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.

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.

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.

Neurodevelopmental and Cognitive Outcomes in Children With Intestinal Failure.

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.

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.

Ultrashort bowel syndrome in children.

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.

Health-related quality of life in children with intestinal failure.

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.

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.

Hidden attraction: a menacing meal of magnets and batteries.

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.

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.

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.

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.

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.

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.

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.