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Enhanced recovery protocols (ERPs) are effective means of standardizing and improving the quality of surgical care in adults. Our purpose was to retrospectively compare outcomes before and after implementation of ERPs in children undergoing laparoscopic Heller myotomy for achalasia.A pediatric-specific ERP was used for children undergoing laparoscopic Heller myotomy starting July 2017 at two pediatric surgery centers within a single metropolitan healthcare system. A retrospective review of 8 patients undergoing Heller myotomies between July 2014 and July 2017 was performed as a control. This cohort was compared to 14 patients managed post-ERP implementation (2017-2020). Outcomes of interest investigated included opioid use during admission, narcotics at discharge, time to regular diet, length of stay (LOS), and readmissions.There was a significant decrease in opioid use both while in the hospital and at time of discharge. Mean morphine equivalent use was 4.50 mg in the pre-ERP cohort and 1.97 mg in the post-ERP cohort. Furthermore, 8 out of 14 (57%) patients in the post-ERP cohort received no opioids during the admission compared with only 2 out of 8 (25%) patients in the pre-ERP cohort. Only 1 out of 14 (7.14%) patients in the post-ERP cohort was discharged with a prescription for opioid medication while 6 out of 8 (75%) in the pre-ERP cohort were discharged with an opiate prescription.The use of ERP in children undergoing laparoscopic Heller myotomy surgery is safe and effective and leads to a reduction in opioid use during admission and at discharge.Level III.

Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder affecting respiratory control and autonomic nervous system function caused by variants in the paired-like homeobox 2B (

Utilization of extracorporeal membrane oxygenation (ECMO) in the pediatric population requires consideration of numerous factors, including the size of the patient and indication. This chapter reviews several common indications for ECMO cannulation in pediatric patients, consideration for ECMO modality (i.e., veno-arterial [V-A] ECMO) versus veno-venous ([V-V] ECMO), and cannula placement site. ECMO is a well-established means to provide cardiopulmonary support in the neonatal/pediatric populations with select diagnoses, including respiratory failure, acute cardiac failure, and septic shock. Given the variability of patient size, diagnoses, and cardiopulmonary support requirements, various ECMO cannulation/configurations exist and each must be considered on a patient-by-patient basis. Three case presentations are followed by key point discussions and board-type multiple choice questions summarizing this chapter.

This is a commentary on the manuscript titled “Long-Term Outcomes of Congenital Diaphragmatic Hernia: A Single Institution Experience” by Gerall C, Stewart L, Price J, et al.

Purpose: The purpose of this study was to determine if utilization of biologic mesh underlay during thoracoscopic congenital diaphragmatic hernia (CDH) primary repair (PR) results in reduc...

Purpose The aim of this study was to determine long-term outcomes for congenital diaphragmatic hernia (CDH) patients including quality of life (QoL), symptom burden, reoperation rates, and health status. Methods A chart review and phone QoL survey were performed for patients who underwent CDH repair between 2007 and 2014 at a tertiary free-standing children’s hospital. Comprehensive outcomes were collected including subsequent operations and health status. Associations with QoL were tested using Wilcoxon Rank-Sum tests and Pearson correlation coefficients. Results Of 102 CDH patients identified, 46 (45.1%) patient guardians agreed to participate with mean patient age of 5.8 (SD, 2.2) years at time of follow-up. Median PedsQLTM and PedsQLTM Gastrointestinal scores were 91.8 (IQR, 84.8–95.8) and 95.8 (IQR, 93.0–98.2), out of 100. Thoracoscopic repair was associated with higher PedsQLTM scores while defects with an intrathoracic stomach were associated with increased gas and bloating. No difference in QoL was found when comparing defect side, patch vs primary repair, prenatal diagnosis, extracorporeal membrane oxygenation, or recurrence. Older age weakly correlated with worse school functioning and heartburn. Conclusion Children with CDH have reassuring QoL scores. Given the correlation between older age and poor school function, longer follow-up of patients with CDH may be warranted. Level of Evidence III (Retrospective comparative study).

Neonates with intestinal atresia (IA) undergo either primary anastomosis (PA) or ostomy creation with secondary anastomosis (SA). Our purpose was to compare outcomes for PA and SA and to assess factors influencing procedure selection.We conducted a retrospective cohort study of neonates with IA between 2009 and 2015. Patient characteristics, operative details, and outcomes were collected. Surgeon-level preferences (defined as performing50% PA or SA) were assessed using logistic regression.Of 92 IA patients, 70 (76.1%) underwent PA and 22 (23.9%) underwent SA. Neonates with PA had shorter hospitalizations (27 days vs. 95 days, p 0.001), shorter total parenteral nutrition duration (19 days vs. 74.5 days, p 0.001), and fewer readmissions (33.3% vs. 63.2%, p = 0.024). On multivariable regression analysis, higher Apgar scores (Odds Ratio (OR) 4.16, 95% Confidence Interval (CI) 1.20-14.29) and uncomplicated atresia (OR 3.97, 95% CI 1.37-11.48) were associated with PA. At the surgeon-level, utilization of PA varied from 43.5% to 100%. Surgeon preference is not influenced by the demographic, presentation, or surgical findings of this patient population.PA has better outcomes than SA. Though procedural selection is influenced by the clinical status of the neonate, however surgeon preference plays a significant role in this clinical decision.Level III Treatment Study.

OBJECTIVES To advance our understanding of monogenic forms of intrahepatic cholestasis. METHODS Analyses included participants with pathogenic biallelic mutations in adenosine triphosphate (ATP)-binding cassette subfamily B member 11 (ABCB11) (bile salt export pump; BSEP) or adenosine triphosphatase (ATPase) phospholipid transporting 8B1 (ATP8B1) (familial intrahepatic cholestasis; FIC1), or those with monoallelic or biallelic mutations in adenosine triphosphate (ATP)-binding cassette subfamily B member 4 (ABCB4) (multidrug resistance; MDR3), prospectively enrolled in the Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis (LOGIC; NCT00571272) between November 2007 and December 2013. Summary statistics were calculated to describe baseline demographics, history, anthropometrics, laboratory values, and mutation data. RESULTS Ninety-eight participants with FIC1 (n = 26), BSEP (n = 53, including 8 with biallelic truncating mutations [severe] and 10 with p.E297G or p.D482G [mild]), or MDR3 (n = 19, including four monoallelic) deficiency were analyzed. Thirty-five had a surgical interruption of the enterohepatic circulation (sEHC), including 10 who underwent liver transplant (LT) after sEHC. Onset of symptoms occurred by age 2 years in most with FIC1 and BSEP deficiency, but was later and more variable for MDR3. Pruritus was nearly universal in FIC1 and BSEP deficiency. In participants with native liver, failure to thrive was common in FIC1 deficiency, high ALT was common in BSEP deficiency, and thrombocytopenia was common in MDR3 deficiency. sEHC was successful after more than 1 year in 7 of 19 participants with FIC1 and BSEP deficiency. History of LT was most common in BSEP deficiency. Of 102 mutations identified, 43 were not previously reported. CONCLUSIONS In this cohort, BSEP deficiency appears to be correlated with a more severe disease course. Genotype-phenotype correlations in these diseases are not straightforward and will require the study of larger cohorts.

Few data exist on bisphenol A (BPA) or triclosan (TCS) residue levels in foods consumed by adults in everyday settings. In a further analysis of study data, the objectives were to determine BPA and TCS residue concentrations in duplicate-single solid food items consumed by adults and to estimate dietary exposure and intake doses per food item. A convenience sample of 50 adults was recruited in North Carolina (2009–2011). Participants completed 24 h food diaries and collected 24 h duplicate-diet solid food samples consumed on days 1 and 2 during sampling weeks 1, 2, and 6. A total of 188 of the collected 776 duplicate-diet solid food samples contained a single, solid food item. BPA and TCS residue levels were quantified in the 188 food items using GC–MS. BPA and TCS were detected in 37% and 58% of these food items, respectively. BPA concentrations were highest in a cheese and tomato sandwich (104 ng/g), whereas the highest TCS concentrations were in a burrito (22.1 ng/g). These chemicals co-occurred in 20% of the samples (maximum = 54.7 ng/g). Maximum dietary intake doses were 429 ng/kg/day for BPA in a vegetable soup with tortilla sample and 72.0 ng/kg/day for TCS in a burrito sample.

Pediatric gallstone disease is on the rise in the United States, for two main reasons. First, improved diagnostic tools including high-resolution ultrasound have made the detection of small stones more readily apparent. Secondly, dietary changes have led to an increased incidence of cholesterol stones. Additionally, improved survival in the neonatal population, many of whom are maintained on parenteral nutrition for an extended period of time, has led to pathophysiology previously seen in very limited instances. Laparoscopic cholecystectomy remains the standard operation of choice for patients requiring surgical extirpation of the gallbladder alone. Regional variation in expertise dictates the management of choledocholithiasis using either laparoscopic common bile duct exploration or endoscopic retrograde cholangiopancreatography.

Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has been associated with faster recovery, earlier extubation, and decreased morbidity. Nevertheless, thoracoscopic repair is rarely attempted in the post-extracorporeal membrane oxygenation (ECMO) patient. Commonly cited reasons for not attempting thoracoscopy include concerns that the patients' respiratory status is too tenuous to tolerate insufflation pressures or that presumed defect size is so large that it precludes thoracoscopic repair. Our purpose is to review our experience with post-ECMO thoracoscopic CDH repair and evaluate the success of this approach.We performed retrospective analysis of attempted thoracoscopic CDH repairs after ECMO decannulation at our institution from 2001 to 2015. Primary outcome was rate of conversion. Secondary outcomes were intraoperative end-tidal COWe identified 21 post-ECMO patients in whom thoracoscopic CDH repair was attempted. Thoracoscopic repair was successfully completed in 28%. No patients had reported intolerance to insufflation at 3-7 mmHg. Average end-tidal COThoracoscopic CDH repair is both safe and feasible after ECMO with no increase in operative morbidity or mortality. Insufflation pressures of 3-7 mmHg are well tolerated without undue increase in end-tidal CO

Paragangliomas are rare neuroendocrine tumors where hypoxia-inducible factor plays a critical role in tumorigenesis. It has been suggested that patients with congenital heart disease, in particular, may have cellular environment and relative hypoxia favorable to the development of these neuroendocrine tumors. Here, we present a case of an 11-year-old child with hypoplastic left heart syndrome previously palliated with Fontan procedure, diagnosed with paraganglioma on surveillance imaging. We present the clinical course, intervention, and outcome as well as review the possible contributory mechanisms. As we continue to improve long-term survival for single ventricle patients, awareness of these tumors during surveillance may be warranted as timely intervention may lead to cure.

Congenital diaphragmatic hernia can be approached successfully using minimally invasive techniques. Although there are may be a suggestion of higher recurrence rates with thoracoscopic repair, this may be due to the learning curve. However, open repair is associated with additional morbidity, most notably an increased rate of small bowel obstruction. Appropriate patients who have congenital diaphragmatic hernia should be offered the benefits of minimally invasive repair.

Background Numerous thoracoscopic techniques have been used in the management of primary spontaneous pneumothorax (PSP), including wedge resection, pleurectomy, pleural abrasion, chemical pleurodesis, and staple line covering. The purpose of this systematic review was to compare outcomes for the most commonly reported techniques. Materials and methods A systematic literature search looking at pneumothorax recurrence rate, length of stay, and chest tube duration after surgery was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the PubMed database. Results Fifty-one unique studies comprised of 6907 patients published between January 1988 and June 2015 were identified. Heterogeneity among effect sizes was significant for all outcomes. The lowest recurrence rates were observed in the wedge resection + chemical pleurodesis (1.7%; 95% confidence interval [CI], 1.0%-2.7%) and the wedge resection + pleural abrasion + chemical pleurodesis (2.8%; 95% CI, 1.7%-4.7%) groups. The shortest chest tube duration and length of stay were observed in the wedge resection + staple line covering ± other group (2.1 d; 95% CI, 1.4-2.9 and 3.3 d; 95% CI, 2.6-4.0, respectively). Conclusions The variability in reported outcomes and the lack of published multicenter randomized controlled trials highlights a need for more robust investigations into the optimal surgical technique in the management of PSP. Based on the limited quality studies available, this systematic review favors wedge resection + chemical pleurodesis and wedge resection + pleural abrasion + chemical pleurodesis in terms of recurrence rate after surgery for PSP.

Postoperative pneumothorax and effusion remain a concern following congenital diaphragmatic hernia (CDH) repair. Despite a recent trend away from intraoperative thoracostomy, few studies have actually compared outcomes with and without a chest tube. Rationale commonly cited for the more minimalistic approach include the presumed low likelihood of postoperative complications, potential risk of patch infection, and prolonged intubation. We evaluate these theories, as well as the implications of intraoperative chest tube (IOCT) placement.We performed a retrospective chart review of 174 patients who underwent CDH repair at our academic children's hospital from 2004 to 2015. We compared incidence of clinically significant pleural events between patients who received an IOCT (n = 49) and those who did not (NIOCT, n = 124). We also evaluated time to extubation and rate of patch infections.Clinically significant pneumothorax or effusion occurred in 28% of NIOCT patients versus 10% of IOCT patients (P = .01). After thoracoscopic repair, time to extubation averaged 5.2 days in IOCT patients, 5.4 days in NIOCT patients with no postoperative complications, and 6.4 days in NIOCT patients requiring postoperative intervention. After open repair, time to extubation averaged 13.8, 13.6, and 22.5 days, respectively. There were no documented patch infections.Chest tube placement during CDH repair is associated with significantly lower incidence of clinically significant pleural complications, does not delay extubation, and results in shorter ventilator times than cases that require postoperative intervention. Patch infections are extremely rare. There is no evidence that chest tube placement increases this risk.

Minimally invasive surgery (MIS) in the management of blunt and penetrating pediatric trauma has evolved in the past 30 years. Laparoscopy and thoracoscopy possess high levels of diagnostic accuracy with low associated missed injury rates. Currently available data advocate limiting the use of MIS to blunt or penetrating injuries in the hemodynamically stable child. In the pediatric trauma population, MIS offers both diagnostic and therapeutic potential, as well as reduced postoperative pain, a decreased rate of postoperative complications, shortened hospital stay, and potentially reduced cost.

Physician Burn Out has become endemic as Healthcare has become increasingly complex and challenging to physicians who provide care to critically ill patients. Physicians are more prone to make errors when burnt out. Most Healthcare institutions have not been responsive to support physicians when burned out or when disturbing outcomes occur. Peer support has evolved as the preferred way to help a physician recover after the loss of a patient or a medical error. Best practices are evolving as awareness increases and better research questions arise. Individuals providers, surgical leaders and institutions can all be influential as we address this problem.

Pyrethroid insecticides are widely used to control insects in both agricultural and residential settings worldwide. Few data are available on the temporal variability of pyrethroid metabolites in the urine of non-occupationally exposed adults. In this work, we describe the study design and sampling methodology for the Pilot Study to Estimate Human Exposures to Pyrethroids using an Exposure Reconstruction Approach (Ex-R study). Two major objectives were to quantify the concentrations of several pyrethroid metabolites in bedtime, first morning void (FMV), and 24-h urine samples as concentration (wet weight), specific-gravity (SG) corrected, creatinine (CR) corrected, and excretion rate values for 50 Ex-R adults over a six-week monitoring period and to determine if these correction approaches for urine dilution reduced the variability of the biomarker levels. The Ex-R study was conducted at the United States Environmental Protection Agency's Human Studies Facility in Chapel Hill, North Carolina USA and at participants' homes within a 40-mile radius of this facility. Recruitment of participants and field activities occurred between October 2009 and May 2011. Participants, ages 19-50 years old, provided daily food, activity, and pesticide-use diaries and collected their own urine samples (bedtime, FMV, and 24-h) during weeks 1, 2, and 6 of a six-week monitoring period. A total of 2503 urine samples were collected from the study participants. These samples were analyzed for the pyrethroid metabolites 3-phenoxybenzoic acid (3-PBA), cis/trans-3-(2,2-dichlorovinyl)-2,2-dimethyl-cyclopropane carboxylic acid (cis/trans-DCCA), and 2-methyl-3-phenylbenzoic acid (MPA) using high performance liquid chromatography/tandem mass spectrometry. Only 3-PBA was frequently detected (>50%) in the adult urine samples. Median urinary 3-PBA levels were 0.88 ng/mL, 0.96 ng/mL-SG, 1.04 ng/mg, and 1.04 ng/min for concentration, SG-corrected, CR-corrected, and excretion rate values, respectively, across all urine samples. The results showed that median urinary 3-PBA concentrations were consistently the lowest in FMV samples (0.77 ng/mL, 0.68 ng/mL-SG, 0.68 ng/mg, and 0.58 ng/min) and the highest in 24-h samples (0.92 ng/mL, 1.06 ng/mL-SG, 1.18 ng/mg, and 1.19 ng/min) across all four methods. Intraclass correlation coefficient (ICC) estimates for 3-PBA indicated poor reproducibility (

The appearance of the diaphragmatic curvature and the rib insertion level of the diaphragm on postoperative chest X-ray (CXR) may predict recurrence. Our purpose was to examine the relationship between the curvature of the diaphragm on postoperative CXR and recurrence.We performed a retrospective review of left-sided, Bochdalek congenital diaphragmatic hernia (CDH) surgical repairs from 2004 to 2015 at a single institution. We developed a tool to measure the flatness of the diaphragm on postoperative CXR, termed the diaphragmatic curvature index (τ). The primary outcome of interest was recurrence after surgical repair.Of the 127 patients identified, 54% (n = 69) had a primary repair, while 46% (n = 58) required a patch repair. The overall recurrence rate was 21.3% (n = 27). There was no difference in median lateral rib insertion level in patients with and without recurrence or those who had a primary or patch repair. The overall median diaphragmatic curvature index was 6.29 (interquartile range [IQR] 5.30-8.09) and was not significantly different among patients who had a recurrence (6.00, IQR 5.34-8.24) and those who did not (6.46, IQR 5.24-8.07) (P = .853). Within the primary repair group (6.34 versus 6.93, P = .84) and the patch repair group (5.59 versus 6.18, P = .46), the median diaphragmatic curvature index was not different among patients who had a recurrence and those who did not.A flat appearance of the diaphragm on postoperative CXR as measured by the median diaphragmatic curvature index (τ) is not associated with recurrence. The shape of the diaphragm on CXR after CDH repair may not be predictive of recurrence as previously thought.

Total proctocolectomy (TPC) and ileal pouch anal anastomosis (IPAA) have become the standard of care for patients with ulcerative colitis refractory to medical management. The purpose of our study is to show our single-site approach and to identify maneuvers that improve efficiency.We retrospectively reviewed patients who underwent single-site three-stage TPC-IPAA for ulcerative colitis at our institution. Primary outcomes included operative time, conversion from single site to standard laparoscopy, time to oral intake and stoma function, postoperative complications, and length of stay. The GelPOINT(™) Advanced Access Platform (Applied Medical, Santa Margarita, CA) was used.Eight patients were identified who had undergone single-site surgery with the GelPOINT platform. Six of the 8 patients underwent the first stage, total abdominal colectomy (TAC), and all 8 underwent the second stage (proctectomy/IPAA). The mean operating time for TAC was 242 ± 32 minutes. The mean time until tolerance of clear diet was 1.2 ± 0.4 days, and time until tolerance of regular diet was 3.3 ± 1.2 days. The mean time to stoma function was 1.5 ± 0.55 days, and that for postoperative opioid use was 4.0 ± 1.3 days. The median length of stay was 5 days (range, 3-10 days). There was one postoperative complication. The mean operating time for the proctectomy/IPAA was 283 ± 50 minutes. The mean time until tolerance of clear diet was 1.0 ± 0.5 days, and time until tolerance of regular diet was 3.3 ± 1.1 days. The mean time to stoma function was 1.6 days ± 0.52 days, and that for postoperative opioid use was 3.3 ± 1.4 days. Median length of stay was 4 days (range, 3-9 days). There was one postoperative complication. Technical adaptations that included extracorporeal mesenteric division, rectal eversion, and rotation of the GelPOINT device served to improve the ease and efficiency of the procedure.Single-site TPC-IPAA is both feasible and safe. Incorporation of adapted technical maneuvers can increase efficiency.

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ-glutamyl transpeptidase disease (levels100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs.24 months postoperatively, respectively; P0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation.This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645-1654).

The treatment algorithm for children with suspected choledocholithiasis is not well established because the breadth of minimally invasive surgery and endoscopic techniques continues to evolve. We reviewed our experience with common bile duct explorations (CBDEs) in order to detail the techniques used and describe the rate of complications of laparoscopic CBDE in children.As part of an Institutional Review Board-approved study, medical records were reviewed for all patients, 1 month to 21 years of age, undergoing a cholecystectomy at a large tertiary-care children's hospital over an 11-year period. Those undergoing an intraoperative cholangiogram (IOC) were documented, and operative reports and postoperative records were examined.Over 11 years, 464 cholecystectomies were performed, and an IOC was attempted on 174 patients with a 97% success rate (n=168). Of the patients who underwent a cholangiogram, 30% (n=52) had an obstructing stone. Laparoscopic CBDE was attempted in 50 patients with a conversion rate of 8%. Postoperatively, 3 CBDE patients underwent endoscopic retrograde cholangiopancreatography (ERCP) for the following reasons: retained stone (n=1), persistent hyperbilirubinemia (n=1), and bile leak (n=1).Laparoscopic CBDE is a safe initial approach to choledocholethiasis and is successful at relieving the obstruction the majority of the time. The authors conclude that in situations where there is limited availability of ERCP, laparoscopic CBDE should be considered as a first step in the management of obstructive choledocholethiasis.

Infants with congenital cardiac disease (CCD) often require gastrostomy tube placement (GT) and need antireflux procedures, such as fundoplications. Our purpose was to compare morbidity/mortality rates among infants with CCD undergoing GT, fundoplication, or both. Using the NSQIP-Pediatric, we identified 4070 patients

Noniatrogenic neonatal gastric perforation is a rare and life-threatening condition whose etiology is often unclear. Interstitial cells of Cajal act as gastrointestinal pacemaker cells and express the proto-oncogene c-Kit. Six new cases were identified at our institution which presented with no mechanical, pharmacologic, or otherwise medical-related intervention prior to rupture. The number of interstitial cells of Cajal in nonnecrotic muscularis propria from five random high-power fields per specimen was compared using immunohistochemical stains for c-Kit. The authors show that a lack of interstitial cells of Cajal in the stomach musculature may be implicated in the development of noniatrogenic gastric perforation (p = 0.008). Further large-scale studies, including molecular and genetic analysis, may help to better understand this phenomenon.

Indoor residence times of semivolatile organic compounds (SVOCs) are a major and mostly unavailable input for residential exposure assessment. We calculated residence times for a suite of SVOCs using a fugacity model applied to residential environments. Residence times depend on both the mass distribution of the compound between the "mobile phase" (air and dust particles settled on the carpet) and the "non-mobile phase" (carpet fibers and pad) and the removal rates resulting from air exchange and cleaning. We estimated dust removal rates from cleaning processes using an indoor-particle mass-balance model. Chemical properties determine both the mass distribution and relative importance of the two removal pathways, resulting in different residence times among compounds. We conducted a field study after chlorpyrifos was phased out for indoor use in the United States in 2001 to determine the decreases in chlorpyrifos air concentrations over a one-year period. A measured average decrease of 18% in chlorpyrifos air concentrations indicates the residence time of chlorpyrifos is expected to be 6.9 years and compares well with model predictions. The estimates from this study provide the opportunity to make more reliable estimates of SVOCs exposure in the indoor residential environment.

Enteritis caused by Clostridium difficile toxin (Tx) is a nosocomial disease of increasing clinical concern, but the local mediators of C. difficile TxA inflammation are unknown. The potent vasodilator calcitonin gene-related peptide mediates neurogenic inflammation via the calcitonin receptor-like receptor (CLR). Here we examined the ileum-specific effects of reducing CLR on TxA ileitis by local preinjection of double-stranded RNAs. Treatment with CLR dsRNA for 7 d decreased CLR immunoreactivity, whereas treatment with non-CLR dsRNA did not. Subsequent injection of TxA in the same location increased CLR in rats treated with non-CLR dsRNA but not in rats treated with CLR dsRNA, documenting that local injection of dsRNA is effective in preventing the increase in CLR immunoreactivity in response to local TxA. After non-CLR dsRNA pretreatment, TxA induced robust intestinal secretion, myeloperoxidase activity, and histopathologic indications of inflammation including epithelial damage, congestion, neutrophil infiltration, loss of mucin from goblet cells, and increase in mast cell numbers. After CLR dsRNA pretreatment, TxA-induced changes in intestinal secretion and histopathologic inflammation were improved, including normal mucin staining and fewer resident mast cells. Loss of CLR prevented TxA-mediated activation of NF-κB and concomitant increases in pERK1/2 and TNF-α mRNA. Locally produced CLR plays a proinflammatory role in TxA ileitis via MAPK signaling and TNF-α. The results reported here strongly suggest that a local injection of dsRNA targeting CLR could be an effective local therapeutic approach at the inflammation site in the treatment of a growing, clinically relevant hospital-acquired disease, C. difficile infection.

Ciliated hepatic foregut cysts (CHFCs) are rare congenital legions that arise from the embryonic foregut. The cysts are formed during fetal development by evagination from their respective portions of the foregut, and are characterized by a ciliated epithelial lining. Approximately 100 cases of CHFC have been reported, of which only 13 were in children. Although CHFC is typically benign, malignant transformation to squamous cell carcinoma (SCC) has been reported in 3 cases. Survival rate after progression to malignancy is poor, as SCC in this setting is biologically aggressive. We present 4 new cases of CHFC in children between 5 months and 17 years old. Our cases are unusual, as some of the cysts exhibit multilocularity and biliary communication, and 2 of our patients were diagnosed under the age of 1. Additionally, 1 of the cysts was 19.3 cm in diameter, making it the largest reported CHFC to our knowledge. Ciliated hepatic foregut cysts should be included in the differential diagnosis of hepatic lesions.

Unlike other sharp objects, pens and pencils are readily available to children both at home and school. Although case reports are published, no series of pen or pencil injuries have been reported in the recent literature. We therefore reviewed the incidence and injury profiles of writing instruments as compared with other sources of penetrating trauma. The trauma registry from a large urban pediatric hospital system was queried for nonmissile, nonbite penetrating injuries from 2005 through 2009. Retrospective data was collected on demographics, injuries, operations, admissions, and mortalities. Additionally, data regarding pen and pencil injuries from 2009 to 2010 were collected prospectively, and one case from 2003 was included retrospectively. Fourteen injuries from writing instruments were seen and involved the head and neck (9), chest (1), bladder/perineum (2), and extremities (2). Eleven children were admitted and eight required surgical intervention. One child died from a transhemispheric brain injury after intraorbital penetration by a pencil. Penetrating trauma from writing instruments is not an uncommon source of injury and often requires surgical intervention to remove the object. Injuries from pens and pencils can be severe or even fatal. Appropriate parent and teacher education regarding the potential risks may help to prevent such injuries.

Background/Purpose Closure of abdominal wall defects in children poses a challenge for pediatric surgeons. We describe a technique using tissue expanders placed either intraperitoneally or in the abdominal wall to aid in the reconstruction of a variety of complex abdominal wall defects. Methods The tissue expanders are inserted under general anesthesia. Initial expansion is done in the operating room with attention to peak airway pressure, urine output, and end-tidal carbon dioxide. The expanders are inflated in the outpatient setting via percutaneous access until the calculated inflation volume is achieved. They are then removed; and definitive closure is accomplished using a combination of native tissue flaps, abdominal component separation techniques, biomaterials, and synthetic material. Results Six children underwent tissue expansion for treatment of abdominal wall defects (omphalocele, n=3), trauma (n = 1), and thoracopagus twins (n = 1 pair). One to 4 expanders were used per patient, with all having a successful reconstruction of their abdominal walls. Two to 3 operations were required to restore abdominal domain and consisted of expander insertion, removal with reconstruction, and possible revision of the reconstruction. Conclusions Tissue expanders possess a broad range of applications for abdominal wall reconstruction and can be used in patients of all ages.

Background/Purpose Progressive familial intrahepatic cholestasis syndromes are characterized by impaired bile acid secretion resulting in pruritus, coagulopathy, diarrhea, and malnutrition leading to progressive liver failure and death in childhood. Partial internal or external biliary drainage can relieve symptoms and slow the progression of the disease. Objections to partial external biliary drainage include the need for a permanent biliary stoma with all the inherent complications of a stoma. We propose a novel approach to these diseases—placement of a "button" cholecystostomy tube. Methods Under general anesthesia and through a small right subcostal incision, a MIC-KEY button (Kimberly-Clark Worldwide, Inc, Draper, UT) is inserted into the mobilized fundus of the gallbladder and secured with 2 purse-string sutures. Time of drainage is adjusted to relieve pruritus. Results Three children with progressive familial intrahepatic cholestasis achieved adequate bile drainage via the cholecystostomy button to relieve pruritus for 1, 2, and 2 ½ years postoperatively, with drainage periods of 12 to 14 hours per day. There were no episodes of cholangitis. Dislodged tubes can be replaced, or stones can be retrieved via the tract that is formed. Patient (parent) acceptance has been excellent. Conclusion Button cholecystostomy is simple to perform, relieves pruritus with intermittent (nighttime) drainage, avoids complications of a permanent stoma, avoids an enteric anastomosis, and is accepted by parents.

Iatrogenic esophageal perforation in infants is an uncommon though recognized complication resulting from the insertion of a transesophageal echocardiogram (TEE) probe into the oropharynx. Infants requiring TEE are almost universally affected by underlying cardiac disease; thus, minimizing unnecessary interventions is the goal. We reviewed our institution's experience with esophageal perforation resulting from TEE probes in order to define effective management strategies. After IRB approval, we conducted a 12-year retrospective review of our institution's experience with esophageal perforation in infants resulting from TEE probes. During our study period, 3322 infants had a TEE probe placed. Four infants (age range 2–120 days) sustained an esophageal perforation from a TEE probe, indicating that the incidence at our institution is 0.12%. Evaluation with contrast esophagram or direct laryngoscopy confirmed the presence of perforation in all cases. Management consisted of broad-spectrum antibiotics and nothing per os. One patient developed a pseudodiverticulum, which regressed spontaneously. There were no other complications resulting from perforation. Transesophageal echocardiogram probe insertion in infants with cardiac anomalies can lead to esophageal perforation. These patients can be managed non-operatively with broad-spectrum antibiotics and nothing per os. Oral feeding may resume once the perforation is healed on esophagram.

Vascular endothelial growth factor (VEGF) is required for blood vessel formation during lung growth and repair. Alteration of VEGF isoform expression has been demonstrated in response to fetal tracheal occlusion and in models of lung injury. The purpose of this study was to investigate VEGF expression during compensatory lung growth in the mouse.Under general anesthesia, adult mice underwent left thoracotomy with (n = 5) or without (sham, n = 5) pneumonectomy. The right lungs were harvested at 1, 3, and 7 d after the operation. Lung-to-body weight ratio as well as total DNA and protein content were measured. VEGF protein expression was analyzed by Western blot and ELISA. VEGF isoform expression was evaluated using semi-quantitative PCR followed by Imagequant optical densitometry. Values were compared by Student's t-test and ANOVA using Fisher's protected least significant difference post-hoc test where appropriate.Compensatory lung growth was observed as measured by increases in right lung-to-body weight ratio and in DNA and protein content. Total VEGF RNA and protein expression did not change after pneumonectomy. However, on post-operative day 1, there was a decrease in the relative percentage of VEGF188 mRNA (P0.01), and an increase in the relative percentage of VEGF164 mRNA (P = 0.05). At 3 d postpneumonectomy, low relative VEGF188 expression persisted (P0.05), VEGF164 expression normalized, and relative VEGF120 expression increased (P0.01). Isoform expression in the pneumonectomy animals was identical to sham animals by the seventh d. There were no differences observed in VEGF receptor expression.During compensatory lung growth, we have observed an early postoperative reversion of VEGF isoform expression to the pattern seen during fetal lung development and in lung injury models.

Intestinal atresias are a common cause of newborn bowel obstruction (Dalla Vecchia LK, Grosfeld JL, West KW, et al, Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133[5]:490-496). Hereditary multiple intestinal atresias, first reported by Guttman et al in 1973, is the rarest form of multiple atresias (Guttman FM, Braun P, Garance PH, et al, Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg 1973;8:633-640; Bass J, Pyloric atresia associated with multiple intestinal atresias and immune deficiency. J Pediatr Surg 2002;37:941-942.). It has been proposed to be autosomal recessive, to involve atresias in a variable combination of sites from stomach to rectum, and to be universally fatal (Bilodeau A, Prasil P, Cloutier R, et al, Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg 2004;39:726-730; Moreno LA, Gottrand F, Turck D, et al, Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family. Am J Med Genet 1990;37:143-146). Patients have significant intestinal dysfunction and unrelenting sepsis stemming from a poorly defined, severe immunologic defect. Our case report presents 2 full siblings to nonconsanguineous parents with pyloric atresia, multiple small bowel and colonic atresias, and severe immune dysfunction. Care was withdrawn within 3 months of life on both siblings after multiple bouts of sepsis. Data suggest that the immune defect may not be primary, but in fact be secondary to intestinal dysfunction. Although the subjects in this article ultimately had fatal outcomes, a comprehensive immunologic/physiologic picture is presented in hopes of furthering the understanding of this grave disease.

Respiratory distress syndrome (RDS) is a major cause of neonatal morbidity and mortality due to insufficient surfactant production in the lungs (1–3). The disease primarily affects premature infants and is the seventh leading cause of death in infants under 1 year of age (1,2). Excreted surfactant aggregates to form a monolayer complex that reduces alveolar surface tension and facilitates alveolar inflation (4). Insufficient surfactant at birth results in collapse of the fetal alveoli and RDS. Surfactant is composed predominantly of phosphatidylcholine (lecithin) (70%) with lesser amounts of other phospholipids, including phosphatidylglycerol, phosphatidylethanolamine, and phosphatidylinositol (5). Measurement of the lecithin-to-sphingomyelin (LS) ratio in amniotic fluid samples is traditionally considered to be the gold standard for fetal lung maturity testing, but the assay is time-consuming and technically challenging to perform. Consequently, many hospitals now measure the surfactant-to-albumin (SA) ratio, which is a faster and simpler test, although there is not good concordance between the two measurements (6). These traditional methods of evaluating fetal lung maturity have a number of disadvantages. Analysis of amniotic fluid requires amniocentesis, with the associated risks of this invasive procedure including infection and miscarriage (7,8). The tests are also associated with a high rate of false-positive results in fetuses with intermediate lung maturity, even when several different measurements are incorporated into a more comprehensive assessment (9–11). In these cases the laboratory tests can indicate fetal lung maturity yet the fetus may still develop RDS (12). Choline, a major component of surfactant, can be easily detected and quantified by 1H MR spectroscopy ex vivo. Case reports of 1H MRS of amniotic fluid pockets in utero have generated interest in pursuing this technique for the potential noninvasive evaluation of fetal lung maturity in vivo (13–15). However, in vivo studies are lower in spectroscopic resolution and sensitivity, and are expensive and technically challenging to perform (16). Accordingly, a systematic study of amniotic fluid samples ex vivo would be useful to establish metabolic markers of fetal lung maturity (such as choline) and provide proof of concept to justify pursuing in vivo protocol development. High-resolution magic angle spinning (HR-MAS) spectroscopy is an ex vivo MRS technique that can be applied to intact tissues, cells, and biofluids. Although HR-MAS spectroscopy is normally used to improve spectral resolution, in the case of amniotic fluid, which contains mostly water, the small sample volume relative to the coil size allows for much better water suppression than a conventional liquids probe. Because reproducibility of measurements is an important fundamental parameter that needs to be established in such studies, the reproducibility of the MRS quantification technique first needs to be evaluated. Thus, the purpose of this study was to determine the intra- and interobserver reproducibility of metabolite concentration measurements (including choline concentration) detectable by 1H HR-MAS spectroscopy using a semiautomated software program for peak fitting.

Fetal urinary tract obstruction with oligohydramnios produces pulmonary hypoplasia and renal dysplasia. Decompression of the obstructed urinary tract may restore amniotic fluid and allow lung growth, but transabdominal catheter shunt decompression is often inadequate and does not allow for cycling of the bladder, while open procedures cause significant maternal morbidity. Disruption of the anatomic obstruction, usually posterior urethral valves in a male fetus, would be ideal but has proven technically difficult. Here we describe a new technique of percutaneous fetal cystoscopy and disruption of posterior urethral valves, and the case report of our first application of this technique. We pre-sent a case of a 17-week male fetus with posterior urethral valves which underwent fetal cystoscopy for mechanical disruption of posterior urethral valves. This minimally invasive approach to disruption of posterior urethral valves in a fetus is a novel method for decompressing the urinary tract. The technique offers a minimal degree of maternal morbidity and, if instituted early enough, can restore amniotic fluid volume, avert fatal pulmonary hypoplasia and may preserve renal function.

Background/Purpose Surgical intervention that improves pancreatic ductal drainage is a reasonable treatment strategy for recurrent pancreatitis in children. Methods This study was approved by the Committee on Human Research (San Francisco, CA). A retrospective chart review was performed on children aged 0 to 17 years given the International Classification of Diseases, Ninth Revision coding diagnosis of chronic pancreatitis who underwent surgical intervention from 1981 to 2005. Results From 1981 to 2005, 32 children were treated for the diagnosis of chronic pancreatitis. The etiologies were obstructive (n = 13), idiopathic (n = 10), hereditary (n = 6), medications (n = 2), and infection (n = 1). Fifteen patients underwent 17 operations for chronic pancreatitis, including Puestow (n = 9), cystenterostomy (n = 2), Whipple (n = 1), distal pancreatectomy (n = 1), Frey (n = 1), DuVal (n = 1), excision of enteric duplication cyst (n = 1), and pancreatic ductal dilation (n = 1). The mean age at presentation of patients undergoing surgery was 6.0 ± 4.1 years (mean ± SD). The mean time from presentation to operation was 3.3 ± 3.3 years. There were no deaths after surgical intervention. Of 15 patients, 2 (13%) required rehospitalization within 90 days of surgery, one for bowel obstruction, the other for splenic infarction. The median length of stay postoperatively was 8 days (range, 5-66 days). Conclusions Chronic pancreatitis in children differs markedly in etiology when compared with adults. In most cases seen in our institution, chronic pancreatitis resulted from ineffective ductal drainage. These disorders are amenable to surgical decompression, which, ultimately, can prevent disease recurrence.

Polybrominated diphenyl ethers (PBDEs) are used as flame retardants in furniture foam, electronics, and other home furnishings. A field study was conducted that enrolled 139 households from California, which has had more stringent flame retardant requirements than other countries and areas. The study collected passive air, floor and indoor window surface wipes, and dust samples (investigator collected using an HVS3 and vacuum cleaner) in each home. PentaBDE and BDE209 were detected in the majority of the dust samples and many floor wipe samples, but the detection in air and window wipe samples was relatively low. Concentrations of each PBDE congener in different indoor environmental media were moderately correlated, with correlation coefficients ranging between 0.42 and 0.68. Correlation coefficients with blood levels were up to 0.65 and varied between environmental media and age group. Both investigator-collected dust and floor wipes were correlated with serum levels for a wide range of congeners. These two sample types also had a relatively high fraction of samples with adequate mass for reliable quantification. In 42 homes, PBDE levels measured in the same environmental media in the same home 1year apart were statistically correlated (correlation coefficients: 0.57-0.90), with the exception of BDE209 which was not well correlated longitudinally.

Background Polybrominated diphenyl ethers (PBDEs) are used as flame retardants in many household items. Given concerns over their potential adverse health effects, we identified predictors and evaluated temporal changes of PBDE serum concentrations. Methods PBDE serum concentrations were measured in young children (2-8 years old; N = 67), parents of young children (

Foregut duplications are rare entities that include both esophageal and bronchogenic cysts. The diagnosis of foregut duplication cyst is made most often from an incidental finding on chest radiograph, or due to respiratory compromise due to mass effect or infection. Treatment consists of complete resection. Recurrences are associated with incomplete resection. Six cases of foregut duplication cysts are presented that were resected thoracoscopically.From May 1998 to April 2003, six patients underwent thoracoscopy for resection of foregut duplication cyst. One patient required conversion to open thoracotomy due to esophageal perforation. The distribution of cysts was 4 on the left and 2 on the right; all procedures were performed with three or four ports. Single lung ventilation was used in three patients. The masses were removed via a port site after intrathoracic decompression. Chest tubes were placed in all patients, and most were removed within 12 hours.Five of six cases underwent successful thoracoscopic resection. Pathology demonstrated esophageal duplication cyst in three patients and bronchogenic cyst in the other three patients. Average hospital stay was 5.5 days. Complications included aspiration pneumonia and chest tube dislodgment. There were no deaths, and no recurrences.Thoracoscopic resection is a safe and effective method of treating foregut duplications. Outcomes have been good with little short-term morbidity and no mortality. Morbidity and cosmesis are improved by avoiding thoracotomy. Thoracoscopic resection should be considered the first-line therapy for these benign masses.

Background/Purpose Amniocentesis is an invasive procedure with inherent risks. Magnetic resonance (MR) spectroscopy is a safe noninvasive way of measuring levels of choline-containing compounds (including surfactant) and other metabolites. The purpose of this study was to test the feasibility of assessing fetal lung maturity in vivo and ex vivo using MR spectroscopy to determine differences in amniotic fluid choline concentrations between the second and third trimesters. Methods Magnetic resonance spectroscopy was performed on ex vivo samples of amniotic fluid from second- and third-trimester fetuses. In vivo MR spectroscopy was performed on amniotic fluid and fetal lungs in third-trimester fetuses. Spectral acquisition and analysis were performed by an attending radiologist in conjunction with an MR spectroscopist. Results Choline-containing compounds were observed from 3.20 to 3.25 ppm. Comparison of spectra from second- and third-trimester amniocentesis revealed a trend toward increased choline at later gestational ages. Spectra from amniotic fluid and lungs of a third-trimester fetus showed that choline can be detected in the in vivo setting. Conclusions Magnetic resonance spectroscopy is a safe noninvasive procedure that enables measurement of choline-containing compounds in fetal lung and amniotic fluid. Magnetic resonance spectroscopy shows a trend toward an increased quantity of choline in third- vs second-trimester amniocentesis.