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Mycotic aneurysm formation in a visceral artery carries a significant risk of mortality and morbidity. The authors present a case of a symptomatic superior mesenteric artery aneurysm secondary to a septic embolus in a patient who had undergone aortic valve replacement. The patient initially presented with evidence of acute intestinal ischemia from a presumed embolic source. Although an extensive bowel resection was performed, an adequate search for the embolus was not carried out. Prompt diagnosis and removal of suspected septic emboli must be performed to avoid the formation of delayed mycotic aneurysms. [ABSTRACT FROM AUTHOR]

Compassionate care of the surgical patient recognizes the wholeness of each individual. Patients and their caregivers come to healthcare providers with the hope of relief from pain and suffering and aspirations for the potential to feel well or be "normal" again. Many lean on their personal faith and prayer for spiritual comfort and petitions for healing. We discuss a case in which prayer is incorporated into the surgical Time Out, a scenario not uncommon in faith-based hospitals, and offer a framework to evaluate the practice that incorporates ethical principles of beneficence, non-maleficence, patient/parental autonomy, justice, and the fiduciary responsibility of the healthcare provider., (© 2024 International Society of Surgery/Société Internationale de Chirurgie (ISS/SIC).)

Introduction: Reducing soybean lipid emulsion (SLE) dose may prevent parenteral nutrition-associated cholestasis (PNAC) but effects on growth and neurodevelopment are unknown. The purpose of this study was to evaluate the effect of reduced dose SLE on growth and neurodevelopment., Methods: Surgical neonates at 4 centers were randomized to standard SLE (3 g/kg/day) or reduced SLE (1 g/kg/day) over a 12-week period. Bilirubin levels and growth parameters were measured baseline and weekly while on study. The effects of time and group on direct bilirubin and growth were evaluated with a linear mixed effects model. Neurodevelopmental outcomes were assessed at 12- and 24-months corrected gestational age., Results: Twenty-one individuals were randomized (standard dose = 9, reduced dose = 12). Subjects in the reduced dose group had slower rates of direct bilirubin increase and overall levels decreased earlier than those in the standard dose group. There was a trend toward a faster direct bilirubin decrease in the reduced dose group (p = 0.07 at day 84). There were no differences in the rates of change in weight (p = 0.352 at day 84) or height Z-scores (p = 0.11 at day 84) between groups. One subject in the reduced dose group had abnormal neurodevelopmental testing at 24 months., Conclusions: Surgical neonates randomized to a reduced dose of SLE had improved trends in direct bilirubin levels without clinically significant differences in overall growth and neurodevelopment., Type of Study: Randomized Controlled Trial., Level of Evidence: II., Competing Interests: Conflicts of interest The authors have no competing financial interests to disclose., (Copyright © 2024. Published by Elsevier Inc.)

More knowledge is needed about long-term ADHD medication and symptom, daily functioning, comorbidity, and tolerability outcomes. This "Long-term Medication for ADHD (LMA) trial" was a prospective observational 2-year trial in children and adolescents aged 6-18 years (extension of 1-year trial). Participants met criteria for DSM-5 ADHD (inattentive or combined), with complex comorbidities; autism spectrum disorder (31%), autistic traits (24%), oppositional symptoms (59%), anxiety (32%), dyslexia/language disorder (16%), borderline intellectual functioning (17%). Medication was individually tailored and followed-up at clinical visits (1, 2, 3, 6, 12, 18, 24 months). Primary outcome: Clinical Global Impression-Severity and Improvement scales (CGI-S, CGI-I). Secondary outcomes: Investigator-rated ADHD-Rating Scale, Weiss Functional Impairment Rating Scale-Parent report (WFIRS-P; Family, School Learning and Behavior, Life Skills, Self-Concept, Social Activities, and Risky Activities domains), comorbidity symptoms and adverse events (AEs). One hundred twenty-eight participants were enrolled (1-year trial only n = 27, LMA trial n = 101). Of these 29 (23%) discontinued, mainly due to AEs (n = 7), moving (n = 7), or no longer needing medication (n = 6). Main AEs were poor appetite, low mood, anxiety, irritability, fatigue. Improvements from baseline to 2 years were large in CGI-S (effect size (ES) 2.28), ADHD-RS (ES 2.06), and moderate to large in WFIRS-P (ES total 0.73, learning 0.4, family 0.67). Overall, the trial showed robust and sustained improvements in ADHD symptom severity and daily functioning over a period of 2 years of ADHD medication in children and adolescents with ADHD and complex comorbidities. Most AEs were mild. Comorbidity symptoms were improved after 1 year, particularly oppositional symptoms, depression, and anxiety., (© 2024. The Author(s).)

Background: Pleural drainage following lung resection is almost universally practiced in pediatric surgery, but its necessity has been questioned in adult literature. We performed a cross-sectional study of pediatric patients undergoing lung resection to characterize chest tube (CT) practices and clarify their utility., Method: Retrospective chart review of patients <21 years of age undergoing pulmonary lobectomy or wedge resection at an academic children's hospital from 2013 to 2022. Variables regarding demographics and post-operative CT management were recorded., Results: 130 procedures meet inclusion criteria: 59 lobectomies (group 1), 19 diagnostic wedges (group 2), and 52 excisional wedges (group 3). 74.6% of group 1 patients had no air leak, and median CT duration was 2 days. In group 2, 89.5% had no air leak and median CT duration was 1 day. In Group 3, 80.8% had no air leak and median CT duration was 1 day. Overall, 43.1% patients had their CT removed on post-operative day 1 and 21.5% on post-operative day 2., Conclusion: CT duration following lung resection in pediatric patients is typically brief, with most patients having no air leak and CT removal within 2 days of surgery. Obligatory CT drainage may not be necessary in select patients undergoing lung resection., Level of Evidence: Level IV., Type of Study: Retrospective Study., (Copyright © 2023. Published by Elsevier Inc.)

More than twenty years ago, the Accreditation Council for Graduate Medical Education and the American Board of Medical Specialties began the conversion of graduate medical education from a structure- and process-based model to a competency-based framework. The educational outcomes assessment tool, known as the Milestones, was introduced in 2013 for seven specialties and by 2015 for the remaining specialties, including pediatric surgery. Designed to be an iterative process with improvements over time based on feedback and evidence-based literature, the Milestones started the evolution from 1.0 to 2.0 in 2016. The formation of Pediatric Surgery Milestones 2.0 began in 2019 and was finalized in 2021 for implementation in the 2022-2023 academic year. Milestones 2.0 are fewer in number and are stated in more straightforward language. It incorporated the harmonized milestones, subcompetencies for non-patient care and non-medical knowledge that are consistent across all medical and surgical specialties. There is a new Supplemental Guide that lists examples, references and links to other assessment tools and resources for each subcompetency. Milestones 2.0 represents a continuous process of feedback, literature review and revision with goals of improving patient care and maintaining public trust in graduate medical education's ability to self-regulate. LEVEL OF EVIDENCE: V., Competing Interests: Declaration of Competing Interest None to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Medical and surgical care for children with intestinal failure has evolved so that long-term life expectancy is common even in the setting of the shortest bowel lengths. The long-term administration of parenteral nutrition has become safe with alterations in lipid formulation, and the risk of liver injury has been dramatically reduced. Well-established techniques for bowel lengthening and tapering exist to increase the absorptive capacity of the remnant bowel. These advances allow for ongoing intestinal rehabilitation in the child with the ultimate goal of enteral autonomy while the use of intestinal transplantation in this population has declined in recent years., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Recent studies have focused on parent-reported health-related quality of life (HRQOL) in children with intestinal failure (IF). However, there is a paucity of data on HRQOL from the perspective of the child with IF., Methods: A prospective study of child self-reported HRQOL was performed in a regional intestinal rehabilitation program from 2015 to 2019. The PedsQL 4.0 Generic Core Scales were administered annually to children with IF ages five years and older along with their parents. Survey data was stratified by age and compared with parent-proxy scores and reference populations of healthy and chronically ill children. Linear mixed-effect models were constructed to identify associations with child self-reported HRQOL., Results: A total of 140 surveys were administered to 69 children and their parents. Median child age at survey was 8 (IQR 6-10) years. Child self-reported HRQOL scores increased with each increasing age range. Children reported higher HRQOL scores compared to parent-proxy data in all age groups. Children with IF had lower HRQOL scores compared to healthy children in all survey dimensions (p < 0.001) and to children with chronic illness in the school and social functioning dimensions (p < 0.05). In adjusted analysis, longer remnant bowel length was independently associated with decreased HRQOL scores in children (p < 0.05)., Conclusions: Children with IF reported better HRQOL compared to parent-proxy data. While these HRQOL scores improved with age, they remain significantly lower than healthy and chronically ill peers. The association between bowel length and child-reported HRQOL deserves further investigation., Level of Evidence: Level II., (Copyright © 2021. Published by Elsevier Inc.)

Background: Due to altered nutrition regimens and complex medical needs, pediatric intestinal failure (IF) may have a powerful impact on health-related quality of life (HRQOL). Studies have shown that children with IF experience lower HRQOL. Data on the HRQOL of families of children with IF are lacking., Methods: We performed a prospective analysis of the HRQOL of families of children with IF in a regional intestinal rehabilitation program from 2011 to 2018. The Pediatric Quality of Life Family Impact Module (FIM) was administered annually to parents. FIM scores were regressed on risk factors using linear mixed-effect models that accounted for repeated surveys within families., Results: A total of 117 families completed 272 surveys. FIM scores increased with patient age across nearly all survey dimensions. Total FIM scores were lower when compared to families of healthy children (median differences = -5, P = .01) and similar to families of chronically ill children. While IF families reported major deficits in the Communication (-11, P < .001) and Worry (-17, P < .001) dimensions, they also reported higher Family Relationship scores (+7, P < .01). On multivariable regression, presence of a major comorbidity and four or more hospital admissions in the prior year were associated with lower family HRQOL (P < .05). Parenteral nutrition dependence was independently associated with lower scores in the Communication (-7, P = .03) and Daily Activities (-10, P = .02) dimensions., Conclusion: Families of children with IF experience a decreased HRQOL that may improve with patient age. Intestinal rehabilitation programs should address the HRQOL of families in addition to patients., (© 2021 American Society for Parenteral and Enteral Nutrition.)

Background: Infants with gastroschisis require prolonged hospitalization for surgical repair and gradual advancement of feeds. The present study explores the effect of a change in a protocolized enteral feeding regimen with length of hospital stay (LOS) and total costs in newborns with gastroschisis., Methods: A retrospective review was performed in neonates with uncomplicated gastroschisis at a free-standing pediatric institution from 2012 to 2020. The effect of two different enteral feed advancement protocols on clinical outcomes and hospital costs was analyzed., Results: Seventy-four patients were identified, of which 50 (68%) underwent 10 ml/kg/day feeding advancements, and 24 (32%) underwent 20 ml/kg/day feeding advancements. Compared to neonates who underwent 10 ml/kg/day enteral advancements, neonates receiving 20 ml/kg/day advancements reached goal feeds faster (14 vs 20 days, p<0.001), were younger at goal feeds (26 vs 34 days, p = 0.001), required fewer days of parenteral nutrition (22 vs 29 days, p = 0.001), and had shorter LOS (30 vs 36 days, p = 0.001). On multivariable analysis, total costs decreased by 9.77% in the 20 ml/kg/day advancement cohort (p = 0.071)., Conclusion: In neonates with uncomplicated gastroschisis who underwent primary repair, a nutritional protocol that incorporated 20 ml/kg/day feeding advancements was safe and resulted in faster attainment of goal feeds and shorter LOS., Level of Evidence: II/III., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: Necrotizing enterocolitis (NEC) totalis is a devastating disease of the newborn intestine. A precise clinical definition of the extent of gastrointestinal involvement is lacking in the existing literature, and the clinical outcomes are typically viewed as grim., Methods: Herein, we present a series of clinical case examples of patients with varying degrees of NEC totalis and other co-morbid conditions, with possible anticipated outcomes based on current data., Results: We define the key ethical issues and provide a framework and discussion of the ethical issues involved in the care of patients with NEC totalis and recommendations of how to approach discussions with the family of these patients We discuss the ethical considerations for both the providers caring for these patients, and the patient's family members., Conclusion: The management of patients with NEC totalis is complex and ethically challenging., Level of Evidence: V., Competing Interests: Declaration of Competing Interest None, (Copyright © 2021. Published by Elsevier Inc.)

Peer review is an essential tool for institutions and providers to meet the modern goals of safety and quality in health care. It is a mechanism that leads to a just culture within a health care institution whereby errors and complications are considered products of the system rather than isolated actions by an individual. The benefits and potential drawbacks of peer review are outlined in this review with a special emphasis on the interface between peer review and principles of medical ethics. It is argued that peer review, in the ideal setting, is founded upon the principles of beneficence and justice, and to varying levels on non-maleficence and autonomy., (Copyright © 2021. Published by Elsevier Inc.)

Introduction: In March 2020, the COVID-19 pandemic threatened to overwhelm entire healthcare systems. Here we characterize changes in surgical volumes at a regional tertiary pediatric hospital during the early phase of the COVID-19 pandemic., Methods: Data on all procedures performed during the state-wide ban on elective procedures (March 19th, 2020 to May 18th, 2020) that required anesthesia involvement were collected retrospectively and compared to the same time period in 2019., Results: A total of 5785 procedures were performed: 4005 (69%) in 2019, and 1780 (31%) in 2020, representing a 55% decrease in total cases. The percentage decrease was disproportionate across surgical services. Add-on cases increased from 23% to 39%, and outpatient procedures decreased from 60% to 27%., Discussion: The ban on elective procedures during the COVID-19 pandemic resulted in a significant decrease in the volume of procedures performed at a tertiary pediatric hospital that differed among surgical services., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to disclose. Approval for this study was obtained from the Seattle Children’s Hospital Institutional Review Board., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Newborns with gastroschisis require appropriate fluid resuscitation but are also at risk for hyponatremia that may lead to adverse outcomes. The etiology of hyponatremia in gastroschisis has not been defined., Methods: Over a 24-month period, all newborns with gastroschisis in a free-standing pediatric hospital had sodium levels measured from serum, urine, gastric output, and the bowel bag around the eviscerated contents for the first 48 h of life. Total fluid intake and output were measured. Maintenance fluids were standardized at 120 mL/kg/day. Hyponatremia was defined as a serum sodium <132 mEq/L. A logistic regression model was created to determine independent predictors of hyponatremia., Results: 28 infants were studied, and 14 patients underwent primary closure. While serum sodium was normal in all patients at birth, 9 (32%) infants developed hyponatremia at a median of 17.4 h of life. On univariate analysis, hyponatremic babies had a greater net positive fluid balance (74.9 vs 114.7 mL/kg, p = 0.001) primarily due to a decrease in total fluid output (p = 0.05). On multivariable regression, a 10 mL/kg increase in overall fluid balance was associated with an increased risk of developing hyponatremia (OR 1.84 [1.23, 3.45], p = 0.016). No differences in the sodium content of urine, gastric, or bowel bag fluid were observed, and sodium balance was equivalent between cohorts., Discussion: Hyponatremia in babies with gastroschisis in the early postnatal period was associated with positive fluid balance and decreased fluid output. Prospective studies to determine the appropriate fluid resuscitation strategy in this population are warranted., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: SMOFlipid is a mixed-lipid emulsion approved for adults in the United States as an alternative to soybean oil-based lipid (SO). There are limited data on the use of SMOFlipid in pediatrics and its effect on the fatty acid (FA) profile. Our objective was to characterize changes in FA profile, liver function, and growth in pediatric patients with intestinal failure (IF), following transition from SO or a fish-oil (FO) and SO combination to SMOFlipid., Methods: A retrospective case series was conducted on pediatric parenteral nutrition-dependent IF patients transitioned to SMOFlipid. Demographics, anthropometrics, labs, and achievement of nutrition goals were assessed. Linear mixed-effect models assessed effects on FA levels and clinical outcomes., Results: One hundred thirty-nine FA panels were collected from 20 patients. Median SMOFlipid dose at study completion was 2 g/kg/d (interquartile range, 1.6-2). During the 1.5 years after SMOFlipid initiation, ω-6 FA increased to physiologic levels, arachidonic acid increased from 298 to 461 nmol/mL (P < .001), and linoleic acid increased from 1172 to 1922 nmol/mL (P < .001). ω-3 FA remained within normal limits. Body mass index z-score and length z-score increased, though no significant changes were found. In addition, no significant changes were found in mead acid, hepatic function, triene-to-tetraene ratio, or triglycerides., Conclusion: In 20 pediatric IF patients, SMOFlipid allowed greater ω-6 FA provision while maintaining ω-3 FA, hepatic function, and patient growth. This longitudinal study identified improved FA profile associated with SMOFlipid use in comparison with SO with or without FO., (© 2020 American Society for Parenteral and Enteral Nutrition.)

Background: In newborns with gastroschisis, both primary repair and delayed fascial closure with initial silo placement are considered safe with similar outcomes although cost differences have not been explored., Methods: A retrospective review was performed of newborns admitted with gastroschisis at a single center from 2011 to 2016. Demographic, clinical, and cost data during the initial hospitalization were collected. Differences between procedure costs and clinical endpoints were analyzed using multivariable linear regression adjusting for prematurity, complicated gastroschisis, and performance of additional operations., Results: 80 patients with gastroschisis met inclusion criteria. Rates of primary fascial, primary umbilical cord closure, and delayed closure were 14%, 65%, and 21%, respectively. Delayed closure was associated with an increase in total hospital costs by 57% compared to primary repair (p < 0.001). In addition, delayed closure was associated with increased total and NICU LOS (p < 0.05), parenteral nutrition duration (p = 0.02), ventilator days (p < 0.001), time to goal enteral feeds (p = 0.01), and all cost sub-categories except ward room costs (p < 0.01)., Conclusion: Delayed fascial closure was associated with significantly greater hospital costs during the index admission., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To evaluate disease-specific and age-related factors contributing to health-related quality of life (HRQOL). in children with intestinal failure., Study Design: A prospective study of HRQOL was performed in a regional intestinal rehabilitation program. Parent-proxy Pediatric Quality of Life Inventory surveys were administered annually to families of 91 children with intestinal failure over a 6-year period. Survey data was stratified by age and compared with pediatric HRQOL data in healthy and chronically ill populations. Linear mixed-effect models using multivariable regression were constructed to identify associations with HRQOL., Results: A total of 180 surveys were completed by 91 children and their families. HRQOL scores were lowest for children ages 5-7 years (P < .001) and 8-12 years (P < .01), and these changes were primarily related to school dimension scores. In multivariable regression, age of 5 years and older and developmental delay were independently associated with lower HRQOL scores. The trend toward lower HRQOL scores parallels reference data from healthy and chronically ill children, although patients with intestinal failure scored lower than both populations at school age., Conclusions: Children with intestinal failure experience lower parent-proxy HRQOL scores in the 5-7 and 8-12 year age groups primarily related to school dimension scores. Multicenter data to validate these findings and identify interventions to improve QOL for children with intestinal failure are needed., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: To assess providers' recommendations as to comfort care versus medical and surgical management in clinical scenarios of newborns with severe bowel loss and to assess how a variety of factors influence providers' decision making., Study Design: We conducted a survey of pediatric surgeons and neonatologists via the American Pediatric Surgical Association and American Academy of Pediatrics Section of Neonatal-Perinatal Medicine. We examined how respondents' recommendations were affected by a variety of patient and provider factors., Results: There were 288 neonatologists and 316 pediatric surgeons who responded. Irrespective of remaining bowel length, comfort care was recommended by 73% of providers for a premature infant with necrotizing enterocolitis and 54% for a full-term infant with midgut volvulus. The presence of comorbidities and earlier gestational age increased the proportion of providers recommending comfort care. Neonatologists were more likely to recommend comfort care than surgeons across all scenarios (OR, 1.45-2.00; P < .05), and this difference was more pronounced with infants born closer to term. In making these recommendations, neonatologists placed more importance on neurodevelopmental outcomes (P < .001), and surgeons emphasized experience with long-term quality of life (P < .001)., Conclusion: Despite a contemporary survival of >90% in infants with intestinal failure, a majority of providers still recommend comfort care in infants with massive bowel loss. Significant differences were identified in clinical decision making between surgeons and neonatologists. These data reinforce the need for targeted education on long-term outcomes in intestinal failure to neonatal and surgical providers., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Multidisciplinary intestinal failure programs have played a leading role in the improved outcomes observed in children with intestinal failure over the past two decades. These teams evolved from the world of transplantation and now provide comprehensive care of intestinal failure patients and their families. In addition, they provide the foundation for outcomes research and clinical trials in pediatric intestinal failure. The history and composition of multidisciplinary intestinal failure programs is outlined here with a particular emphasis on long-term patient outcomes as reported from the programs themselves. The care of children with intestinal failure has rapidly evolved over the past two decades. In the contemporary era, children with intestinal failure now have a favorable long-term prognosis, and survival is routinely greater than 90%. The improvement in outcomes in this population is secondary to a variety of advances including safe strategies to deliver chronic parenteral nutrition (PN), innovative bowel lengthening techniques, preservation of vascular access, and prevention of sepsis. However, the underlying driver of these advances in care is widely considered to be the advent of multidisciplinary intestinal failure and rehabilitation programs to manage these patients in a comprehensive fashion., (Copyright © 2018. Published by Elsevier Inc.)

Background: The advent of regional multidisciplinary intestinal rehabilitation programs has been associated with improved survival in pediatric intestinal failure. Yet, the optimal timing of referral for intestinal rehabilitation remains unknown. We hypothesized that the degree of intestinal failure-associated liver disease (IFALD) at initiation of intestinal rehabilitation would be associated with overall outcome., Methods: The multicenter, retrospective Pediatric Intestinal Failure Consortium (PIFCon) database was used to identify all subjects with baseline bilirubin data. Conjugated bilirubin (CBili) was used as a marker for IFALD, and we stratified baseline bilirubin values as CBili<2 mg/dL, CBili 2-4 mg/dL, and CBili>4 mg/dL. The association between baseline CBili and mortality was examined using Cox proportional hazards regression., Results: Of 272 subjects in the database, 191 (70%) children had baseline bilirubin data collected. 38% and 28% of patients had CBili >4 mg/dL and CBili <2 mg/dL, respectively, at baseline. All-cause mortality was 23%. On univariate analysis, mortality was associated with CBili 2-4 mg/dL, CBili >4 mg/dL, prematurity, race, and small bowel atresia. On regression analysis controlling for age, prematurity, and diagnosis, the risk of mortality was increased by 3-fold for baseline CBili 2-4 mg/dL (HR 3.25 [1.07-9.92], p=0.04) and 4-fold for baseline CBili >4 mg/dL (HR 4.24 [1.51-11.92], p=0.006). On secondary analysis, CBili >4 mg/dL at baseline was associated with a lower chance of attaining enteral autonomy., Conclusion: In children with intestinal failure treated at intestinal rehabilitation programs, more advanced IFALD at referral is associated with increased mortality and decreased prospect of attaining enteral autonomy. Early referral of children with intestinal failure to intestinal rehabilitation programs should be strongly encouraged., Level of Evidence: Treatment Study, Level III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to evaluate safety, technical success, and clinical outcomes of treatment for venous malformations using n -BCA glue embolization immediately prior to excision. Sixty three patients (22 male, 41 female; mean age 12 years (range 1-25)) who underwent 70 procedures for extremity and trunk venous malformations were reviewed. Indications for treatment included pain (100%), swelling (22%), and diminished range of motion (16%). Thirty seven patients (59%) had undergone prior stand-alone interventional or surgical treatment but were persistently symptomatic. Safety, technical and clinical success were retrospectively assessed., Results: Embolization was technically successful in 100% of patients. Mean lesion size was 3.0 × 2.9 × 5.7 cm. Three patients (5%) underwent planned, second stage procedures for lesions intentionally not treated at the first procedure. Four patients (6%) underwent an unplanned, second stage procedure for residual disease after the primary operation. Mean and median follow-up duration were 18 and 17 months, respectively (range 3 to 35 months). Symptomatic improvement was achieved in 58 patients (92%), of whom 41 (65%) reported complete elimination of pain. There were no recognized instances of nontarget embolization or other complications of the interventional procedure. One patient required additional surgery for wound dehiscence and one patient developed an abscess requiring incision and drainage. Minor surgical complications included surgical site skin infections ( n  = 5) and numbness ( n  = 1). Mean and median surgical blood loss volumes were 131 mL and 10 mL, respectively. One patient required perioperative blood transfusion., Conclusions: Extremity and truncal venous malformations can be safely and effectively treated in a single-stage fashion using glue embolization immediately preceding excision., Competing Interests: Obtained prior to performing study.Obtained.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Introduction: Newborns with gastroschisis have historically undergone surgical repair under general anesthesia. Our institution recently transitioned to the sutureless umbilical closure for gastroschisis. We sought to evaluate the feasibility of bedside gastroschisis repair without endotracheal intubation., Methods: A retrospective review was performed of neonates with gastroschisis who underwent sutureless umbilical closure from 2011 to 2015. Clinical characteristics and outcomes between groups were compared., Results: In total, 53 infants underwent sutureless umbilical closure. Closure without endotracheal intubation was attempted in 23 (43%) babies and was successful in 15 (65%) infants. Two of the 8 patients who required intubation needed a temporary silo. Neonates successfully repaired without intubation were more premature (p < 0.01), smaller at birth (p = 0.01), and repaired nearly an hour sooner (p < 0.01). There were no differences in time to full enteral nutrition, length of stay, bowel ischemia, or sepsis., Conclusion: Bedside sutureless umbilical closure without intubation is feasible and effective in newborns with gastroschisis. The procedure decreases time to gastroschisis closure. Smaller and more premature neonates were more likely to be successfully closed without intubation., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: Recent advances in medical and surgical management have led to improved long-term survival in children with intestinal failure. Yet, limited data exist on their neurodevelopmental and cognitive outcomes. The aim of the present study was to measure neurodevelopmental outcomes in children with intestinal failure., Methods: Children enrolled in a regional intestinal failure program underwent prospective neurodevelopmental and psychometric evaluation using a validated scoring tool. Cognitive impairment was defined as a mental developmental index <70. Neurodevelopmental impairment was defined as cerebral palsy, visual or hearing impairment, or cognitive impairment. Univariate analyses were performed using the Wilcoxon rank-sum test. Data are presented as median (range)., Results: Fifteen children with a remnant bowel length of 18 (5-85) cm were studied at age 17 (12-67) months. Thirteen patients remained dependent on parenteral nutrition. Twelve (80%) subjects scored within the normal range on cognitive testing. Each child with cognitive impairment was noted to have additional risk factors independent of intestinal failure including cardiac arrest and extreme prematurity. On univariate analysis, cognitive impairment was associated with longer inpatient hospital stays, increased number of surgical procedures, and prematurity (P < 0.02). In total, 4 (27%) children demonstrated findings consistent with neurodevelopmental impairment., Conclusions: A majority of children with intestinal failure demonstrated normal neurodevelopmental and cognitive outcomes on psychometric testing. These data suggest that children with intestinal failure without significant comorbidity may be at low risk for long-term neurodevelopmental impairment.

Routine supplementation of iodine in parenteral nutrition (PN) solutions is not current practice in the United States. In this case study, we describe an incidental finding of goiter in a long-term PN-dependent adolescent. With increased iodine screening, we then identified additional patients with undetectable urinary iodine concentrations in our population of children with short bowel receiving long-term PN. We hypothesize that 2 practice changes are possibly reducing iodine provision to long-term PN-dependent patients: transition to alcohol-based skin preparations and lipid minimization., (© 2014 American Society for Parenteral and Enteral Nutrition.)

Background: Gastroschisis is a newborn anomaly requiring emergent surgical intervention. We review our experience with gastroschisis to examine trends in contemporary surgical management., Methods: Infants who underwent initial surgical management of gastroschisis from 1996 to 2014 at a pediatric hospital were reviewed. Closure techniques included primary fascial repair using suture or sutureless umbilical closure, and staged repair using sutured or spring-loaded silo (SLS). Data were separated into 3 clinical eras: pre-SLS (1996 to 2004), SLS (2005 to 2008), and umbilical closure (2009 to 2014)., Results: In the pre-SLS era, 60% (34/57) of infants with gastroschisis underwent primary repair. With the advent of SLS, there was a decrease in primary repair (15%, 10/68, P < .0001). Following introduction of sutureless umbilical closure, 61% (47/77) of infants have undergone primary repair. On multivariate regression, primary repair was associated with shorter intensive care unit stays (P < .001) and time to initiate enteral nutrition (P < .01)., Conclusions: Following introduction of a less invasive technique for gastroschisis repair, most infants with gastroschisis were able to be repaired primarily. Primary repair should be considered in all babies with gastroschisis and favorable anatomy., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Young children with intestinal failure are at risk for complications from rotavirus gastroenteritis. To date, the safety and immunogenicity of rotavirus vaccines in these children are not known. We hypothesized that rotavirus vaccination would be safe and confer immunity to infants with intestinal failure and a history of abdominal surgery., Methods: The study population consisted of infants with early intestinal failure who required abdominal surgery and parenteral nutrition for necrotizing enterocolitis, gastroschisis, jejunoileal atresia, or meconium peritonitis. Subjects received a rotavirus vaccine series at the appropriate age. Safety assessments were performed for the first 5 days after each vaccine dose. Viral stool shedding and serum rotavirus antigen were measured at multiple time points after each dose of the vaccine. A 3-fold increase in rotavirus immunoglobulin A titer at study completion defined seroconversion., Results: Fifteen infants were enrolled and 14 infants completed the study protocol. Mild diarrhea, emesis, and fever were noted after vaccination in 33%, 40%, and 7% of subjects, respectively. No subject experienced postvaccine intussusception, viremia, dehydration, or required alterations in nutritional care. Viral stool shedding was noted in 47%, but only 1 child shed virus beyond 2 weeks postvaccination. All subjects who were not considered immune at baseline developed seroconversion to rotavirus after vaccination., Conclusions: In infants with intestinal failure, rotavirus vaccination appears to be safe and immunogenic. We found no evidence for altered viral shedding in this population. Given the profound consequences associated with rotavirus infection and potential benefits of rotavirus vaccine in this cohort, multicenter studies focusing on vaccine efficacy are warranted., (© The Author 2013. Published by Oxford University Press on behalf of the Pediatric Infectious Diseases Society. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.)

Background: Parenteral nutrition (PN) is a lifesaving therapy for children with intestinal failure and can now be used chronically without the life-threatening complications of the past. Adequate intravenous trace element supplementation is required as part of a complete nutrition prescription. According to the U.S. Food and Drug Administration (FDA), the number of drug shortages, including sterile injectable agents used as PN components, has increased since 2010. Selenious acid as an individual additive was recently unavailable at our institution for 9 months due to a national shortage., Materials and Methods: To assess the impact of the selenious acid shortage, we retrospectively compiled data from existing clinical records for eligible patients. We included children with intestinal failure on full PN support who were older than 1 year at the onset of the selenium shortage. Whole-blood selenium concentrations prior to the selenious acid shortage were compared with concentrations drawn during the shortage., Results: Five patients with intestinal failure and complete PN dependence were identified, and all 5 patients had normal serum selenium concentrations prior to the shortage. All 5 patients developed severe biochemical selenium deficiency in direct correlation with the shortage of selenium. No morbidity associated with selenium deficiency was observed. Selenium concentrations recovered after selenium supplementation was reinstituted., Conclusion: A national selenious acid shortage was associated with biochemical selenium deficiency in a cohort of children with intestinal failure. Despite very low selenium concentrations, none of our patients exhibited clinical signs of deficiency.

Objectives: Survival of infants with intestinal failure (IF) has increased in the past decade; however, data on their health-related quality of life (HRQOL) are lacking. We hypothesized that HRQOL would be lower among children with IF compared with that of healthy children., Methods: We performed a cross-sectional study of the HRQOL of children enrolled in the outpatient intestinal rehabilitation program at Seattle Children's Hospital using the PedsQL 4.0 Generic Core Scales parent proxy-report and the Family Impact Module questionnaires. Parents were asked 2 open-ended questions pertaining to the suitability and completeness of the PedsQL to assess their and their child's HRQOL., Results: Parents of 23 children with IF completed the questionnaires. Compared with norms for healthy children, parents reported significantly lower total PedsQL scores for children ages 1 to 2 years (mean difference -13.16, 95% confidence interval [CI] -21.86 to -4.46; P = 0.003) and 2 to 6 years (mean difference -15.57, 95% CI -22.66 to -8.48; P < 0.001). Scores were also lower for children younger than 1 year (mean difference -6.43, 95% CI -13.93 to 1.07), although this test was not statistically significant. No measured demographic or clinical characteristics were associated with HRQOL. The majority of parents (65%) said the PedsQL failed to address important effects of IF on children and their families., Conclusions: Children with IF and their parents have a decreased HRQOL compared with healthy children as measured by the PedsQL survey. A disease-specific module or separate HRQOL questionnaire is needed for a more comprehensive assessment of HRQOL in children with IF.

Background: Intestinal lengthening remains a treatment option in pediatric short bowel syndrome. However, clinical indications and nutritional outcomes from bowel lengthening are not well defined., Methods: A retrospective review of a prospective database was conducted of patients who underwent bowel lengthening using serial transverse enteroplasty (STEP) at a single center., Results: Sixteen children who underwent 19 STEP procedures were identified. STEP increased median small bowel length from 84 cm (range, 19 to 295 cm) to 103 cm (range, 24 to 375 cm) (P = .0001). Caloric provisions from parenteral nutrition were decreased after STEP (69% vs 0%, P < .01). Of 15 subjects in the nutritional analysis, 12 (80%) had improved enteral tolerance, and 9 (60%) achieved enteral autonomy after STEP. Six STEP procedures were not associated with improvements in enteral nutrition, and 5 (83%) were performed in children with gastroschisis., Conclusions: A majority of children with short bowel syndrome were weaned off parenteral nutrition after STEP. Gastroschisis may portend a less optimal outcome from the procedure., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: Surgical infants requiring long-term parenteral nutrition (PN) are at risk for parenteral nutrition-associated liver disease (PNALD). The purpose of this study was to determine the effect of a lipid restricted PN regimen in preventing the development of PNALD in surgical infants., Methods: In 2009, we implemented a lipid restricted strategy in surgical infants expected to be on long-term PN using a soy-based lipid emulsion at a goal provision of 1g/kg/day throughout a patient's entire PN course. An experimental cohort of surgical infants treated with lipid restriction from 2009 to 2011 (n=82) was retrospectively compared to a control cohort of infants from 2005 to 2008 receiving standard intravenous lipid dosing (n=132). A multivariable relative risk regression model was constructed analyzing the association between lipid restriction and PNALD., Results: Patients admitted during the lipid restriction era had reduced daily lipid provisions compared to the control group (p<0.001). There were no significant differences in demographic or measured clinical characteristics between the two groups. A significant reduction in the incidence of PNALD was demonstrated in the lipid restricted group compared to the control group (22% vs. 43%, p=0.002). On multivariable relative risk regression, patients treated with standard lipid provisions were 1.77 times more likely to develop PNALD than patients who were lipid restricted (95% CI: 1.2-2.7; p=0.007)., Conclusion: Restriction of intravenous soy-based lipid in PN-fed surgical infants is associated with a reduction in the incidence of liver disease. Early lipid restriction should be considered in all surgical infants who require PN as a preventative measure against PNALD., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: Recent data have demonstrated improved survival in children with intestinal failure. We hypothesized that this trend would also be observed in children with ultrashort bowel syndrome., Methods: A prospective database from Seattle Children's Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with 10 cm or less of small bowel enrolled in the program since 2005. Data are listed as median (range)., Results: Five patients were identified with a bowel length of 6 (1-10) cm and follow-up of 54 (43-61) months. All children have survived and are currently between 3.5 and 5.5 years of age. One patient underwent isolated intestinal transplantation and one patient is currently listed for intestinal transplantation. The transplanted child is fully enterally fed. The other patients remain at least partially dependent on parenteral nutrition. None of these patients have current evidence of parenteral nutrition-associated liver disease. Patients have required extensive care after referral to our program, including 18 (15-32) visits to the emergency room, 152 (114-273) days of inpatient care, and 6 (5-9) central line-associated blood stream infections., Conclusions: Long-term survival in children with ultrashort bowel length is possible after referral to an intestinal failure program, although extensive medical management is required. These children may be reasonable candidates for long-standing intestinal rehabilitation as a bridge to intestinal transplantation.

We report the case of a 14-year-old boy with pectus excavatum and mild scoliosis. The patient underwent a minimally invasive Nuss repair with excellent cosmetic result. He returned 3 weeks post-operatively with bilious emesis and a 3.6-kg weight loss (5.5% of total body weight). Radiographic evaluation was consistent with the diagnosis of superior mesenteric artery syndrome and the child ultimately required nasojejunal feedings. Following return to his baseline weight, he was transitioned to oral feedings and has done well in follow-up. This is the first report of SMA syndrome following minimally invasive pectus excavatum repair., (Published by Elsevier Inc.)

Background: Magnet and button battery ingestions are increasingly common, and can result in significant morbidity. Timely identification of hazardous foreign body ingestions can be difficult in non-verbal and non-disclosing children., Objectives: We aim to present a case that demonstrates some of the challenges around identifying and correctly locating magnets and batteries, and the importance of prompt identification and removal., Case Report: We describe an older child with the covert ingestion of multiple magnets and batteries, with magnets that attracted across the stomach and a loop of jejunum. Mild symptoms and signs resulted in a delayed diagnosis and serious consequences. Radiographs suggested a gastric location of the foreign bodies., Conclusion: Health care workers should consider the possibility of battery or magnet ingestions in children with vomiting and abdominal pain, even when well-appearing. Like esophageal batteries, multiple gastrointestinal magnets and combined magnet-battery ingestions can cause significant morbidity, and prompt identification is important. Providers should ask verbal children for ingestion histories, and consider radiographs when symptoms are atypical or persistent. Like esophageal batteries, gastrointestinal magnet-battery ingestions should be removed promptly to prevent complications. Caregivers should supervise or limit the use of toys that include magnets and batteries., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Hepatic artery thrombosis (HAT) remains a significant cause of graft failure and mortality after pediatric liver transplantation. Conditions not associated with hepatic failure, such as liver tumors, may be more prone to thrombotic problems after transplant. We hypothesized that liver transplant for hepatic malignancies may be associated with increased rates of HAT in the posttransplant period., Methods: We conducted a retrospective review of pediatric patients (age, 0-21 years) who underwent primary liver transplantation at a free-standing children's hospital from 1990 to 2009. We reviewed cause of underlying liver disease, age, sex, weight, occurrence of HAT, use of antiplatelets and anticoagulants perioperatively, as well as reintervention, retransplant, and death., Results: A total of 129 children underwent 146 liver transplants, and 15 (12%) patients developed HAT. Nine liver transplants were performed for hepatic malignancy, and 4 (44%) of these patients developed HAT (relative risk, 4.85; 95% confidence interval, 1.9-12.2; P = .0015). All 4 children with hepatic malignancy and HAT required reintervention, including 3 retransplants (75%). One of these patients died., Conclusions: Hepatic artery thrombosis occurs approximately 5 times more often and appears to be more morbid in children with hepatic malignancy after transplantation. Prospective evaluation of prophylactic anticoagulation regimens in the setting of hepatic malignancy requiring transplantation is warranted., (Copyright © 2012 Elsevier Inc. All rights reserved.)

In this article, we present an unusual case of a young boy who presented with abdominal pain and was found to have a sewing needle that had migrated through the abdominal wall into the peritoneal space. After imaging and endoscopy, the needle was extracted laparoscopically without any evidence of intra-abdominal organ injury and with a good long-term outcome for the child. There are no other such reported cases in the literature. This case highlights the subtleties in management of intra-abdominal foreign bodies in children including rare causes such noningested foreign bodies., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Despite advances in pediatric nutritional support and a renewed focus on management of intestinal failure, there are limited recent data regarding the risk of parenteral nutrition (PN)-associated liver disease in surgical infants. This study investigated the incidence of cholestasis from PN and risk factors for its development in this population., Methods: A retrospective review was performed of all neonates in our institution who underwent abdominal surgery and required postoperative PN from 2001 to 2006. Cholestasis was defined as 2 conjugated bilirubin levels greater than 2 mg/dL over 14 days. Nonparametric univariate analyses and multivariate logistic regression were used to model the likelihood of developing cholestasis. Median values with range are presented., Results: One hundred seventy-six infants met inclusion criteria, and patients received PN for 28 days (range, 2-256 days). The incidence of cholestasis was 24%. Cholestatic infants were born at an earlier gestational age (34 vs 36 weeks; P < .01), required a 3-fold longer PN duration (76 vs 21 days; P < .001), had longer inpatient stays (86 vs 29 days; P < .001), and were more likely to be discharged on PN. The median time to cholestasis was 23 days. Cholestasis was an early development; 77% of cholestatic infants developed cholestasis by 5 weeks of PN exposure. On multivariate regression, only prematurity was significantly associated with development of cholestasis (P < .05)., Conclusion: In this analysis, the development of PN-associated liver disease occurred early in the course of exposure to PN. These data help to define the time course and prognosis for PN-associated cholestasis in surgical infants., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: Multidisciplinary treatment of pediatric intestinal failure has shown promising results. However, there are limited data as to the optimal time frame for referral of patients to intestinal failure programs. The aim of this study was to explore the relationship of hyperbilirubinemia at referral with patient outcomes in a multidisciplinary program., Methods: A retrospective analysis was performed of a prospectively collected database from a multidisciplinary intestinal failure program. Multivariable logistic regression adjusted for age at referral was used to model the association between the conjugated bilirubin at referral and risk of mortality. Median values with range are reported., Results: Sixty-two patients were referred from 2005 to 2009. Patients presented at age 6.4 months (0.4-261.4 months) and were followed up for 16.8 (0.3-53.0) months. Nine subjects (14.5%) died, and 12 subjects (19.4%) were listed for combined liver-intestine transplant. A 50% mortality was seen in patients referred with a conjugated bilirubin ≥ 7.2 mg/dL (n = 12), whereas mortality at referral bilirubin levels <7.2 mg/dL was 6%. After adjusting for age at referral, patients with a conjugated bilirubin ≥ 7.2 mg/dL at referral were 15.4 times more likely to die than patients who presented with lower bilirubin levels (P = .001; 95% confidence interval, 2.8-83.4)., Conclusion: Within a pediatric intestinal failure program, mortality is associated with the degree of hyperbilirubinemia at time of referral. These data strongly suggest that these patients should be referred to a multidisciplinary program early in the evolution of their liver disease., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Wilms' tumor is the most common malignant renal tumor of childhood. Surgical resection is an important aspect of therapy and is traditionally performed through an open transabdominal approach. The advent of advanced laparoscopic techniques for benign renal lesions has led to interest in applying a minimally invasive approach to Wilms' tumors in children. We describe a 2-year-old girl who presented with a right renal mass measuring 18 × 13 cm with peritoneal seeding and pulmonary metastatic disease. Wilms' tumor was confirmed on open biopsy. After neoadjuvant chemotherapy, a laparoscopic resection of the tumor with right radical nephroureterectomy, retroperitoneal lymph node dissection, and resection of peritoneal metastases was achieved using one 12-mm and four 5-mm ports. The specimens were removed in an endoscopic retrieval bag through a small Pfannenstiel incision by extending the 12-mm port site. The patient had an uncomplicated recovery and was discharged on the fifth post-operative day. She remains disease free at 19 months postoperatively. Minimally invasive techniques may be considered for resection of Wilms' tumor provided oncologic principles are carefully followed., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Juvenile granulosa cell tumor (JGCT) is an uncommon gonadal stromal tumor that occurs rarely in the testis. We report a newborn boy with bilateral intra-abdominal JGCT presenting with abdominal distention and respiratory distress at birth. He was taken to the operating room emergently, and 2 large masses connected by gubernacula to the inguinal canals were resected. Associated abnormalities included a constitutional chromosome 4 abnormality, polymicrogyria, and renal cysts. This report describes a rare presentation of JGCT with abdominal compression and expands the literature to include bilateral testicular involvement. Additionally, it is the 1st report of JGCT associated with a chromosome 4 abnormality, highlighting a genetic region that may be important in JGCT development.

Surgical resections of massive sacrococcygeal teratomas (SCTs) carry significant risk due to baseline hemodynamic instability and the potential for significant hemorrhage. In this case report, a fetus with sacrococcygeal teratoma developed high-output cardiac instability at 32 weeks' gestation. After urgent cesarian delivery, a damage-control operation using Teflon-pledgeted mattress sutures allowed for hemodynamic control of bleeding into the tumor. One week later, after subsequent fluid resuscitation and stabilization, an elective staged resection of the complete mass including the pelvic portion was performed. The patient was discharged home on postoperative day 30 and was doing well at her most recent clinic visit at 30 months of age., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: The aim of this study was to evaluate the clinical experience of a regional multidisciplinary intestinal failure program for children established in 2005., Methods: Data were collected from a prospective internal database. Univariate analyses were performed to compare pre- and post-treatment outcomes. Median values are reported., Results: Forty-nine children were referred at an age of 7 months. Remnant small bowel length was 29 cm. With follow-up of 14 months, overall patient survival was 88%. Thirteen bowel-lengthening procedures were performed, thereby increasing small bowel length from 83 to 132 cm (P < .05). Enteral autonomy was achieved in 22 patients (45%), and the caloric requirement for parenteral nutrition was decreased from 100% to 41% (P < .01). Conjugated bilirubin was reduced from 4.1 to 0 mg/dL (P < .05)., Conclusion: A multidisciplinary approach to pediatric intestinal failure that prioritizes intestinal rehabilitation can achieve successful enteral feeding advancement, improved liver function, and excellent survival in intermediate-range follow-up., (Copyright 2010 Elsevier Inc. All rights reserved.)

In patients with intestinal malrotation, Ladd bands are known to cause obstruction of the duodenum and small bowel. However, acute obstruction of the colon from Ladd bands has not been previously described. We report a case of complete obstruction of the hepatic flexure of the colon secondary to a Ladd band in a previously healthy teenage boy.

Congenital pyloric atresia (CPA) is a rare disorder that has traditionally been diagnosed in the postnatal period. With improvements in ultrasound technology and increasing use of MRI, CPA is now diagnosed with increasing accuracy in utero. This also allows for identification of concomitant anomalies, which greatly affects infant survival. In addition, antenatal diagnosis of CPA and associated anomalies allow for family counseling and planning of treatment for the perinatal period including early referral to a center with pediatric surgical services. Here, we present a case of CPA with preoperative diagnosis using ultrasound and MRI.

Mesenteric lipoblastoma is a rare tumor and, its presentation as a bowel obstruction with possible midgut volvulus has only been reported once before. A 7-year-old girl presented with nausea and vomiting but a benign abdominal examination. Upper gastrointestinal contrast study demonstrated possible malrotation with midgut volvulus. During emergency laparotomy, segmental small bowel volvulus secondary to a large mesenteric lipoblastoma was found. The lipoblastoma was resected with a segment of small bowel. Resection margins were negative for tumor, and the patient is doing well with no evidence of recurrence.

Background: Serial transverse enteroplasty (STEP) is a novel surgical therapy for short bowel syndrome and is being used with increasing frequency worldwide. Because no single center is likely to obtain sufficient experience for meaningful analysis, we created the International STEP Data Registry to allow for larger, multicenter patient accrual and followup. This report describes patient characteristics, operative parameters, and early results of STEP in the first 38 patients enrolled in the International STEP Data Registry., Study Design: After IRB approval, data were entered online through password-protected enrollment and followup forms. Patient and procedural characteristics were analyzed. Pre- and postoperative small bowel length and enteral feeding tolerance were compared with the paired t-test., Results: Between September 1, 2004, and April 30, 2006, 19 centers from 3 countries enrolled 38 patients. Median followup from STEP procedure to analysis was 12.6 months (range 0 to 66.9 months). Indications for STEP were short bowel syndrome (SBS, n=29), bacterial overgrowth (n=6), and neonatal atresia (n=3). Mean small intestine length was substantially increased in all groups (68+/-44 cm versus 115+/-87 cm, p < 0.0001, n=27). Notable complications included intraoperative staple line leak (n=2), bowel obstruction (n=2), and fluid collection or abscess (n=3). Late outcomes included progression to transplantation (n=3) and mortality (n=3). For the short bowel syndrome cohort, enteral tolerance was notably increased from 31%+/-31% to 67%+/-37% of calories (p < 0.01, n=21)., Conclusions: STEP has been performed at multiple centers with minimal complications and encouraging outcomes. Indications for the procedure have broadened beyond short bowel syndrome to include bacterial overgrowth and neonatal intestinal obstruction with dilated proximal intestine. Continued accrual and followup of patients in the International STEP Data Registry will elucidate the longterm safety and efficacy of the procedure, with the goal of improved patient selection and operative timing.

Background & Aims: The aim of this study was to describe the long-term nutritional outcomes of 4 patients with short bowel syndrome (SBS) who underwent a newly described bowel-lengthening procedure, the serial transverse enteroplasty (STEP) procedure., Methods: We performed a retrospective review of the medical records of 4 children who underwent STEP at 1 center. The primary outcome measure was the percentage of total dietary energy received by the enteral route before and after STEP. Other outcome measures were weight and height Z scores and body composition, as measured by arm anthropometric values., Results: Four children with SBS were identified, all of whom had been dependent on parenteral nutrition since birth. The mean preoperative follow-up period was 234 days (range, 63-502 days), and the mean postoperative follow-up period was 362 days (range, 252-493 days). By using model-based mean estimates, the mean enteral nutrition intake was 48% preoperatively vs 62% postoperatively (P = .02). The model mean weight for age Z score increased by .7 SDs postoperatively (P = .01), and the model mean weight for height increased by .6 SDs (P < .0001). The percent standard mid-upper-arm circumference increased by 13.1% postoperatively (P = .03), and the percent standard triceps skinfold increased by 24.5% postoperatively (P < .0001)., Conclusions: The STEP procedure was associated with improved growth and body composition among 4 children with SBS in the face of a decreasing need for parenteral nutrition. Among patients with refractory SBS and dilated small intestine, the STEP procedure is associated with improved clinical and nutritional outcomes in the first year after surgery.

Objective: Critically ill neonates on extracorporeal life support (ECLS) demonstrate elevated rates of protein breakdown that, in turn, are associated with increased morbidity and mortality. This study sought to determine if the administration of the anabolic hormone insulin improved net protein balance in neonates on ECLS., Methods: Twelve parenterally fed neonates, on ECLS, were enrolled in a randomized, prospective, crossover trial. Subjects were administered a hyperinsulinemic euglycemic clamp and a control saline infusion. Protein metabolism was quantified using ring-D5-phenylyalanine and ring-D2-tyrosine stable isotopic infusions. Statistical comparisons were made by paired sample t tests (significance at P < 0.05)., Results: Serum insulin concentration increased 20-fold during insulin infusion compared with saline infusion control (P < 0.0001). Protein breakdown was significantly decreased during insulin infusion compared with controls (7.98 +/- 1.82 vs. 6.89 +/- 1.03 g/kg per day; P < 0.05). Serum amino acid concentrations were significantly decreased by insulin infusion (28,450 +/- 9270 vs. 20,830 +/- 8110 micromol/L; P < 0.02). Insulin administration tended to decrease protein synthesis (9.58 +/- 2.10 g/kg per day vs. 8.60 +/- 1.20; P = 0.05). For the whole cohort, insulin only slightly improved net protein balance (protein synthesis minus protein breakdown) (1.60 +/- 0.80 vs. 1.71 +/- 0.89 g/kg per day; P = 0.08). In neonates receiving > or =2 g/kg per day of dietary amino acids insulin significantly improved net protein balance (2.17 +/- 0.34 vs. 2.40 +/- 0.26 g/kg per day; P < 0.01)., Conclusions: Insulin effectively decreases protein breakdown in critically ill neonates on ECLS. However, this is associated with a significant reduction in plasma amino acids and a trend toward decreased protein synthesis. Insulin administration significantly improves net protein balance only in those ECLS neonates in whom adequate dietary protein is provided.

Unlabelled: OBJECTIVE/SUMMARY BACKGROUND DATA: Serial transverse enteroplasty (STEP) is a new intestinal lengthening procedure that has been shown to clinically increase bowel length. This study examined the impact of the STEP procedure upon intestinal function in a model of short bowel syndrome., Methods: Young pigs (n=10) had a reversed segment of bowel interposed to induce bowel dilatation. Five pigs underwent a 90% bowel resection with a STEP procedure on the remaining dilated bowel while 5 served as controls and had a 90% bowel resection without a STEP procedure. Determinations of nutritional status, absorptive capacity, and bacterial overgrowth were conducted 6 weeks after resection. Statistical comparisons were made by 2-sample t test (significance at P<0.05)., Results: The STEP procedure lengthened the bowel from 105.2+/-7.7 cm to 152.2+/-8.3 cm (P<0.01). The STEP animals showed improved weight retention compared with controls (mean, -0.5%+/-1.8% body weight versus -17.6%+/-1.5%, P<0.001). Intestinal carbohydrate absorption, as measured by d-Xylose absorption and fat absorptive capacity as measured by serum vitamin D and triglyceride levels, were increased in the STEP group versus controls. Serum citrulline, a marker of intestinal mucosal mass, was significantly elevated in the STEP pigs compared with controls. None of the STEP animals but 4 of 5 control animals were noted to have gram-negative bacterial overgrowth in the proximal bowel., Conclusions: STEP improves weight retention, nutritional status, intestinal absorptive capacity, and serum citrulline levels in a porcine short bowel model. A salutary effect upon bacterial overgrowth was also noted. These data support the use of this operation in short bowel syndrome.