Contemporary Outcomes of Infants with Gastroschisis in North America: A Multicenter Cohort Study.

OBJECTIVE: To quantify outcomes and analyze factors predictive of morbidity and mortality in infants with gastroschisis. STUDY DESIGN: Clinical data regarding neonates with gastroschisis born between 2009 and 2014 were prospectively collected at 175 North American centers. Multivariate regression was used to assess risk factors for mortality and length of stay (LOS). RESULTS: Gastroschisis was diagnosed in 4420 neonates with median birth weight 2410 g (IQR 2105-2747). Survival (discharge home or alive in hospital at 1 year) was 97.8% with a 37 day median LOS (IQR 27-59). Sepsis, defined by positive blood or cerebrospinal fluid culture, was the only significant independent predictor of mortality (P = .04). Significant independent determinants of LOS and the percentage of neonates affected were as follows: bowel resection (9.8%, P < .0001), sepsis (8.6%, P < .0001), presence of other congenital anomalies (7.6%, including 5.8% with intestinal atresias, P < .0001), necrotizing enterocolitis (4.5%, P < .0001), and small for gestational age (37.3%, P = .0006). Abdominal surgery in addition to gastroschisis repair occurred in 22.3%, with 6.4% receiving gastrostomy or jejunostomy tubes and 6.3% requiring ostomy creation. At discharge, 57.0% were less than the 10th percentile weight for age. The mode of delivery (52.4% cesarean delivery) was not associated with any differences in outcome. CONCLUSIONS: Although neonates with gastroschisis have excellent overall survival they remain at risk for death from sepsis, prolonged hospitalization, multiple abdominal operations, and malnutrition at discharge. Outcomes appear unaffected by the use of cesarean delivery. Further opportunities for quality improvement include sepsis prevention and enhanced nutritional support.

Paget-Schroetter syndrome in 21 children: outcomes after multidisciplinary care.

OBJECTIVE: To review the presentation, management, and outcomes of Paget-Schroetter syndrome (PSS) in children and propose a multidisciplinary treatment algorithm involving pediatric and vascular surgery, interventional radiology, and hematology. STUDY DESIGN: Patients with PSS presenting between 2003 and 2013 were reviewed. Demographics, symptoms, therapies, and functional outcomes were noted. Data from early patients informed the development of a multidisciplinary treatment algorithm applied to later patients. RESULTS: Of 21 patients, mean ± SD age was 16 ± 1.6 years and 11 (52%) were male. Of patients with complete presentation data, common symptoms were edema (84%), discoloration (58%), and pain (58%). Thrombophilia workup revealed one heterozygote for factor V Leiden, 2 patients with factor VIII elevation and 1 patient with mildly low antithrombin. The most recent 8 patients were treated according to an algorithm developed by a multidisciplinary working group through experience with the first 13 cases. All patients underwent a venogram, endovascular intervention (including 15 receiving catheter-directed thrombolysis), and operative ipsilateral thoracic outlet decompression (first rib resection, anterior scalenectomy, and venolysis). Postoperative complications included hemothorax (2), pneumothorax (1), and recurrent thrombosis (2). Follow up duration was 12 ± 9.5 months. Symptoms recurred transiently in 1 patient. CONCLUSION: Pediatric patients with PSS can be treated successfully using a multidisciplinary treatment algorithm including anticoagulation, catheter-directed thrombolysis, and operative decompression of the thoracic outlet. Early outcomes are promising.

Preservation of biochemical liver function with low-dose soy-based lipids in children with intestinal failure-associated liver disease.

OBJECTIVES: Intestinal failure-associated liver disease (IFALD) contributes to significant morbidity in pediatric patients with intestinal failure (IF); however, the use of parenteral nutrition (PN) with a fish oil-based intravenous (IV) emulsion (FO) has been associated with biochemical reversal of cholestasis and improved outcomes. Unfortunately, FO increases the complexity of care: because it can be administered only under Food and Drug Administration compassionate use protocols requiring special monitoring, it is not available as a 3-in-1 solution and is more expensive than comparable soy-based IV lipid emulsion (SO). Because of these pragmatic constraints, a series of patient families were switched to low-dose (1 g kg(-1) day(-1)) SO following biochemical resolution of cholestasis. The present study examines whether reversal of cholestasis and somatic growth are maintained following this transition. METHODS: The present study is a chart review of all children with IFALD who switched from FO to SO following resolution of cholestasis. Variables are presented as medians (interquartile ranges). Comparisons were performed using the Wilcoxon signed-rank test. RESULTS: Seven patients ages 25.9 (16.2-43.2) months were transitioned to SO following reversal of cholestasis using FO. At a median follow-up of 13.9 (4.3-50.1) months, there were no significant differences between pretransition and post-transition serum alanine and aspartate aminotransferases, direct bilirubin, and weight-for-age z scores. Because of recurrence of cholestasis, 1 patient was restarted on FO after 4 months on SO. CONCLUSIONS: Biochemical reversal of IFALD and growth were preserved after transition from FO to SO in 6 of 7 (86%) patients. Given the challenges associated with the use of FO, SO may be a viable alternative in select patients with home PN.

Tight Glycemic Control With Insulin Does Not Affect Skeletal Muscle Degradation During the Early Postoperative Period Following Pediatric Cardiac Surgery.

OBJECTIVE: Critical illness is associated with significant catabolism, and persistent protein loss correlates with increased morbidity and mortality. Insulin is a potent anticatabolic hormone; high-dose insulin decreases skeletal muscle protein breakdown in critically ill pediatric surgical patients. However, insulin's effect on protein catabolism when given at clinically utilized doses has not been studied. The objective was to evaluate the effect of postoperative tight glycemic control and clinically dosed insulin on skeletal muscle degradation in children after cardiac surgery with cardiopulmonary bypass. DESIGN: Secondary analysis of a two-center, prospective randomized trial comparing tight glycemic control with standard care. Randomization was stratified by study center. PATIENTS: Children 0-36 months who were admitted to the ICU after cardiac surgery requiring cardiopulmonary bypass. INTERVENTIONS: In the tight glycemic control arm, insulin was titrated to maintain blood glucose between 80 and 110 mg/dL. Patients in the control arm received standard care. Skeletal muscle breakdown was quantified by a ratio of urinary 3-methylhistidine to urinary creatinine. MEASUREMENTS AND MAIN RESULTS: A total of 561 patients were included: 281 in the tight glycemic control arm and 280 receiving standard care. There was no difference in 3-methylhistidine to creatinine between groups (tight glycemic control, 249 ± 127 vs standard care, 253 ± 112, mean ± SD in µmol/g; p = 0.72). In analyses restricted to the patients in tight glycemic control arm, higher 3-methylhistidine to creatinine correlated with younger age, as well as lower weight, weight-for-age z score, length, and body surface area (p < 0.005 for each) and lower postoperative day 3 serum creatinine (r = -0.17; p = 0.02). Sex, prealbumin, and albumin were not associated with 3-methylhistidine to creatinine. During urine collection, 245 patients (87%) received insulin. However, any insulin exposure did not impact 3-methylhistidine to creatinine (t test, p = 0.45), and there was no dose-dependent effect of insulin on 3-methylhistidine to creatinine (r = -0.03; p = 0.60). CONCLUSION: Although high-dose insulin has an anabolic effect in experimental conditions, at doses necessary to achieve normoglycemia, insulin appears to have no discernible impact on skeletal muscle degradation in critically ill pediatric cardiac surgical patients.

Whole Body Protein Turnover and Net Protein Balance After Pediatric Thoracic Surgery: A Noninvasive Single-Dose 15 N Glycine Stable Isotope Protocol With End-Product Enrichment.

BACKGROUND: We used the 15 N glycine urinary end-product enrichment technique to quantify whole body protein turnover following thoracic surgery. MATERIALS AND METHODS: A single dose of 15 N glycine (2 mg/kg) was administered orally on postoperative day 1 to children (1-18 years) following thoracic surgery. 15 N enrichment of ammonia and urea was measured in mixed urine after 12 and 24 hours, respectively, and protein synthesis, breakdown, and net balance determined. Nitrogen balance (dietary intake minus urinary excretion) was calculated. Urinary 3-methylhistidine:creatinine ratio was measured as a marker of skeletal muscle protein breakdown. RESULTS: We enrolled 19 subjects-median (interquartile range): age, 13.8 years (12.2-15.1); weight, 49.2 kg (38.4-60.8)-who underwent thoracotomy (n = 12) or thoracoscopic (n = 7) surgery. Protein synthesis and breakdown by 15 N enrichment were 7.1 (5.5-9) and 7.1 (5.6-9) g·kg-1 ·d-1 with ammonia (12 hours) as the end product, and 5.8 (3.8-6.7) and 6.7 (4.5-7.6) with urea (24 hours), respectively. Net protein balance by the 15 N glycine and urinary urea nitrogen methods were -0.34 (-0.47, -0.3) and -0.48 (-0.65, -0.28) g·kg-1 ·d-1 , respectively (rs = 0.828, P < .001). Postoperative change in 3-methylhistidine:creatinine ratio did not correlate significantly with protein breakdown or balance. CONCLUSION: The single-dose oral administration of 15 N glycine stable isotope with measurement of urinary end-product enrichment is a feasible and noninvasive method to investigate whole body protein turnover in children. After major surgery, children manifest increased protein turnover and net negative balance due to increased protein breakdown.

High prevalence of same-sex twins in patients with cloacal exstrophy: Support for embryological association with monozygotic twinning.

PURPOSE: Previous studies have hypothesized that cloacal exstrophy may be caused by errors early in embryological development related to monozygotic twinning. This study reports the prevalence of twins in a large cohort of patients with cloacal exstrophy. METHODS: Patients with cloacal exstrophy treated 1974-2015 were reviewed for reports of multiple gestation or conjoined twinning. The genetic sex of the patient and their twin, and any mention of anomaly in the twin were recorded. Neither placental exam nor genetic testing results were available to definitively determine zygosity. RESULTS: Of 71 patients, 10 had a live born twin (14%), all of whom were of the same genetic sex as the affected patient. One additional patient's twin suffered intrauterine fetal demise, and another patient had a conjoined heteropagus twin. None of the twins were affected by exstrophy-epispadias complex. The rate of twin birth in this cohort was 4.4-7.7 higher than that reported by the Centers for Disease Control in the general population time period (P<0.001), with a striking preponderance of same-sex pairs. CONCLUSIONS: The highly significant prevalence of same-sex twin pairs within this cohort supports the hypothesis that the embryogenesis of cloacal exstrophy may be related to errors in monozygotic twinning. LEVEL OF EVIDENCE: 2b.

Ileostomy Prolapse in Children with Intestinal Dysmotility.

BACKGROUND: A relationship between intestinal motility and ileostomy prolapse has been suggested but not demonstrated objectively. AIMS: This study evaluated the association between ileostomy prolapse and intestinal dysmotility in children. METHODS: IRB-approved retrospective review of 163 patients with ileostomies (1998-2014) at a single institution. Patients were categorized as having clinical dysmotility as a primary diagnosis (n = 33), clinically suspected dysmotility based on underlying diagnosis (n = 60), or intestinal dysmotility unlikely (n = 70) at the time of ileostomy present. Intestinal manometry was categorized as normal (n = 13) or abnormal (n = 10). Primary outcome was pathologic stoma prolapse. Multivariate analysis using a logistic regression model and log-rank test to compare stoma prolapse rates over time between motility groups were used. RESULTS: Clinical diagnosis of dysmotility (p ≤ 0.001) and manometric findings of dysmotility (p = 0.024) were independently associated with stoma prolapse. Clinical dysmotility correlated with manometric findings (κ = 0.53). Prolapse occurred in 42% of patients with dysmotility, 34% of patients with suspected dysmotility, and 24% of patients with normal motility. One-year prolapse-free stoma "survival" was 45% for dysmotility, 72% for suspected dysmotility, and 85% for intestinal dysmotility unlikely groups (p = 0.006). CONCLUSIONS: Children with intestinal dysmotility are at great risk for stoma prolapse. Intestinal manometry could help identify these patients preoperatively.

Enteral autonomy, cirrhosis, and long term transplant-free survival in pediatric intestinal failure patients.

PURPOSE: Patient selection for transplant evaluation in pediatric intestinal failure is predicated on the ability to assess long-term transplant-free survival. In light of trends toward improved survival of intestinal failure patients in recent decades, we sought to determine if the presence of biopsy-proven hepatic cirrhosis or the eventual achievement of enteral autonomy were associated with transplant-free survival. METHODS: After IRB approval, records of all pediatric intestinal failure patients (parenteral nutrition (PN) >90 days) treated at a single intestinal failure center from February 2002 to September 2014 were reviewed. Chi-squared, Mann-Whitney, and log-rank testing were performed as appropriate. RESULTS: Of 313 patients, 174 eventually weaned off PN. Liver biopsies were available in 126 patients (most common indication was intestinal failure associated liver disease, IFALD), and 23 met histologic criteria for cirrhosis. Transplant-free survival for the whole cohort of 313 patients was 94.7% at 1 year and 89.2% at 5 years. Among patients with liver biopsies, transplant-free survival in cirrhotics vs. noncirrhotics was 95.5% vs. 94.1% at one year and 95.5% vs. 86.7% at 5 years (P=0.29). Transplant-free survival in patients who achieved enteral autonomy compared with patients who remained PN dependent was 98.2% vs. 90.3% at one year and 98.2% vs. 76.9% at 5 years (P<0.001). There was no association between cirrhosis and eventual enteral autonomy (P=0.88). CONCLUSIONS: Achieving enteral autonomy was associated with improved transplant-free survival in pediatric intestinal failure patients. There was no association between histopathological diagnosis of cirrhosis and transplant-free survival in the cohort. These data suggest that automatic transplant referral may not be required for histopathological diagnosis of cirrhosis alone, and that ongoing efforts aimed at achievement of enteral autonomy remain paramount in pediatric intestinal failure.

Necrotizing enterocolitis is associated with earlier achievement of enteral autonomy in children with short bowel syndrome.

PURPOSE: Necrotizing enterocolitis (NEC) remains one of the most common underlying diagnoses of short bowel syndrome (SBS) in children. The relationship between the etiology of SBS and ultimate enteral autonomy has not been well studied. This investigation sought to evaluate the rate of achievement of enteral autonomy in SBS patients with and without NEC. METHODS: Following IRB approval, 109 patients (2002-2014) at a multidisciplinary intestinal rehabilitation program were reviewed. The primary outcome evaluated was achievement of enteral autonomy (i.e. fully weaning from parenteral nutrition). Patient demographics, primary diagnosis, residual small bowel length, percent expected small bowel length, median serum citrulline level, number of abdominal operations, status of the ileocecal valve (ICV), presence of ileostomy, liver function tests, and treatment for bacterial overgrowth were recorded for each patient. RESULTS: Median age at PN onset was 0 weeks [IQR 0-0]. Median residual small bowel length was 33.5 cm [IQR 20-70]. NEC was present in 37 of 109 (33.9%) of patients. 45 patients (41%) achieved enteral autonomy after a median PN duration of 15.3 [IQR 7.2-38.4]months. Overall, 64.9% of patients with NEC achieved enteral autonomy compared to 29.2% of patients with a different primary diagnosis (p=0.001, Fig. 1). Patients with NEC remained more likely than those without NEC to achieve enteral autonomy after two (45.5% vs. 12.0%) and four (35.7% vs. 6.3%) years on PN (Fig. 1). Logistic regression analysis demonstrated the following parameters as independent predictors of enteral autonomy: diagnosis of NEC (p<0.002), median serum citrulline level (p<0.02), absence of a jejunostomy or ileostomy (p=0.013), and percent expected small bowel length (p=0.005). CONCLUSIONS: Children with SBS because of NEC have a significantly higher likelihood of fully weaning from parenteral nutrition compared to children with other causes of SBS. Additionally, patients with NEC may attain enteral autonomy even after long durations of parenteral support.

Growth morbidity in patients with cloacal exstrophy: a 42-year experience.

PURPOSE: Cloacal exstrophy is associated with multiple comorbidities that affect growth. This report describes long-term growth outcomes in a large cohort of patients with cloacal exstrophy and explores associated comorbidities. METHODS: Records of 71 patients with cloacal exstrophy who were treated between 1974 and 2015 were reviewed, and 62 patients with growth data from 2 to 20years of age were included. Genetic sex, gender of rearing, and all heights, weights, and comorbidities were noted for each patient. Height-for-age, weight-for-age, and body mass index z-scores (HAZ, WAZ, and BMIZ) were determined using US Centers for Disease Control 2000 growth data, and average patient z-scores were calculated. RESULTS: There were 904 height and 1301 weight measurements available for 62 patients. 31 were genetically 46,XY, 21 of whom underwent gonadectomy in infancy and were raised female. 46,XX patients, 46,XY male patients, and 46,XY female patients all had median HAZ and WAZ substantially lower than the general population, with median HAZ less than -2, while maintaining normal BMIZ. Short bowel syndrome and enterocystoplasty with intestine were associated with lower z-scores for all parameters. CONCLUSIONS: Patients with cloacal exstrophy have significant multifactorial long-term growth failure. These benchmark data can be used to further optimize management. LEVEL OF EVIDENCE: 2b.

Hospital transfers and patterns of mortality in very low birth weight neonates with surgical necrotizing enterocolitis.

PURPOSE: The objectives of this study were to evaluate mortality rates in very low birth weight (VLBW) infants with surgical necrotizing enterocolitis (NEC) by level of available surgical resources and to determine the effect of hospital transfer on mortality. METHODS: Mortality among 4328 VLBW neonates with surgical NEC born 2009-2013 was assessed using the Vermont Oxford Network database. NICUs were classified by availability of resources as a marker of overall center capability: type A (restrictions on ventilation or do not routinely perform major neonatal surgery), type B (perform major neonatal surgery but not cardiac bypass), and type C (perform major surgery, including cardiac bypass in infants). RESULTS: Mortality was higher among those who had surgery at type B centers versus type C centers (44.3% vs 36.4%, adjusted prevalence ratio 1.20 (95% CI: 1.08, 1.33)). Neonates who were not transferred between birth and surgery had a higher mortality compared to those transferred (44.6% vs 31.6%, adjusted prevalence ratio 1.39 (95% CI: 1.25, 1.55)). CONCLUSION: Transfer between birth and surgery and a higher level of surgical resources at the operative center were associated with lower mortality. Early transfer of high risk neonates to centers with higher levels of surgical resources may be warranted.

Factors Affecting Spontaneous Closure of Gastrocutaneous Fistulae After Removal of Gastrostomy Tubes in Children With Intestinal Failure.

BACKGROUND: Children with intestinal failure (IF) frequently require gastrostomy tubes (GTs) for long-term nutrition support. Risk factors for persistent gastrocutaneous fistulae (GCFs) in pediatric patients with IF are largely unknown but may include underlying nutrition status and duration of indwelling GT. MATERIALS AND METHODS: Records of patients with IF having undergone GT removal and allowed a trial at spontaneous closure were reviewed. Nonparametric continuous variables were analyzed using the Wilcoxon rank sum test. Post hoc analysis was performed to identify the optimal threshold of GT duration predicting probability of spontaneous closure identified using receiver operating characteristic curve analysis. RESULTS: Fifty-nine children with IF undergoing GT removal were identified. Spontaneous closure occurred in 36 (61%) sites, while 23 (39%) underwent operative closure at a median 67 days after GT removal. The duration of indwelling GT was significantly shorter in the spontaneous closure group (11.5 vs 21 months, P = .002). Of 33 GT indwelling for ≤ 18 months, 28 (85%) closed spontaneously, compared with only 9 of 26 (35%) with duration >18 months (P < .001). With GCF persisting beyond 7 days, only 21% (6/28) of sites closed spontaneously, but this dropped to 6% (1/18) of cases with concurrent GT duration >18 months. CONCLUSIONS: Of the risk factors evaluated, only prolonged GT duration was associated with an increased likelihood of failure to close spontaneously. It is significantly less likely in pediatric patients with IF in whom GCF persists >7 days, particularly if the duration of GT is >18 months. Relatively earlier operative closure should be considered in this group.

Metabolic bone disease in pediatric intestinal failure patients: prevalence and risk factors.

PURPOSE: Patients with intestinal failure (IF) are known to have impaired absorption of nutrients required for maintenance of skeletal mass. Rates and risk factors of low bone mineral density (BMD) are unknown in pediatric IF patients. METHODS: Following IRB approval, patients with IF having undergone DXA scans were identified and laboratory, clinical, and nutritional intake variables were recorded. Low BMD was defined by a z-score of less than or equal to -2.0. Univariate followed by multivariable regression analysis was performed. RESULTS: Sixty-five patients underwent a total of 99 routine DXA scans. Twenty-seven (41%) had vitamin D deficiency, 22 (34%) had low BMD, and nineteen (29%) had a history of fractures. Variables noted to be associated with low BMD (p<0.1) on univariate analysis were considered for multivariable regression. Multivariable regression identified WAZ and serum calcium levels (p<0.05) as independent predictors of low BMD z-score. None of the other evaluated factors were associated with the risk of low BMD. Low BMD was not associated with risk of fractures. CONCLUSION: There is a significant incidence of low BMD in children with IF. WAZ and lower serum calcium levels are associated with risk of low BMD. Additional long term prospective studies are needed to further characterize the risk factors associated with low BMD.

Extraluminal distraction enterogenesis using shape-memory polymer.

PURPOSE: Although a few techniques for lengthening intestine by mechanical stretch have been described, they are relatively complex, and the majority involve placement of an intraluminal device. Ideally, techniques applicable to humans would be easy to perform and extraluminal to avoid the potential for mucosal injury. This study of distraction enterogenesis used an extraluminal, radially self-expanding shape-memory polymer cylinder and a simple operative approach to both elongate intestine and grow new tissue. METHODS: Young Sprague Dawley rats (250-350 g) underwent Roux-en-Y isolation of a small intestinal limb and were divided in three groups: no further manipulation (Control 1, C1); placement of a nonexpanding device (Control 2, C2); or placement of a radially expanding device by the limb (Experimental, Exp). For C2 and Exp animals, the blind end of the limb was wrapped around the radially expanding cylindrical device with the limb-end sutured back to the limb-side. Bowel length was measured at operation and at necropsy (14 days) both in-situ and ex-vivo under standard tension (6g weight). Change in length is shown as mean ± standard deviation. A blinded gastrointestinal pathologist reviewed histology and recorded multiple measures of intestinal adaptation. The DNA to protein ratio was quantified as a surrogate for cellular proliferation. Changes in length, histologic measures, and DNA:protein were compared using analysis of variance, with significance set at P<0.05. RESULTS: The length of the Roux limb in situ increased significantly in Exp animals (n=8, 29.0 ± 5.8mm) compared with C1 animals (n=5, -11.2 ± 9.0mm, P<0.01). The length of the Roux limb ex vivo under standard tension increased in the Exp group (25.8 ± 4.2mm) compared with the C2 group (n=6, -4.3 ± 6.0, P<0.01). There were no differences in histologic measures of bowel adaptation between the groups, namely villous height and width, crypt depth, crypt density, and crypt fission rate (all P ≥ 0.08). Muscularis mucosal thickness was also not different (P=0.25). There was no difference in DNA:protein between groups (P=0.47). CONCLUSION: An extraluminally placed, radially expanding shape-memory polymer cylinder successfully lengthened intestine, without damaging mucosa. Lack of difference in muscularis thickness and a constant DNA:protein ratio suggests that this process may be related to actual growth rather than mere stretch. This study demonstrated a simple approach that warrants further study aiming at potential clinical applicability.

Operative indications in recurrent ileocolic intussusception.

BACKGROUND: Air-contrast enema (ACE) is standard treatment for primary ileocolic intussusception. Management of recurrences is less clear. This study aimed to delineate appropriate therapy by quantifying the relationship between recurrence and need for bowel resection, pathologic lead points (PLP), and complication rates. METHODS: After IRB approval, a single institution review of patients with ileocolic intussusception from 1997 to 2013 was performed, noting recurrences, outcomes, and complications. Fisher's exact and t-tests were used. RESULTS: Of 716 intussusceptions, 666 were ileocecal. Forty-four underwent bowel resection, with 29 PLPs and 9 ischemia/perforation. Recurrence after ACE occurred in 96 (14%). Recurrence did not predict PLP (P=0.25). Recurrence (≥3) was associated with higher resection rate (P=0.03), but not ischemia/perforation (P=0.75). ACE-related complications occurred in 4 (0.5%) patients. Successful initial ACE had 98% negative predictive value for resection and PLP (e.g., after successful ACE, 2% had resections, 2% PLP). After failed initial ACE, 36% received resection, and 23% had PLP (P<0.001). CONCLUSIONS: Recurrence is associated with a greater risk of resection but not PLP or ACE-complication. Failed ACE is associated with increased risk for harboring PLP and receiving resection. ACE should be the standard treatment in recurrent intussusception, regardless of number of recurrences.

Serious congenital heart disease and necrotizing enterocolitis in very low birth weight neonates.

BACKGROUND: Infants with serious congenital heart disease (CHD) appear to be at increased risk for necrotizing enterocolitis (NEC). This study aimed to quantify the incidence and mortality of NEC among very low birth weight (VLBW) neonates with serious CHD, and identify specific CHD diagnoses at the highest risk for developing NEC. STUDY DESIGN: Data were prospectively collected on 257,794 VLBW (401 to 1,500 g) neonates born from 2006 to 2011 and admitted to 674 Vermont Oxford Network US centers. Entries were coded for specific CHD diagnoses and reviewed for completeness and consistency. Survival was defined as alive in-hospital at 1 year or discharge. RESULTS: Of eligible neonates, 1,931 had serious CHD. Of these, 253 (13%) developed NEC (vs 9% in infants without CHD, adjusted odds ratio [AOR] 1.80, p<0.0001). Mortality for neonates with CHD and no NEC was 34%, vs 55% for those with CHD and NEC (p<0.0001). Both groups of CHD patients had higher mortality than infants with NEC without CHD (28%, p<0.0001). Although NEC mortality overall decreases with higher birth weight, mortality for NEC and CHD together does not. CONCLUSIONS: The incidence of NEC is significantly higher in VLBW neonates when CHD is present. The mortality of CHD and NEC together is substantially higher than that with each disease alone. Infants with atrioventricular canal appear to have higher risk for developing NEC than other CHD diagnoses. In addition to providing benchmark incidence and mortality data, these findings may have utility in the further study of the pathophysiology of NEC.

Magnitude of surgical burden associated with pediatric intestinal failure: a multicenter cohort analysis.

BACKGROUND: Pediatric intestinal failure (IF) patients require many surgical procedures over the course of their illness. The number and variety of surgical procedures, as well as patient characteristics associated with this burden of surgical procedures, remain largely unknown. METHODS: Data from a large, multicenter retrospective study of pediatric intestinal failure (PIFCON) were reviewed. Infants from 14 multidisciplinary IF programs were enrolled, with study entry defined as PN dependence for >60days. RESULTS: A total of 272 infants were followed for a median (IQR) of 33.5 (16.2, 51.5) months, during which time they underwent 4.0 (3.0, 6.0) abdominal surgical procedures. Intestinal resections were performed in 88/97 (92%) necrotizing enterocolitis patients versus 138/175 (80%) in non-NEC patients (P<0.05). Patients who underwent ≥5 operations had more septic events, compared to those who underwent ≤2 operations (3 (1, 6) versus 1 (0, 3), respectively, P<0.01). Patients treated at centers with transplantation capability had lower odds of undergoing >2 abdominal operations [OR 0.37 (95% CI: 0.21, 0.65)] after multivariable adjustment. CONCLUSIONS: Individual and center-specific characteristics may help determine surgical practices experienced by infants with IF. Further study may delineate additional details about the nature of these characteristics, with the goal of optimizing patient care and minimizing individual and overall healthcare burden.

Patterns of surgical practice in very low birth weight neonates born in the United States: a Vermont Oxford Network analysis.

BACKGROUND/PURPOSE: The distribution of surgical care of very low birth weight (VLBW) neonates among centers with varying specialized care remains unknown. This study quantifies operations performed on VLBW neonates nationally with respect to center type. METHODS: VLBW neonates born 2009-2012 were assessed using a prospectively collected multi-center database encompassing 80% of all VLBW neonates in the United States. Surgical centers were categorized based on availability of pediatric surgery (PS) and anesthesia (PA). RESULTS: 48,711 major procedures (29,512 abdominal operations) were performed on 24,318 neonates. Of all patients, 20,892 (85.9%) underwent surgery at centers with PS and PA available on site. 1663 (6.8%) patients were treated at centers with neither specialty on site. Neonates requiring complex operations were more likely to receive surgery at centers with both PS and PA on staff than those requiring non-complex operations (95.6% vs 93.6%). CONCLUSION: This study confirms that most operations on VLBW neonates in the U.S. are performed at centers with pediatric surgeons and anesthesiologists on staff. Further research is necessary, however, to elucidate why a significant minority of this challenging population continues to be managed at centers without pediatric specialists.

Surgical Safety Checklist compliance: a job done poorly!

BACKGROUND: The Surgical Safety Checklist (SSC) has been introduced as an effective tool for reducing perioperative mortality and complications. Although reported completion rates are high, objective compliance is not well defined. The purpose of this retrospective analysis is to determine SSC compliance as measured by accuracy and completion, and factors that can affect compliance. STUDY DESIGN: In September 2010, our institution implemented an adaptation of the World Health Organization's SSC in an effort to improve patient safety and outcomes. A tool was developed for objective evaluation of overall compliance (maximum score 40) that was an aggregate score of completion and accuracy (20 each). Random samples of SSCs were analyzed at specific, predefined, time points throughout the first year after implementation. Procedure start time, operative time, and case complexity were assessed to determine association with compliance. RESULTS: A total of 671 SSCs were analyzed. The participation rate improved from 33% (95 of 285) at week 1 to 94% (249 of 265) at 1 year (p < 0.0001, chi-square test). Mean overall compliance score was 27.7 (± 5.4 SD) of 40 possible points (69.3% ± 13.5% of total possible score; n = 671) and did not change over time. Although completion scores were high (16.9 ± 2.7 out of 20 [84.5% ± 13.6%]), accuracy was poor (10.8 ± 3.4 out of 20 [54.1% ± 16.9%]). Overall compliance score was significantly associated with case start-time (p < 0.05), and operative time and case complexity showed no association. CONCLUSIONS: Our data indicate that although implementation of an SSC results in a high level of overall participation and completion, accuracy remained poor. Identification of barriers to effective use is needed, as improper checklist use can adversely affect patient safety.