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Purpose To report outcomes of sutured and sutureless closure for gastroschisis across a large multi-institutional cohort. Methods A retrospective study of infants with uncomplicated gastroschisis at 11 children's from 2014 to 2016 was performed. Outcomes of sutured and sutureless abdominal wall closure were compared. Results Among 315 neonates with uncomplicated gastroschisis, sutured closure was performed in 248 (79%); 212 undergoing sutured closure after silo and 36 undergoing primary sutured closure. Sutureless closure was performed in 67 (21%); 37 primary sutureless closure, 30 sutureless closure after silo placement. There was no significant difference in gestational age, gender, birth weight, total days on TPN, and time from closure to initial oral intake or goal feeds. Sutureless closure patients had less general anesthetics, ventilator use/time, time from birth to final closure, antibiotic use after closure, and surgical site/deep space infections. Subgroup analysis demonstrated primary sutureless closure had less ventilator use and anesthetics than primary sutured closure. Sutureless closure after silo led to less ventilator use/time, anesthetics, and antibiotics compared to those with sutured closure after silo. Conclusion Sutureless abdominal wall closure of neonates with gastroschisis was associated with less general anesthetics, antibiotic use, surgical site/deep space infections, and decreased ventilator time. These findings support further prospective study by our group. Level of Evidence Level III.

Purpose The purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM). Methods We performed a multi-institutional cohort study of children born with ARM in 2007–2011 who had spinal and sacral imaging. Questions from the Baylor Social Continence Scale were used to assess fecal continence at the age of ≥ 4 years. Factors present at birth that predicted continence were identified using multivariable logistic regression. Results Among 144 ARM patients with a median age of 7 years (IQR 6–8), 58 (40%) were continent. The rate of fecal continence varied by ARM subtype (p = 0.002) with the highest rate of continence in patients with perineal fistula (60%). Spinal anomalies and the lateral sacral ratio were not associated with continence. On multivariable analysis, patients with less severe ARM subtypes (perineal fistula, recto-bulbar fistula, recto-vestibular fistula, no fistula, rectal stenosis) were more likely to be continent (OR = 7.4, p = 0.001). Conclusion Type of ARM was the only factor that predicted fecal continence in children with ARM. The high degree of incontinence, even in the least severe subtypes, highlights that predicting fecal continence is difficult at birth and supports the need for long-term follow-up and bowel management programs for children with ARM. Type of Study Prospective Cohort Study. Level of Evidence II.

Prior data suggest that infants with gastroschisis are at high risk for hypothermia and infectious complications (ICs). This study evaluated the associations between perioperative hypothermia (PH) and ICs in gastroschisis using a multi-institutional cohort.Retrospective review of infants with gastroschisis who underwent abdominal closure from 2013-2017 was performed at 7 children's hospitals. Any-IC and surgical site infection (SSI) were stratified against the presence or absence of PH, and perioperative characteristics associated with PH and SSI were determined using multivariable logistic regression.Of 256 gastroschisis neonates, 42% developed PH, with 18% classified as mild hypothermia (35.5-35.9 °C), 10.5% as moderate (35.0-35.4 °C), and 13% severe (35 °C). There were 82 (32%) ICs with 50 (19.5%) being SSIs. No associations between PH and any-IC (p = 0.7) or SSI (p = 0.98) were found. Pulmonary comorbidities (odds ratio (OR)=3.76, 95%CI:1.42-10, p = 0.008) and primary closure (OR=0.21, 95%CI:0.12-0.39, p0.001) were associated with PH, while silo placement (OR=2.62, 95%CI:1.1-6.3, p = 0.03) and prosthetic patch (OR=3.42, 95%CI:1.4-8.3, p = 0.007) were associated with SSI on multivariable logistic regression.Primary abdominal closure and pulmonary comorbidities are associated with PH in gastroschisis, however PH was not associated with increased risk of ICs. Independent risk factors for SSI include silo placement and prosthetic patch closure.

Introduction Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol. Materials and Methods A retrospective study of neonates with uncomplicated gastroschisis at 11 children's hospitals from 2013 to 2016 was performed.Outcomes of infants fed via institutional-specific protocols were compared with those fed without a protocol. Subgroup analyses of protocol use with immediate versus delayed closure and with sutured versus sutureless closure were conducted. Results Among 315 neonates, protocol-based feeding was utilized in 204 (65%) while no feeding protocol was used in 111 (35%). There were less surgical site infections (SSI) in those fed with a protocol (7 vs. 16%, p = 0.019). There were no differences in TPN duration, time to initial oral intake, time to goal feeds, ventilator use, peripherally inserted central catheter line deep venous thromboses, or length of stay. Of those fed via protocol, less SSIs occurred in those who underwent sutured closure (9 vs. 19%, p = 0.026). Further analyses based on closure timing or closure method did not demonstrate any significant differences. Conclusion Across this multi-institutional cohort of infants with uncomplicated gastroschisis, there were more SSIs in those fed without an institutional-based feeding protocol but no differences in other outcomes.

To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling. A retrospective study of 394 neonates with gastroschisis at 11 children’s hospitals from January 2013 to March 2017 was performed. Analysis by Fisher’s exact tests and Wilcoxon rank sum tests were performed. Outcomes of complex and simple gastroschisis are reported. There were 315 (80%) SG and 79 (20%) CG. CG had increased time from birth to closure (6 vs 4.4 days), closure to goal feeds (69 vs 23 days), ventilator use (90% vs 73%), SSIs (31% vs 11%), NEC (14% vs 6%), PN use (71 vs 24 days), LOS (104.5 vs 33 days), and mortality (11% vs 0%). This study provides generalizable estimates for expected outcomes of patients with both SG and CG that can be utilized during counseling. CG has significantly worse in-hospital outcomes.

This study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM).We performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 children's hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed.Among 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥1 abnormal finding on any screening test varied by type of ARM (p0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%).Screening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients.Multi-institutional retrospective cohort study.III.

Background/purpose Elective preterm delivery (EPD) of a fetus with gastroschisis may prevent demise and ameliorate intestinal injury. While the literature on optimal timing of delivery varies, we hypothesize that a potential benefit may be found with EPD. Methods A meta-analysis of publications describing timing of delivery in gastroschisis from 1/1990 to 8/2016 was performed, including studies where either elective preterm delivery (group 1, G1) or preterm gestational age (GA) (group 2, G2) were evaluated against respective comparators. The following outcomes were analyzed: total parenteral nutrition (TPN), first enteral feeding (FF), length of stay, ventilator days, fetal demise, complex gastroschisis, sepsis, and death. Results Eighteen studies describing 1430 gastroschisis patients were identified. G1 studies found less sepsis ( p p =0.03), and 11days less of TPN ( p =0.07) in the preterm cohort. Comparatively, G2 studies showed less days to FF in term GA ( p =0.02).Whereas G1 BWs were similar, G2 preterm had a significantly lower BW compared to controls ( p =0.001). Conclusions Elective preterm delivery appears favorable with respect to feeding and sepsis. However, benefits are lost when age is used as a surrogate of EPD. A randomized, prospective, multi-institutional trial is necessary to delineate whether EPD is advantageous to neonates with gastroschisis. Type of study Treatment study. Level of evidence Level III.

Perioperative hypothermia decreases nutrient and oxygen delivery to tissues and, in adult studies, increases the risk of infectious complications (ICs). Gastroschisis (GS) places newborns at risk for hypothermia by nature of exposed viscera and excessive heat loss. Although hypothermia is a known cause of mortality in GS, the rate of ICs in this at-risk cohort has not yet been delineated.A retrospective cohort study was performed at our single tertiary-referral hospital, evaluating patient and operative characteristics of all GS infants who underwent operative closure. Intraoperative temperatures were recorded, defining hypothermia as mild (35.5°C-35.9°C), moderate (35.0°C-35.4°C), or severe (35°C). Temperature nadirs, procedural and anesthesia duration were observed. The primary outcome was 30-d surgical site infections. Secondary measures included other ICs.Among 43 GS neonates, 21 (48.8%) had intraoperative hypothermia, classified as mild in 2 (4.7%), moderate in 8 (18.6%), and severe in 11 (25.6%). Nineteen ICs occurred in 35.9% of patients, including 10 (23.3%) surgical site infections. There was no association between hypothermia and ICs. Patient and operative characteristics were similar between normothermic and hypothermic groups, except that normothermic infants were more likely to have silos placed with delayed closure than hypothermic patients (63.6% versus 23.8%, P = 0.01).Infants with GS are at high risk for hypothermia and ICs, though newborns with silos were less subject to temperature lability. A multiinstitutional study with greater power is needed to further investigate the relationship between perioperative hypothermia and surgical ICs.

Introduction Isomerism, or heterotaxy syndrome, affects many organ systems anatomically and functionally. Intestinal malrotation is common in patients with isomerism. Despite a low reported risk of volvulus, some physicians perform routine screening and prophylactic Ladd procedures on asymptomatic patients with isomerism who are found to have intestinal malrotation. The primary aim of this study was to determine if isomerism is an independent risk factor for volvulus. Methods Kid's Inpatient Database data from 1997 to 2012 was utilized for this study. Characteristics of admissions with and without isomerism were compared with a particular focus on intestinal malrotation, volvulus, and Ladd procedure. A logistic regression was conducted to determine independent risk factors for volvulus with respect to isomerism. Results 15,962,403 inpatient admissions were included in the analysis, of which 7970 (0.05%) patients had isomerism, and 6 patients (0.1%) developed volvulus. Isomerism was associated with a 52-fold increase in the odds of intestinal malrotation by univariate analysis. Of 251 with isomerism and intestinal malrotation, only 2.4% experienced volvulus. Logistic regression demonstrated that isomerism was not an independent risk factor for volvulus. Conclusion Isomerism is associated with an increased risk of intestinal malrotation but is not an independent risk factor for volvulus. Type of Study Prognosis study. Level of Evidence Level III.

Introduction Venous thromboembolism (VTE) is increasingly prevalent in injured children admitted to the intensive care unit (ICU). Few data exist to support VTE pharmacologic prophylaxis or ultrasound (US) surveillance in children with high bleeding risk. After implementation of screening US guidelines, we sought to describe our experience, hypothesizing that screening US of children at highest risk for VTE results in earlier detection and management. Study design A retrospective analysis was conducted on prospectively collected data of injured children admitted to an American College of Surgeons Verified level 1 Pediatric Trauma Center from 2010 to 2015. In patients at high risk for both VTE and bleeding (HRHR), guidelines recommended deferral of pharmacologic prophylaxis and a screening US at ≥ 7 ICU days if bleeding risk remained. Outcomes analyzed included VTE rates, guideline compliance, and US timing. The rate of deep vein thrombosis (DVT) detection (number of DVT captured/number of US obtained) was examined. Results Of 4061 trauma patients, 588 (14.5%) were critically injured including 112 patients who met HRHR criteria. The rate of VTE in the HRHR group ≥ 7 ICU days was 25% (14/56). Of 23 VTE diagnosed in the ICU, 17 were detected by 49 US performed (34.7%), with the remaining 6 diagnosed by computed tomography. DVT was detected earlier than the US guideline recommended 7 days, independent of symptoms. Guideline compliance was 86%. Conclusion Critically injured children at risk for bleeding frequently develop VTE. Surveillance ultrasound in patients at high risk for both VTE and bleeding allows earlier detection and treatment. Level of Evidence Therapeutic study, level II.

This study examines the association between time to appendectomy and risk of complicated appendicitis and postoperative complications.

Background Anastomotic stricture is the most common postoperative complication in infants undergoing repair of esophageal atresia with or without tracheoesophageal fistula (EA/TEF). Stricture indices (SIs) are used to predict infants at risk for stricture requiring dilation. We sought to determine the most accurate SI and optimal timing for predicting anastomotic dilation. Materials and methods A retrospective study of infants undergoing repair of EA/TEF between 2008 and 2013 was performed. Esophagrams were used to calculate four SIs (upper pouch esophageal anastomotic stricture index [U-EASI], lower pouch esophageal anastomotic stricture index [L-EASI], lateral SI, and anterior/posterior SI). The best performing SI was identified. Logistic regression analysis was performed to determine if a first or second esophagram SI threshold was associated with dilation. A receiver operating characteristic curve measured the accuracy of the model using SIs to predict dilation. Results Of 45 EA/TEF infants included, 20 (44%) had postoperative strictures requiring dilation. As the best performing SI, logistic regression analysis showed that U-EASI as a continuous variable was predictive of dilation ( P = 0.03) but was not significant at U-EASI ≤ 0.37. However, U-EASI ≤ 0.37 was associated with needing earlier dilation. On second esophagram (median, 38 days), U-EASI of ≤0.39 was significantly associated with dilation (OR: 7.8, 95% CI: 1.05-57.58, P = 0.04). The area under the receiver operating characteristic curve of the U-EASI model controlling for days to esophagram demonstrated improved predictive ability from first (AUC 0.73) to second esophagram (AUC 0.81). Conclusions Calculation of the SI utilizing a U-EASI ≤ 0.39 on the delayed esophagram is associated with future anastomotic dilation. A multi-institutional study is necessary to confirm the predictive ability of the U-EASI.

Comparative outcomes of enhanced percutaneous endoscopic gastrostomy (PEG) and laparoscopic gastrostomy (LG) have not been elucidated in infants. We describe the outcomes and procedural episodic expenditures of PEG versus LG in this high-risk population.One hundred eighty-three gastrostomies in children under 1year were reviewed from our institution spanning 1/2011-6/2015. Pertinent demographics and 3-month complications (mortality, gastrocolic fistula, reoperation, cellulitis, granulation, pneumonia, and tube dislodgement6weeks) were collected. Facility and professional administrative data was used to conduct a charge and cost analysis of PEG and LG procedures as well as their statistically significant complications.Seventy-eight PEG and 105 LG infants were compared. LG infants were significantly younger, had higher ASA class, and increased frequency of cardiopulmonary disease. Significant major complications included a 3.8% incidence of gastrocolic fistula among PEGs (3.8% vs 0%, p=0.04) and 7.6% early tube dislodgements among LG infants (0 vs. 7.6%, p=0.01), resulting in $86,896 of additional charges with PEG complication. Incorporating complication frequency, average charges and variable cost per case were $8964 and $253 greater using PEG.Despite a healthier cohort, infants undergoing enhanced PEG have more morbid and costly complications. LG may be the less burdensome approach to gastrostomy in infants.Case-Control Study/Retrospective Comparative Study - Level III.

Skeletal muscle contractility and myosin function decline following ovariectomy in mature female mice. In the present study we tested the hypothesis that estradiol replacement can reverse those declines. Four-month-old female C57BL/6 mice ( n = 69) were ovariectomized (OVX) or sham operated. Some mice were treated immediately with placebo or 17β-estradiol (OVX + E2) while other mice were treated 30 days postsurgery. Thirty or sixty days postsurgery, soleus muscles were assessed in vitro for contractile function and susceptibility to eccentric contraction-induced injury. Myosin structural dynamics was analyzed in extensor digitorum longus (EDL) muscles by electron paramagnetic resonance spectroscopy. Maximal isometric tetanic force was affected by estradiol status ( P < 0.001) being ∼10% less in soleus muscles from OVX compared with sham-operated mice [168 mN (SD 16.7) vs. 180 mN (SD 14.4)] and was restored in OVX + E2 mice [187 mN (SD 17.6)]. The fraction of strong-binding myosin during contraction was also affected ( P = 0.045) and was ∼15% lower in EDL muscles from OVX compared with OVX + E2 mice [0.263 (SD 0.034) vs. 0.311 (SD 0.022)]. Plasma estradiol levels were correlated with maximal isometric tetanic force ( r = 0.458; P < 0.001) and active stiffness ( r = 0.329; P = 0.044), indicating that circulating estradiol influenced muscle and myosin function. Estradiol was not effective in protecting muscle against an acute eccentric contraction-induced injury ( P ≥ 0.401) but did restore ovariectomy-induced increases in muscle wet mass caused by fluid accumulation. Collectively, estradiol had a beneficial effect on female mouse skeletal muscle.

Background The management of intestinal rotational abnormalities (IRA) in heterotaxy syndrome (HS) remains inconsistent. Because of the high incidence of malrotation in HS, screening of asymptomatic patients is standard of care in some institutions. The Ladd procedure is the treatment for malrotation, and has been reported to have high complication rates in HS patients. Methods We performed a systematic review of publications describing IRA in pediatric HS patients from January 1993 to present. The incidence of volvulus on surgical exploration was determined. Perioperative and long-term outcomes were analyzed to determine complication and mortality rates. Results Eleven retrospective studies describing 649 HS patients were identified. Of all patients with HS, 27% (176/649) underwent Ladd procedure. Only 1.2% (8/649) of HS patients included had volvulus. Postoperative complications occurred in 25 patients (14%), including a 10% incidence of small bowel obstruction. Perioperative and overall mortality rates after Ladd procedure were 3% and 21%, respectively. Six studies described mesenteric width, reporting 43% to have narrow mesentery. Conclusion The Ladd procedure is not without significant morbidity and mortality in heterotaxy patients. Further prospective studies should investigate predictors of mesenteric width to spare the unnecessary morbidity of surgery in patients who are at low risk for volvulus.

The purpose of this study was to determine the extent to which hindlimb muscles of mdx mice adapt to a voluntary endurance type of exercise. mdx and C57BL mice engaged in 8 weeks of wheel running or maintained normal cage activities. Beneficial adaptations that occurred in mdx mice included changes in muscle mass, fiber size, and fiber types based on myosin heavy chain (MHC) isoform expression. These adaptations occurred without increases in fiber central nuclei and embryonic MHC expression. An undesirable outcome, however, was that muscle mitochondrial enzyme activities did not improve with exercise in mdx mice as they did in C57BL mice. Cellular remodeling of dystrophic muscle following exercise has not been studied adequately. In this study we found that some, but not all, of the expected adaptations occurred in mdx mouse muscle. We must better understand these (non)adaptations in order to inform individuals with DMD about the benefits of exercise.