Prevention of Parenteral Nutrition-associated Cholestasis Using Reduced Dose Soybean Lipid Emulsion: A Multicenter Randomized Trial.

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.

A time out for prayer.

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.

Chest Tube Management following Lung Resection in Pediatric Patients: A Retrospective Analysis.

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.

Medical and Surgical Aspects of Intestinal Failure in the Child.

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.

Pediatric surgery milestones 2.0: A primer.

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.

Child self-reported quality of life in pediatric intestinal failure.

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.

The role of feeding advancement strategy on length of stay and hospital costs in newborns with gastroschisis.

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.

Ethical dilemmas in the management of infants with necrotizing enterocolitis totalis.

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.

Prospective evaluation of the family's health-related quality of life in pediatric intestinal failure.

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.

Peer review and its ethical implications.

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.

Impact of COVID-19 on procedure volume at a tertiary pediatric hospital.

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.

The association between fluid restriction and hyponatremia in newborns with gastroschisis.

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.

Evaluation of SMOFlipid in Pediatric Intestinal-Failure Patients and Its Effects on Essential Fatty Acid Levels.

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.

Variation in hospital costs for gastroschisis closure techniques.

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.

Factors Associated with Health-Related Quality of Life in Children with Intestinal Failure.

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.

Organization and outcomes of multidisciplinary intestinal failure teams.

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.

Attitudes Surrounding the Management of Neonates with Severe Necrotizing Enterocolitis.

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.

The extent of intestinal failure-associated liver disease in patients referred for intestinal rehabilitation is associated with increased mortality: an analysis of the Pediatric Intestinal Failure Consortium database.

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.

Combined glue embolization and excision for the treatment of venous malformations.

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.

Outcomes of bedside sutureless umbilical closure without endotracheal intubation for gastroschisis repair in surgical infants.

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.