two female) with a median age of 11 years 2 months (range 8-15 years) with FTRP refractory to conservative treatment underwent diagnostic pre-operative dMRI. Median perineal descent from PC line on straining during dMRI was 3.5 cm (range 1-4 cm). Three of the seven children with severe descent initially underwent a Delorme's procedure, and all required surgical revision. Five with severe descent and one with moderate descent achieved a cure following rectopexy. Two patients with mild descent underwent a Delorme's procedure. One achieved a cure, and the other developed recurrence. Of the ten patients, seven had no prolapse at the last clinic review, and three have persisting symptoms. Median follow-up was 3.5 years (range 1-6). CONCLUSION: The findings from this small study favour rectal suspension techniques for surgical management of moderate to severe perineal descent on dMRI. Delorme's procedure should only be applied to those with mild descent. Pre-operative dMRI assessment may have a potential role in guiding surgical intervention for children. However, future prospective studies will be required to confirm this assertion.

Surgical repair of traumatic amputation of the glans.

Traumatic amputation of the penis in the pediatric population is rare. We present the case of a 4-year-old boy who, shortly after a plastibell circumcision, with the ring still in situ, experienced trauma to his glans, resulting in complete amputation. We describe our technique and outcome after surgical repair.

Single-center experience of laparoscopically assisted percutaneous endoscopic gastrostomy placement.

INTRODUCTION: Percutaneous endoscopic gastrostomy (PEG) is associated with major complications. Recently, laparoscopy has been used with or without endoscopy, in an attempt to reduce complications. The aim of this study was to evaluate a prospective audit of laparoscopically assisted PEG placement. METHODS: A prospective study supported by a case note review of children undergoing laparoscopically assisted PEG placement, with and without Boix-Ochoa fundoplication, between July 2006 and July 2008, was carried out. Data were analyzed for predetermined parameters. SURGICAL TECHNIQUE: Two 5-mm ports are utilized with the working port in the right-upper quadrant. The gastroscope is placed in the stomach. An atraumatic grasper is used to secure the stomach. The needle and sheath is passed through the anterior wall and into the stomach under direct vision, ensuring placement in the desired location. The gastrostomy-tube (GT) position is checked by the endoscopist and laparoscopist. RESULTS: Twenty-nine children underwent a laparoscopically assisted PEG placement with (4 infants) and without (25 infants) laparoscopic fundoplication. There were 13 girls and 16 boys. The median age was 1 year and 8 months (range, 6 months to 16 years and 2 months). There were no complications related to laparoscopy. There were 3 superficial gastrostomy wound infections. Follow-up is 6 (range, 1-21) months. CONCLUSIONS: Laparoscopically assisted PEG placement is a safe alternative to conventional methods. This technique provides direct visualization of the gastrostomy placement, avoiding other viscera, and allowing for the ideal siting of the GT.

A radiological chronicle of the presentation and management of a long gap oesophageal atresia.

Long gap oesophageal atresia is a clinically and technically challenging condition to manage. Documentation of the gap between the upper and lower pouches is critical to deciding the timing and feasibility of a primary anastamosis. Integral to this process is the role of radiology. We present a case of long gap oesophageal atresia accompanied by chronological radiography demonstrating its' staged management and highlighting some common complications.

Natural history and long-term follow-up of antenatally detected liver cysts.

BACKGROUND/PURPOSE: Antenatally detected liver cysts are rare; their diagnostic accuracy is unknown, and their management is controversial. This study assessed the natural history of these lesions. METHODS: We conducted a retrospective review of infants with isolated intrahepatic cysts that were detected antenatally. Data are expressed as median (range). RESULTS: Fifteen infants presented during the period 1991-2004 with an antenatally detected liver cyst. Their gestational age at detection was 22 (18-34) weeks, and the maximum diameter of their cyst was 23 (10-120) mm. Serial scans, which were performed in 9 fetuses, showed cyst enlargement in 5 cases, diminution in 1 case, and no change in 3 cases. In utero percutaneous aspiration was required in 1 infant. Three infants underwent postnatal surgery. One fetus (postfetal intervention) had a subtotal excision of a large subcapsular cyst filling the abdominal cavity on day 2. Another infant required partial excision and marsupialization (complex cyst arising from segment IV) at 5 months, and a third infant underwent a cyst cholecystostomy at 4 months. Postnatal investigations (including hepatic scintigraphy) suggested that the remaining lesions were either simple parenchymal (n = 10) or isolated intrahepatic choledochal (ie, type V; n = 2) cysts. The median follow-up for these patients was 44 (27-167) months. Serial postnatal ultrasonography showed cyst diminution in 4 cases, an enlargement in 1 case, and no dimensional change in 7 cases. CONCLUSIONS: Most antenatally detected liver cysts appear to be simple and of parenchymal origin and do not require fetal intervention. Their postnatal history is variable, but regression without treatment is seen in most cases.

Network activity-independent coordinated gene expression program for synapse assembly.

Global biological datasets generated by genomics, transcriptomics, and proteomics provide new approaches to understanding the relationship between the genome and the synapse. Combined transcriptome analysis and multielectrode recordings of neuronal network activity were used in mouse embryonic primary neuronal cultures to examine synapse formation and activity-dependent gene regulation. Evidence for a coordinated gene expression program for assembly of synapses was observed in the expression of 642 genes encoding postsynaptic and plasticity proteins. This synaptogenesis gene expression program preceded protein expression of synapse markers and onset of spiking activity. Continued expression was followed by maturation of morphology and electrical neuronal networks, which was then followed by the expression of activity-dependent genes. Thus, two distinct sequentially active gene expression programs underlie the genomic programs of synapse function.