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We evaluate the incidence, outcomes, and management of peri‑umbilical hernias after sutured or sutureless gastroschisis closure.A retrospective, longitudinal follow-up of neonates with gastroschisis who underwent closure at 11 children's hospitals from 2013 to 2016 was performed. Patient encounters were reviewed through 2019 to identify the presence of a peri‑umbilical hernia, time to spontaneous closure or repair, and associated complications.Of 397 patients, 375 had follow-up data. Sutured closure was performed in 305 (81.3%). A total of 310 (82.7%) infants had uncomplicated gastroschisis. Peri-umbilical hernia incidence after gastroschisis closure was 22.7% overall within a median follow-up of 2.5 years [IQR 1.3,3.9], and higher in those with uncomplicated gastroschisis who underwent primary vs. silo assisted closure (53.0% vs. 17.2%, p0.001). At follow-up, 50.0% of sutureless closures had a persistent hernia, while 16.4% of sutured closures had a postoperative hernia of the fascial defect (50.0% vs. 16.4%, p0.001). Spontaneous closure was observed in 38.8% of patients within a median of 17 months [9,26] and most frequently observed in those who underwent a sutureless primary closure (52.2%). Twenty-seven patients (31.8%) underwent operative repair within a median of 13 months [7,23.5]. Rate and interval of spontaneous closure or repair were similar between the sutured and sutureless closure groups, with no difference between those who underwent primary vs. silo assisted closure.Peri-umbilical hernias after sutured or sutureless gastroschisis closure may be safely observed similar to congenital umbilical hernias as spontaneous closure occurs, with minimal complications and no additional risk with either closure approach.Level II.

Disparities in surgical management have been documented across a range of disease processes. The objective of this study was to investigate sociodemographic disparities in young females undergoing excision of a breast mass.A retrospective study of females aged 10-21 y who underwent surgery for a breast lesion across eleven pediatric hospitals from 2011 to 2016 was performed. Differences in patient characteristics, workup, management, and pathology by race/ethnicity, insurance status, median neighborhood income, and urbanicity were evaluated with bivariate and multivariable regression analyses.A total of 454 females were included, with a median age of 16 y interquartile range (IQR: 3). 44% of patients were nonHispanic (NH) Black, 40% were NH White, and 7% were Hispanic. 50% of patients had private insurance, 39% had public insurance, and 9% had other/unknown insurance status. Median neighborhood income was $49,974, and 88% of patients resided in a metropolitan area. NH Whites have 4.5 times the odds of undergoing preoperative fine needle aspiration or core needle biopsy compared to NH Blacks (CI: 2.0, 10.0). No differences in time to surgery from the initial imaging study, size of the lesion, or pathology were observed on multivariable analysis.We found no significant differences by race/ethnicity, insurance status, household income, or urbanicity in the time to surgery after the initial imaging study. The only significant disparity noted on multivariable analysis was NH White patients were more likely to undergo preoperative biopsy than were NH Black patients; however, the utility of biopsy in pediatric breast masses is not well established.

To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs.Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.

Firearm sales in the United States (U.S.) markedly increased during the COVID-19 pandemic. Our objective was to determine if firearm injuries in children were associated with stay-at-home orders (SHO) during the COVID-19 pandemic. We hypothesized there would be an increase in pediatric firearm injuries during SHO.This was a multi institutional, retrospective study of institutional trauma registries. Patients18 years with traumatic injuries meeting National Trauma Data Bank (NTDB) criteria were included. A "COVID" cohort, defined as time from initiation of state SHO through September 30, 2020 was compared to "Historical" controls from an averaged period of corresponding dates in 2016-2019. An interrupted time series analysis (ITSA) was utilized to evaluate the association of the U.S. declaration of a national state of emergency with pediatric firearm injuries.Nine Level I pediatric trauma centers were included, contributing 48,111 pediatric trauma patients, of which 1,090 patients (2.3%) suffered firearm injuries. There was a significant increase in the proportion of firearm injuries in the COVID cohort (COVID 3.04% vs. Historical 1.83%; p 0.001). There was an increased cumulative burden of firearm injuries in 2020 compared to a historical average. ITSA showed an 87% increase in the observed rate of firearm injuries above expected after the declaration of a nationwide emergency (p 0.001).The proportion of firearm injuries affecting children increased during the COVID-19 pandemic. The pandemic was associated with an increase in pediatric firearm injuries above expected rates based on historical patterns.

Anastomotic stricture is the most common complication after esophageal atresia (EA) repair. We sought to determine if postoperative acid suppression is associated with reduced stricture formation.A prospective, multi-institutional cohort study of infants undergoing primary EA repair from 2016 to 2020 was performed. Landmark analysis and multivariate Cox regression were used to explore if initial duration of acid suppression was associated with stricture formation at hospital discharge (DC), 3-, 6-, and 9-months postoperatively.Of 156 patients, 79 (51%) developed strictures and 60 (76%) strictures occurred within three months following repair. Acid suppression was used in 141 patients (90%). Landmark analysis showed acid suppression was not associated with reduction in initial stricture formation at DC, 3-, 6- and 9-months, respectively (p = 0.19-0.95). Multivariate regression demonstrated use of a transanastomotic tube was significantly associated with stricture formation at DC (Hazard Ratio (HR) = 2.21 (95% CI 1.24-3.95, p0.01) and 3-months (HR 5.31, 95% CI 1.65-17.16, p0.01). There was no association between acid suppression duration and stricture formation.No association between the duration of postoperative acid suppression and anastomotic stricture was observed. Transanastomotic tube use increased the risk of anastomotic strictures at hospital discharge and 3 months after repair.

The impact of thoracoscopic surgery on outcomes in children with congenital lung malformations (CLM) remains controversial. The purpose of this study was to determine the effect of operative approach on perioperative outcomes in infants undergoing lobectomy for an asymptomatic CLM.After IRB approval, a retrospective cohort study was conducted on 506 children with a CLM resected at one of eleven children's hospitals over a seven-year period. Infants undergoing elective lobectomy were identified, and covariates were balanced based on operative approach using propensity scores with full matching. Outcomes were analyzed based on intention to treat with weighted conditional regression.One hundred seventy-five infants met inclusion criteria. There were 67 (38.3%) open, 89 (50.9%) thoracoscopic, and 19 (10.9%) thoracoscopic-converted-to-open lobectomies. Thoracoscopic lobectomy was associated with significantly longer operative times (26 min, 95% CI 6-47 min, p = 0.012) but used less epidural anesthesia (OR 0.02, 95% CI 0.004-0.11, p0.001) when compared to open lobectomy. There were no significant differences in intraoperative blood loss, postoperative complications, chest tube duration, or length of stay.Thoracoscopy has become the most common operative approach for elective lobectomy in infants with asymptomatic CLMs. The non-inferiority of thoracoscopic lobectomy in postoperative outcomes supports its continued use as an alternative to open lobectomy.Treatment study, Level III.

Perianal fistulas are among the most severe complications of Crohn disease, but limited data regarding their outcomes are available in children. Our objective was to determine predictors of perianal fistula healing among pediatric patients newly diagnosed with Crohn disease.This single-center retrospective study followed patients with perianal fistulas at Crohn disease diagnosis until fistula healing. Time to healing was analyzed using Cox proportional hazard regression models considering relevant covariates including patient demographics, disease characteristics, medical therapies [no anti-tumor necrosis factor (TNF)α therapy, anti-TNFα therapy ±therapeutic drug monitoring], and perianal surgical procedures including fistulotomy, fistulectomy, removal of perianal lesions, seton placement, and incision and drainage.Of 485 patients identified, 107 (22%) had a perianal fistula at Crohn disease diagnosis. Multivariate analysis identified that perianal fistulotomy, fistulectomy, and lesion removal [hazard ratio (HR) 0.46; P = 0.028], non-White race (HR 0.30, P0.01), and male sex (HR 0.42; P = 0.02) were associated with delayed fistula healing. Faster fistula healing was associated with treatment with anti-TNFα with therapeutic drug monitoring (HR 1.78, P = 0.009). There were no other differences in healing by treatment.Fistulotomy, fistulectomy, and perianal lesion removal as well as non-White race were associated with delayed fistula healing. Anti-TNFα therapy was associated with faster fistula healing when combined with therapeutic drug monitoring, compared to all other medical treatment groups, including anti-TNFα therapy without therapeutic drug monitoring.

Background The objective of our study was to describe the workup, management, and outcomes of pediatric patients with breast masses undergoing operative intervention. Materials and methods A retrospective cohort study was conducted of girls 10-21 y of age who underwent surgery for a breast mass across 11 children's hospitals from 2011 to 2016. Demographic and clinical characteristics were summarized. Results Four hundred and fifty-three female patients with a median age of 16 y (IQR: 3) underwent surgery for a breast mass during the study period. The most common preoperative imaging was breast ultrasound (95%); 28% reported the Breast Imaging Reporting and Data System (BI-RADS) classification. Preoperative core biopsy was performed in 12%. All patients underwent lumpectomy, most commonly due to mass size (45%) or growth (29%). The median maximum dimension of a mass on preoperative ultrasound was 2.8 cm (IQR: 1.9). Most operations were performed by pediatric surgeons (65%) and breast surgeons (25%). The most frequent pathology was fibroadenoma (75%); 3% were phyllodes. BI-RADS scoring ≥4 on breast ultrasound had a sensitivity of 0% and a negative predictive value of 93% for identifying phyllodes tumors. Conclusions Most pediatric breast masses are self-identified and benign. BI-RADS classification based on ultrasound was not consistently assigned and had little clinical utility for identifying phyllodes.

The prevalence and natural history of patent processus vaginalis (PPV) are unknown. An interim analysis was performed of a multi-institutional, prospective, observational study in neonates undergoing laparoscopic pyloromyotomy during which bilateral inguinal canals were evaluated.Infants under 4 months undergoing laparoscopic pyloromyotomy were enrolled at 8 children's hospitals. The presence of a PPV was evaluated and measurements recorded. Patients with a PPV are undergoing annual phone follow-up to 18 years of age. Interim analysis was performed.In a cohort of 610 patient, 80 did not have a PPV examined, 4 had consent issues and were excluded, leaving 526 patients. Of these, 433 (82%) were male, median age 1.2 months (IQR 0.9, 1.6), median weight 3.89 kg (IQR 3.4, 4.46), and EGA 39 weeks (IQR 37, 40). There were 283 PPVs, 132 bilateral (47%), 116 right (41%), and 35 left (12%). Patients with a PPV were significantly younger (1.1 months (IQR 0.9, 1.5) vs 1.3 months (IQR 0.9, 1.7), p=0.02), weighed less (3.76kg (IQR 3.35, 4.26) vs 3.9kg (IQR 3.4, 4.5) p=0.03) and had a significantly lower EGA at birth (38 weeks (IQR 37, 40) vs 39 weeks (IQR 38, 40) p=0.003). Of 246 eligible infants, 208 (85%) responded to at least one annual follow-up. Two patients had an inguinal hernia repair for a symptomatic hernia, 49- and 51-days post pyloromyotomy. One had an orchiopexy and incidental inguinal hernia repair 120 days post pyloromyotomy; for a total of 3 (1.2%) hernia repairs. No additional hernias were identified in 116 patients with the PPV patients who have been followed for1 year.The presence of a PPV at the time of pyloromyotomy is common but the need for hernia repair is rare within the first year of life. Continued long-term longitudinal follow-up of this cohort is needed.II.

The ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear.To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children.This retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020.Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors.Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%.This study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left–right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left–right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.

The use of extracorporeal life support (ECLS) for the pediatric and neonatal population continues to grow. At the same time, there have been dramatic improvements in the technology and safety of ECLS that have broadened the scope of its application. This article will review the evolving landscape of ECLS, including its expanding indications and shrinking contraindications. It will also describe traditional and hybrid cannulation strategies as well as changes in circuit components such as servo regulation, non-thrombogenic surfaces, and paracorporeal lung-assist devices. Finally, it will outline the modern approach to managing a patient on ECLS, including anticoagulation, sedation, rehabilitation, nutrition, and staffing.

In a time when social isolation heavily relies on the use of digital representation, photography, and e-books, it is easy to take for granted the impact imagery has on our society and the pedagogical purposes of illustration, particularly in the teaching of surgery. Illustrations after all are the basis of all anatomical atlases and are quintessential tools that allow for an expedient and thorough understanding of concepts underlying the fabric of the human body. Yet, surgery has not always been taught with an atlas. Illustrations, much like surgery, have seen an incredible transformative process spanning across the ages to achieve their status in modern medicine. Through this brief review, we will not only glean an understanding of the evolution of anatomical illustrations but also the social context in which surgery has also evolved throughout history.

OBJECTIVE To assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. SUMMARY BACKGROUND DATA Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery have not been well defined. METHODS A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at eleven children's hospitals from 2009-2016. Statistical analyses were performed using non-parametric bivariate or multivariable logistic regression. RESULTS Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (p < 0.001). An initial CVR ≤ 1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR < 0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth. CONCLUSION In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.

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

The key landmark for tip position of a central venous catheter (CVC) is the SVC-RA junction. In adults, localization of the SVC-RA junction may be assessed as a function of vertebral body units (VBU) below the carina during CVC placement. We investigated the relationship between the SVC-RA junction and the carina in children. 584 CT scans of 0–18 years were analyzed. The carina was marked automatically by software while the SVC-RA junction and vertebrae were marked manually. The SVC-RA junction to carina (JC) distance was the primary study measurement reported in both VBU and mm. The data show an average JC distance of 1.25 VBU for 0–1 year, 1.27 VBU for 1–4 years, 1.34 VBU for 4–9 years, 1.53 VBU for 9–15 years, and 1.64 VBU for 15–18 years. A positive relationship between weight and JC distance was also demonstrated. JC distance is a useful predictor of SVC-RA junction location in children. Significant relationships were shown between JC distance and both age and weight. Due to small differences between age groups, however, average JC distance for all comers (1.48 VBU, 95% CI 0.7 – 2.3) can be used for SVC-RA junction identification in CVC placement.

Introduction The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. Methods After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. Results Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1–12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6–11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). Conclusion This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients. Level of evidence Level II.

To examine whether a service guideline reducing postoperative opioid prescription quantities and caregiver-reported education to use nonopioid analgesics first are associated with caregiver-reported pain control after pediatric tonsillectomy.Prospective cohort study (July 2018-April 2019).Pediatric otolaryngology service at a tertiary academic children's hospital.Caregivers of patients aged 1 to 11 years undergoing tonsillectomy (N = 764) were surveyed 7 to 21 days after surgery regarding pain control, education to use nonopioid analgesics first, and opioid use. Respondents who were not prescribed opioids or had missing data were excluded. Logistic regression modeled caregiver-reported pain control as a function of service guideline implementation (December 2018) recommending 20 rather than 30 doses for postoperative opioid prescriptions and caregiver-reported analgesic education, adjusting for patient demographics.Among 430 respondents (56% response), 387 patients were included. The sample was 43% female with a mean age of 5.0 years (SD, 2.5). Pain control was reported as good (226 respondents, 58%) or adequate/poor (161 respondents, 42%). Mean opioid prescription quantity was 27 doses (SD, 7.9) before and 21 doses (SD, 6.1) after guideline implementation (Caregiver education to use nonopioid analgesics first may be a modifiable health care practice to improve pain control as postoperative opioid prescription quantities are reduced.

Initial waves of the COVID-19 pandemic have largely spared children. With the advent of vaccination in many older age groups and the spread of the highly contagious Delta variant, however, children now represent a growing percentage of COVID-19 cases. PICU capacity is far less than that of adult ICUs. Adult ICUs may need to support pediatric care, much as PICUs provided adult care earlier in the pandemic. Critically ill children selected for care in adult settings should be at least 12 years of age and ideally have conditions common in children and adults alike (eg, community-acquired sepsis, trauma). Children with complex, pediatric-specific disorders are best served in PICUs and are not recommended for transfer. The goal of such transfers is to maintain critical capacity for those children in greatest need of the PICU's unique abilities, therefore preserving systems of care for all children.

It is rare for children to receive more than one course of support with extracorporeal membrane oxygenation, and in those who do undergo multiple episodes, the interval is usually days to weeks between events. Little data exists on re-cannulation years after an initial extracorporeal membrane oxygenation run, and late repeat cannulation can pose unique challenges. We report the case of a 10-year-old male patient with right jugular vein occlusion due to a previous course of extracorporeal membrane oxygenation as a neonate, who was successfully supported via central cannulation. This case demonstrates the importance of adequate imaging of target vasculature prior to attempting re-cannulation of a previously used vessel. Establishing a thoughtful strategy for late repeat cannulation is essential to achieve safe access in unusual and challenging situations.

Purpose Chest tube (CT) management for pediatric primary spontaneous pneumothorax (PSP) is associated with long hospital stays and high recurrence rates. To streamline management, we explored simple aspiration as a test to predict need for surgery. Methods A multi-institution, prospective pilot study of patients with first presentation for PSP at 9 children’s hospitals was performed. Aspiration was performed through a pigtail catheter, followed by 6 h observation with CT clamped. If pneumothorax recurred during observation, the aspiration test failed and subsequent management was per surgeon discretion. Results Thirty-three patients were managed with simple aspiration. Aspiration was successful in 16 of 33 (48%), while 17 (52%) failed the aspiration test and required hospitalization. Twelve who failed aspiration underwent CT management, of which 10 (83%) failed CT management owing to either persistent air leak requiring VATS or subsequent PSP recurrence. Recurrence rate was significantly greater in the group that failed aspiration compared to the group that passed aspiration [10/12 (83%) vs 7/16 (44%), respectively, P = 0.028]. Conclusion Simple aspiration test upon presentation with PSP predicts chest tube failure with 83% positive predictive value. We recommend changing the PSP management algorithm to include an initial simple aspiration test, and if that fails, proceed directly to VATS. Type of study Prospective pilot study Level of evidence Level III.

Background The practice of “cutting-away” from venoarterial extracorporeal life support (ECLS) and leaving indwelling heparinized cannulas prior to decannulation is controversial. This study aims to determine the safety and efficacy of this strategy in patients with congenital diaphragmatic hernia (CDH) who require ECLS. Methods A single-center retrospective review of electronic health records was performed on all patients with CDH who underwent elective ECLS decannulation between January 2014 and September 2018. Descriptive statistics are presented as medians with interquartile range. Results Seventy-three percent (19/26) of patients who underwent venoarterial ECLS for CDH were electively decannulated. After a median ECLS run of 10.7 days [6.1–19.5], patients were “cut-away” for a median of 26 h [19.8–43] prior to decannulation. One patient required re-initiation at 36 h for a pulmonary hypertensive crisis (5%). There were no major bleeding or embolic events while “cut-away”, and four (21%) patients had clots removed from the cannulas without clinical sequelae. One patient was recannulated 16 days following initial decannulation. Conclusions Our data suggests that “cutting-away” from ECLS in patients with congenital diaphragmatic hernia is safe and allows a period of observation without significant complications. This strategy may be particularly helpful in patients at risk for recannulation, but better prognostic criteria are needed. Level of Evidence Level IV. Type of Study Treatment Study.

Objectives To update the American Academy of Pediatrics and Society of Critical Care Medicine's 2004 Guidelines and levels of care for PICU. Design A task force was appointed by the American College of Critical Care Medicine to follow a standardized and systematic review of the literature using an evidence-based approach. The 2004 Admission, Discharge and Triage Guidelines served as the starting point, and searches in Medline (Ovid), Embase (Ovid), and PubMed resulted in 329 articles published from 2004 to 2016. Only 21 pediatric studies evaluating outcomes related to pediatric level of care, specialized PICU, patient volume, or personnel. Of these, 13 studies were large retrospective registry data analyses, six small single-center studies, and two multicenter survey analyses. Limited high-quality evidence was found, and therefore, a modified Delphi process was used. Liaisons from the American Academy of Pediatrics were included in the panel representing critical care, surgical, and hospital medicine expertise for the development of this practice guidance. The title was amended to "practice statement" and "guidance" because Grading of Recommendations, Assessment, Development, and Evaluation methodology was not possible in this administrative work and to align with requirements put forth by the American Academy of Pediatrics. Methods The panel consisted of two groups: a voting group and a writing group. The panel used an iterative collaborative approach to formulate statements on the basis of the literature review and common practice of the pediatric critical care bedside experts and administrators on the task force. Statements were then formulated and presented via an online anonymous voting tool to a voting group using a three-cycle interactive forecasting Delphi method. With each cycle of voting, statements were refined on the basis of votes received and on comments. Voting was conducted between the months of January 2017 and March 2017. The consensus was deemed achieved once 80% or higher scores from the voting group were recorded on any given statement or where there was consensus upon review of comments provided by voters. The Voting Panel was required to vote in all three forecasting events for the final evaluation of the data and inclusion in this work. The writing panel developed admission recommendations by level of care on the basis of voting results. Results The panel voted on 30 statements, five of which were multicomponent statements addressing characteristics specific to PICU level of care including team structure, technology, education and training, academic pursuits, and indications for transfer to tertiary or quaternary PICU. Of the remaining 25 statements, 17 reached consensus cutoff score. Following a review of the Delphi results and consensus, the recommendations were written. Conclusions This practice statement and level of care guidance manuscript addresses important specifications for each PICU level of care, including the team structure and resources, technology and equipment, education and training, quality metrics, admission and discharge criteria, and indications for transfer to a higher level of care. The sparse high-quality evidence led the panel to use a modified Delphi process to seek expert opinion to develop consensus-based recommendations where gaps in the evidence exist. Despite this limitation, the members of the Task Force believe that these recommendations will provide guidance to practitioners in making informed decisions regarding pediatric admission or transfer to the appropriate level of care to achieve best outcomes.

Introduction The purpose of this study was to evaluate short-term respiratory outcomes in neonates with symptomatic congenital lung malformations (CLM). Methods Consecutive newborns who underwent surgical resection of a CLM were retrospectively reviewed. Demographic, prenatal, and outcomes data were analyzed as appropriate (p Results Twenty-one neonates were managed at a median gestational age of 36.2 weeks [interquartile range (IQR), 33.8–39.0]. Endotracheal intubation was required in 14 (66.7%) for a median of 7.5 days [interquartile range (IQR), 3.0–25.8]. Three (14.3%) children underwent ex utero intrapartum treatment-to-resection, and another 14 (66.7%) had neonatal lung resections performed at a median age of 2.0 days (IQR, 0.08–19.5 days). Excluding one patient who received comfort care at birth, all neonates survived to hospital discharge with a median length of hospitalization of 36.5 days (IQR, 23.8–56.5). More than one-quarter were discharged on supplemental oxygen by nasal cannula. Based on a median follow up of 35.5 months (IQR, 19.0–80.8), CLM-related morbidity was still evident in 55.0%. Conclusion Our study suggests a high incidence of complications and chronic respiratory morbidity after neonatal lung resection for symptomatic CLMs. These data highlight the need to provide realistic expectations in perinatal counseling discussions with families and the importance of coordinating appropriate multidisciplinary follow up for these children. Level of Evidence: Level IV.

Background Use of ultrasound-guidance for central venous access in adults is the standard of care. There is, however, less clarity in the role of routine ultrasound use in obtaining venous access in children. We sought to evaluate safety and efficiency of the placement of central lines utilizing an ultrasound-guided approach compared to the traditional, landmark approach in pediatric patients. Study design A single-institution retrospective chart review, using CPT codes, was performed for all tunneled central venous catheters in children between 2005 and 2017 by the same pediatric surgery group. During the study period, a practice change occurred from exclusively landmark-based line placement to ultrasound-guided line placement. Groups were divided into three phases: a traditional/landmark era (Phase 1), transitional period (Phase 2), and the ultrasound era (Phase 3). The primary outcomes analyzed were postoperative chest tube insertions and operative time. Results A total of 2010 tunneled central lines were included for analysis: Phase 1 (N = 930), Phase 2 (N = 313) and Phase 3 (N = 767). Venous access for chemotherapy was the most common indication (29%). Phase 1 had a chest tube placement rate of 9.7/1000 procedures, while Phase 2 had a rate of 6.4/1000 procedures, and Phase 3 had no chest tube insertions (p = 0.009). Phase 1 had longer OR times compared to Phase 2 (57 vs. 49, p = 0.0026) and Phase 3 (57 vs. 46 min, p Conclusions This study represents the largest analysis of ultrasound-guided access for children. A complete practice transition to the ultrasound-guided approach was feasible within a two-year period. The ultrasound-guided approach had a shorter operative time and less chest tube insertions than the traditional, landmark technique in children. Level III evidence.

Traditionally, children presenting with appendicitis are referred for urgent appendectomy. Recent improvements in the quality and availability of diagnostic imaging allow for better pre-operative characterization of appendicitis, including severity of inflammation; size of the appendix; and presence of extra-luminal inflammation, phlegmon, or abscess. These imaging advances, in conjunction with the availability of broad spectrum oral antibiotics, allow for the identification of a subset of patients with uncomplicated appendicitis that can be successfully treated with antibiotics alone. Recent studies demonstrated that antibiotics alone are a safe and efficacious treatment alternative for patents with uncomplicated appendicitis. The objective of this study is to perform a multi-institutional trial to examine the effectiveness of non-operative management of uncomplicated pediatric appendicitis across a group of large children’s hospitals. A prospective patient choice design was chosen to compare non-operative management to surgery in order to assess effectiveness in a broad population representative of clinical practice in which non-operative management is offered as an alternative to surgery. The risks and benefits of each treatment are very different and a “successful” treatment depends on which risks and benefits are most important to each patient and his/her family. The patient-choice design allows for alignment of preferences with treatment. Patients meeting eligibility criteria are offered a choice of non-operative management or appendectomy. Primary outcomes include determining the success rate of non-operative management and comparing differences in disability days, and secondarily, complication rates, quality of life, and healthcare satisfaction, between patients choosing non-operative management and those choosing appendectomy.

The purpose of this review is to explore the effects of the opioid crisis on pediatric patients in the postoperative setting and provide recommendations for well-tolerated opioid prescribing practices.Opioid overdoses have increased among all age groups, predominantly related to overprescribing and accessibility of opioids in the home. Adverse risks of prescribed opioids include respiratory depression, gastrointestinal distress, accidental ingestion, intentional misuse, new chronic use, diversion to another user, and overdose. Well-tolerated opioid prescribing practices include multimodal pain management; prescribing guided by patient need; risk assessment for potential misuse; and comprehensive patient and family education on risks, safe storage, and disposal practices. Evolving state laws will affect varying institutional policies; thus, providers must ensure their prescribing practices are current and compliant.All age groups have been affected by the opioid crisis, including children and adolescents. When managing postoperative pain, clinicians must balance appropriate pain management with well-tolerated opioid stewardship to minimize harm related to postoperative care.

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

Necrotizing enterocolitis occurs in 14% of infants less than 1000 g. Preoperative management varies widely, and the only absolute indication for surgery is pneumoperitoneum. Multiple biomarkers and scoring systems are under investigation, but clinical practice is still largely driven by surgeon judgment. Outcomes in panintestinal disease are poor, and multiple creative approaches are used to preserve bowel length. Overall, recovery is complicated in the short and long term. Major sequelae are stricture, short gut syndrome, and neurodevelopmental impairment. Resolving controversies in surgical necrotizing enterocolitis care requires multicenter collaboration for centralized data and tissue repositories, benchmarking, and carrying out prospective randomized controlled trials.

Background Congenital chylous ascites poses a significant challenge in neonatal care, and often results in prolonged, complex hospital stays and increased mortality. Few effective options exist in refractory cases. Methods Patients aged 0 to 12 months with refractory chylous ascites underwent retroperitoneal exploration after medical treatment and minimally invasive therapies were unsuccessful. The retroperitoneum was completely exposed via left and right medial visceral rotation and opening the lesser sac. Visible leaks were ligated, and alternating layers of fibrin glue and Vicryl mesh were used to cover the entire retroperitoneum. Results All 4 patients had resolution of their chylous ascites. None required reoperation or reintervention for chyle leaks. All achieved goal enteral feeds at a median of 29 days postoperatively and were discharged from hospital at a median of 42 days postoperatively. Conclusions Management of chylous ascites is extremely challenging in refractory cases. Complete retroperitoneal exposure with fibrin glue and Vicryl mesh application offers a definitive, reliable therapy for achieving cessation of lymphatic leakage and ultimate recovery for patients who fail all nonoperative approaches. Study type Therapeutic. Level of evidence IV

Surgical management for refractory ulcerative colitis (UC) has been restorative proctocolectomy (RP) with ileal-pouch-anal-anastomosis (IPAA) done as one to three stages, with safety and effectiveness of a single-stage operation unclear.Pediatric UC patients from 2004 to 2019 who underwent RP/IPAA in the initial operation were retrospectively reviewed. 1-stage operations were matched 1:2 to 2-stage operations using age, duration of disease, and disease severity.Ninety-nine patients (33 1-stage, 66 2-stage) were identified. The median total operative time was shorter in the 1-stage group (6 h:00 min vs. 7 h:47 min, p = 0.004). Total length of stay was shorter in the 1-stage group (9 vs. 17 days, p = 0.001). Rates of readmission were higher in 2-stage group (30 vs. 9%, p = 0.02). There was no difference in pouch leak rates (p = 1.00). Stricture rates were higher in the 2-stage group (50 vs. 16%, p = 0.005). Functional outcomes including pouchitis (p = 0.13), daily bowel movements (p = 0.37), and incontinence (p = 0.77) were all similar.Restorative proctocolectomy with IPAA in children with UC can be performed as a 1- or 2-stage operation with equivalent short-term, long-term, and functional outcomes in similar risk population. Our findings suggest 1-stage RP/IPAA operations without ileostomy are a safe alternative for patients considered for a 2-stage operation.

BACKGROUND: Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy. METHODS: A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children’s hospitals in the United States. Multivariable logistic regression was used to examine the relationship between preoperative characteristics and risk of malignancy, including pleuropulmonary blastoma (PPB). RESULTS: None of the 344 prenatally diagnosed lesions had malignant pathology (P < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, n = 11; other PPB, n = 3; adenocarcinoma, n = 1) at a median age of 20.7 months (interquartile range, 7.9–58.1). Malignancy was associated with the DICER1 mutation in 8 (57%) PPB cases. No malignant lesion had a systemic feeding vessel (P = .0427). The sensitivity of preoperative chest computed tomography (CT) for detecting malignant pathology was 33.3% (95% confidence interval [CI]: 15.2–58.3). Multivariable logistic regression revealed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology (odds ratios of 42.15 [95% CI, 7.43–340.3; P < .0001] and 42.03 [95% CI, 3.51–995.6; P = .0041], respectively). CONCLUSIONS: In pediatric lung masses initially diagnosed after birth, the risk of PPB approached 10%. These results strongly caution against routine nonoperative management in this patient population. DICER1 testing may be helpful given the poor sensitivity of CT for identifying malignant pathology.

Background/purpose Fetal surgery is a growing field within pediatric surgery. We sought to understand practice patterns of pediatric surgeons who subspecialize in fetal surgery. Methods A survey was sent to all active non-trainee surgeons within the American Pediatric Surgical Association. Respondents were stratified based on self-reported fetal affiliations. Results Of 1015 surveys, we received 405 responses (40%). Fetal surgery practices were described by 77 self-designated fetal center members. Centers provide prenatal consultation (99%), diagnostic imaging (84%), care/delivery coordination (83%), and/or fetal surgery/procedures (52%). The majority (56%) of fetal programs are directed by surgery and maternal fetal medicine. Pediatric surgeons are represented on the fetal team in 96% of centers. Prenatal consultations are primarily seen by any pediatric surgeon in the group (53%), with the pediatric surgeon on call operating on/caring for the baby postnatally in the majority (64%), regardless of who performed the prenatal consultation. Only 29% of fetal center members performed a fetal operation in the last year. Yearly fetal case numbers vary widely per procedure, with the most common being complex twin procedures and needle-based sclerotherapy. Conclusions Fetal centers vary by services offered, team composition, and interventions performed, with few surgeons performing a wide range of fetal surgery. Level of evidence Level III

Introduction Children with fulminant ulcerative colitis(UC) traditionally undergo 2-stage operations: restorative-proctocolectomy(RP/IPAA) and ileostomy followed by ostomy closure. In the biologic era, surgeons have modified their strategy: initial subtotal-colectomy/diversion, followed by RP/IPAA without diversion. Yet, evidence on efficacy and functional outcomes with the “modified 2-stage” approach is limited in children. We sought to compare the timing of pouch creation in 2-stage operations to determine outcomes. Methods This is a retrospective study of children with UC undergoing either a traditional 2-stage RP/IPAA or modified 2-stage RP/IPAA between 2010 and 2019. Complications (leak, stricture, wound-infection) were recorded at 90-days and 1 year from 2nd operation. Results N = 57 (Traditional n = 40, Modified n = 17). Median time to surgery from consultation was shorter in the modified-group (7 vs.25 days, p = 0.01). Preoperatively, the modified-group had lower albumin(p = 0.01), higher CRP(p = 0.01), and more frequently took biologics within 90-daysp=0.001). After re-establishing intestinal continuity, stricture requiring dilation was higher in the traditional-group (59% vs.18%, p = 0.008). No difference in pouch leak (p = 0.38), bowel obstruction(p = 0.35), loperamide dose(p = 0.21), or incontinence(p = 0.38) was observed. Conclusion Delaying pouch creation to the second operation without a protective ileostomy as a modified 2-stage is safe in a sicker and more acute pediatric population.

Recent advances in ECLS technology have led to the adoption of centrifugal pumps for the majority of patients worldwide. Despite several advantages of centrifugal pumps, they remain controversial because a number of studies have shown increased rates of hemolysis. The aim of this study was to assess the impact of transitioning from roller to centrifugal pumps on hemolysis rates at our center. A retrospective analysis of all pediatric ECMO patients at a single center between 2005 and 2017 was undertaken. Hemolysis was defined as a plasma free hemoglobin >50 mg/dL. Multivariable logistic regression was performed correcting for several factors to determine risk factors for hemolysis and analyze outcomes among patients with hemolysis. Significant findings were those with p

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

Introduction The role of advanced care practitioners (ACPs) in pediatric surgery is increasingly important and not well described. Methods Electronic surveys were sent to pediatric surgery division chiefs within the Children's Hospital Association. Results We received 77/163 survey responses (47%). The median number of ACPs per service was 3.0 (range 0–35). ACP number correlated with inpatient census, surgeon number, case volume, trauma centers, intensive care unit status, and fellowship programs but not with presence of residents/hospitalists, hospital setting, or practice type. Nearly all programs incorporated nurse practitioners while almost half utilized physician assistants. Approximately one-third of ACPs were designated for subspecialties (35%) such as trauma and colorectal. Only 9% of centers had surgeon-specific ACPs. ACP responsibilities included both inpatient and outpatient tasks. Nearly all ACPs participated in procedures (89%), mostly bedside (80%). All ACPs worked daytime shifts, with less nights and weekends. Most ACPs billed for services (80%). Satisfaction with ACP coverage was widespread and did not correlate with ACP number. Most respondents felt that ACPs enhance, and not hinder, resident/fellow training (85%). Conclusion ACPs are useful adjuncts in pediatric surgery. A better understanding of practice patterns may help optimize utilization to enhance patient care and can be used to advocate for appropriate resources.

Acute pain after surgery is one of the most frequent indications for opioid prescribing in children. Opioids are often not stored or disposed of safely after their use, placing children and others in the home at risk for accidental ingestion or intentional misuse. We currently lack evidence-based guidelines for post-operative pain management after common ambulatory pediatric urologic procedures. Thus, each surgeon must decide if and how much opioid to prescribe based on his/her own assumptions of perceived post-operative pain.As part of an effort to establish opioid prescribing guidelines across two academic centers, the objectives of this study were to evaluate current variability in pediatric urologists' opioid prescribing factors and identify patients at greatest risk of being prescribed high doses of opioids after common ambulatory pediatric urologic procedures.We retrospectively evaluated post-operative opioid prescribing patterns after common ambulatory pediatric urology procedures (circumcision, orchiopexy, and hernia/hydrocele) at two major children's hospitals. Specifically, we evaluated if and how much opioid was prescribed for all children (18 years or younger) between 2016 and 2017. Bivariate analysis was performed using Kruskal-Wallis Test and Wilcoxon Rank Sum. Multivariable logistic regression was performed to determine patient, surgeon, and procedural factors that predicted the prescription of a high dose of opioids (greater than the median number of doses prescribed for that procedure).Over the two-year period, 811 circumcisions and 883 inguinal surgeries (inguinal orchiopexy and hernia/hydrocele) were performed. 94% of patients undergoing circumcision and 97% of those undergoing inguinal surgery were prescribed opioid analgesia. The median number of doses prescribed for circumcision was 20; for inguinal surgeries, 23.75% of patients received 15 opioid doses or more. Patients ages 0-2 years, who represented the largest age group (41% of all patients), received significantly more opioid doses than all other age groups, followed by those10 years (p 0.01). There was significant variation in opioid prescribing patterns by provider (p 0.01) (Figure 1) On multivariable logistic regression, younger age, pill form, and earlier year were all associated with a greater number of opioid doses prescribed for all surgeries.Across two institutions without a formal post-operative opioid prescribing policy for ambulatory pediatric urologic procedures, we observed considerable variability in provider prescribing patterns, with nearly all patients receiving an opioid, and those 0-2 years receiving the highest number of doses. This highlights the need for evidence-based guidelines for post-operative pain management after ambulatory pediatric urologic surgeries.

This study evaluated compliance with a multi-institutional quality improvement management protocol for Type-C esophageal atresia with distal tracheoesophageal fistula (EA/TEF).Compliance and outcomes before and after implementation of a perioperative protocol bundle for infants undergoing Type-C EA/TEF repair were compared across 11 children's hospitals from 1/2016-1/2019. Bundle components included elimination of prosthetic material between tracheal and esophageal suture lines during repair, not leaving a transanastomotic tube at the conclusion of repair (NO-TUBE), obtaining an esophagram by postoperative-day-5, and discontinuing prophylactic antibiotics 24 h postoperatively.One-hundred seventy patients were included, 40% pre-protocol and 60% post-protocol. Bundle compliance increased 2.5-fold pre- to post-protocol from 17.6% to 44.1% (p 0.001). After stratifying by institutional compliance with all bundle components, 43.5% of patients were treated at low-compliance centers (20%), 43% at medium-compliance centers (20-80%), and 13.5% at high-compliance centers (80%). Rates of esophageal leak, anastomotic stricture, and time to full feeds did not differ between pre- and post-protocol cohorts, though there was an inverse correlation between NO-TUBE compliance and stricture rate over time (ρ = -0.75, p = 0.029).Compliance with our multi-institutional management protocol increased 2.5-fold over the study period without compromising safety or time to feeds and does not support the use of transanastomotic tubes.Level II.Treatment Study.

Necrotizing enterocolitis (NEC) is a leading cause of morbidity and mortality in the neonatal ICU with minimal progress in the research.Federal webpages were queried to look for funding opportunity announcements (FOAs) and to develop lists of funded projects on NEC to identify gaps in NEC-related research topics.Over the past 30 years, the National Institutes of Health (NIH) issued two FOAs to stimulate research on NEC with $4.1 million set aside for the first year of respective funding. We identified 23 recently funded studies of which 18 were research projects, 4 training grants, and 1 conference grant support. Only one grant focused on parent and family engagement in the NICU.There are significant research gaps that can be addressed with adequate funding from the federal government on the prevention and treatment of NEC.

Importance Nonoperative management with antibiotics alone has the potential to treat uncomplicated pediatric appendicitis with fewer disability days than surgery. Objective To determine the success rate of nonoperative management and compare differences in treatment-related disability, satisfaction, health-related quality of life, and complications between nonoperative management and surgery in children with uncomplicated appendicitis. Design, Setting, and Participants Multi-institutional nonrandomized controlled intervention study of 1068 children aged 7 through 17 years with uncomplicated appendicitis treated at 10 tertiary children’s hospitals across 7 US states between May 2015 and October 2018 with 1-year follow-up through October 2019. Of the 1209 eligible patients approached, 1068 enrolled in the study. Interventions Patient and family selection of nonoperative management with antibiotics alone (nonoperative group, n = 370) or urgent (≤12 hours of admission) laparoscopic appendectomy (surgery group, n = 698). Main Outcomes and Measures The 2 primary outcomes assessed at 1 year were disability days, defined as the total number of days the child was not able to participate in all of his/her normal activities secondary to appendicitis-related care (expected difference, 5 days), and success rate of nonoperative management, defined as the proportion of patients initially managed nonoperatively who did not undergo appendectomy by 1 year (lowest acceptable success rate, ≥70%). Inverse probability of treatment weighting (IPTW) was used to adjust for differences between treatment groups for all outcome assessments. Results Among 1068 patients who were enrolled (median age, 12.4 years; 38% girls), 370 (35%) chose nonoperative management and 698 (65%) chose surgery. A total of 806 (75%) had complete follow-up: 284 (77%) in the nonoperative group; 522 (75%) in the surgery group. Patients in the nonoperative group were more often younger (median age, 12.3 years vs 12.5 years), Black (9.6% vs 4.9%) or other race (14.6% vs 8.7%), had caregivers with a bachelor’s degree (29.8% vs 23.5%), and underwent diagnostic ultrasound (79.7% vs 74.5%). After IPTW, the success rate of nonoperative management at 1 year was 67.1% (96% CI, 61.5%-72.31%;P = .86). Nonoperative management was associated with significantly fewer patient disability days at 1 year than did surgery (adjusted mean, 6.6 vs 10.9 days; mean difference, −4.3 days (99% CI, −6.17 to −2.43;P Conclusion and Relevance Among children with uncomplicated appendicitis, an initial nonoperative management strategy with antibiotics alone had a success rate of 67.1% and, compared with urgent surgery, was associated with statistically significantly fewer disability days at 1 year. However, there was substantial loss to follow-up, the comparison with the prespecified threshold for an acceptable success rate of nonoperative management was not statistically significant, and the hypothesized difference in disability days was not met. Trial Registration ClinicalTrials.gov Identifier:NCT02271932

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

Perianal fistulizing complications (PFCs) develop in 15% to 30% of patients with Crohn disease (CD), are difficult to treat, worsen quality of life, increase cost of care, and commonly recur. Evidence-based strategies to prevent PFCs are lacking.To investigate the effectiveness of medical therapy for reducing risk of PFCs among young people with CD and to test the hypothesis that steroid-sparing therapy (SST) use would be associated with reduced risk of PFC development.In this comparative effectiveness analysis of commercial administrative claims from January 1, 2001, through June 30, 2016, patients who did or did not initiate SST were matched via propensity score to adjust for all available confounders. Using Optum's Clinformatics Data Mart, a deidentified database of US commercial administrative claims, all patients aged 5 to 24 years with CD (January 1, 2001, through June 30, 2016) were identified. The index date was the CD diagnosis date. Patients with PFCs or SST use at or before CD diagnosis were excluded. The dates of analysis were October 2017 to February 2020.The primary exposure of interest was SST initiation, including immunomodulators and/or anti-tumor necrosis factor α (anti-TNFα) medications, initiated before either PFC development or the end of the study period.The primary outcome was PFC development. Propensity score matching was used to balance baseline characteristics. Cox proportional hazards multivariable regression analyses were used to estimate hazard ratios (HRs) with 95% CIs for PFC development.Among 2214 young people with CD without PFCs identified, the mean (SD) age at CD diagnosis was 17.0 (4.5) years, and 1151 (52.0%) were male. Among the cohort, 1242 patients (56.1%) initiated SST before PFC development or the end of 24-month follow-up. After propensity score matching, 972 patients remained in each treatment group. Overall, 384 of 1944 (19.8%) developed PFCs within 2 years of the index date. The use of SST was associated with a 59% decreased risk of PFC development (hazard ratio [HR], 0.41; 95% CI, 0.33-0.52; P .001) in 2 years compared with no SST use. Among those who developed PFCs, 55% fewer SST users underwent ostomy than SST nonusers. The use of immunomodulators alone, anti-TNFα alone, and combination therapy was associated with 52% (HR, 0.48; 95% CI, 0.37-0.62; P .001), 47% (HR, 0.53; 95% CI, 0.36-0.78; P = .001), and 83% (HR, 0.17; 95% CI, 0.09-0.30; P .001) reductions in the risk of 2-year PFC development, respectively, compared with no SST use.In this study, PFC development was common among young patients with CD. The use of SST was lower than expected. Compared with no SST, patients who initiated SST were 59% less likely to develop PFCs and fewer underwent ostomy. These results indicate that PFCs may be preventable and emphasize the importance of considering SST for all patients with CD.

Key Points Question What factors are associated with a potentially missed diagnosis of appendicitis in the emergency department among adults and children? Findings In this cohort study of 123 711 patients diagnosed with appendicitis, insurance claims data indicated that appendicitis was potentially missed in 6.0% of adults and 4.4% of children during the initial emergency department visit. Factors associated with potentially missed appendicitis included female sex, the coexistence of abdominal pain and constipation, and the presence of comorbidities. Meaning Population-based estimates of the rates of potentially missed appendicitis reveal opportunities for improvement and identify factors that may alert clinicians and mitigate the risk of missed diagnosis., Importance Appendicitis may be missed during initial emergency department (ED) presentation. Objective To compare patients with a potentially missed diagnosis of appendicitis (ie, patients with symptoms associated with appendicitis, including abdominal pain, constipation, nausea and/or vomiting, fever, and diarrhea diagnosed within 1-30 days after initial ED presentation) with patients diagnosed with appendicitis on the same day of ED presentation to identify factors associated with potentially missed appendicitis. Design, Setting, and Participants In this cohort study, a retrospective analysis of commercially insured claims data was conducted from January 1 to December 15, 2019. Patients who presented to the ED with undifferentiated symptoms associated with appendicitis between January 1, 2010, and December 31, 2017, were identified using the Clinformatics Data Mart administrative database (Optum Insights). The study sample comprised eligible adults (aged ≥18 years) and children (aged, This cohort study uses insurance claims data to compare patients with a potentially missed diagnosis of appendicitis with those diagnosed with appendicitis during the initial emergency department visit to identify factors associated with potentially missed appendicitis among adults and children.

Background Severe congenital chylothorax (SCC) may result in respiratory failure, malnutrition, immunodeficiency, and sepsis. Although typically managed with bowel rest, parenteral nutrition, and octreotide, persistent chylothoraces require surgical management. At our institution, a pleurectomy, unilateral or bilateral, in combination with mechanical pleurodesis and thoracic duct ligation is performed for SCC, and we describe our approach and outcomes. Materials and Methods We reviewed over 15-year period neonatal patients with SCC managed surgically with pleurectomy after medical therapy was unsuccessful. Patients were divided into two groups: those who underwent pleurectomy within 28 d of diagnosis (early group) and those who underwent pleurectomy after 28 d (late group). Resolution of chylothorax was defined by the absence of clinical symptoms as well as absent or minimal pleural effusion on chest X-ray. Results Of 40 patients diagnosed with SCC over the study period, 15 underwent pleurectomy, eight early [mean time to operation = 20 (IQR 17, 23) d] and 7 late [59 (42, 75) d, P = 0.001]. Overall survival was 67% (10 of 15). Seven of 8 (88%) neonates who underwent early pleurectomy survived versus 3 of 7 (43%) who underwent late pleurectomy (P = 0.07). Length of stay was lower in the early group than the late group [73 (57, 79) versus 102 (109, 213) d, P = 0.05]. All patients who survived to discharge had resolution of their chylothorax. Conclusions Pleurectomy with mechanical pleurodesis and thoracic duct ligation is effective in the management of severe congenital chylothorax. When performed earlier, pleurectomy for severe congenital chylothorax may be associated with improved survival and shorter hospital length of stay.

Introduction Autosomal recessive polycystic kidney disease (ARPKD) is a rare cause of renal failure with a highly variable clinical course. Patients who are symptomatic early in life frequently require early nephrectomy and peritoneal dialysis. In these patients there are little data to guide clinicians on whether to select unilateral nephrectomy or bilateral nephrectomy at the initial operative intervention. We review our experience with this disease process. Methods A retrospective review was performed of 11 patients at our institution with ARPKD symptomatic within the first month of life. Charts were reviewed for relevant clinical data, and patients were divided into groups based on undergoing either unilateral or bilateral nephrectomy at their initial intervention. The decision for unilateral versus bilateral nephrectomy was decided by the clinical team without any available guidelines. Results Of the 11 patients reviewed, two patients died within the first two weeks from other complications. The remaining 9 all required nephrectomy, with 5 undergoing synchronous bilateral nephrectomy, and 4 undergoing initial unilateral nephrectomy. All four patients required removal of their contralateral kidney, a median of 25.5 days later. There was no difference in mortality, ventilator free days, or time to full feeds between the two groups, although the group undergoing initial unilateral nephrectomy had more TPN days than their counterparts (28 vs 17 days, p = 0.014). Conclusions In our cohort, there were few significant differences between the groups based on choice of initial unilateral or bilateral nephrectomy, and all children ultimately required removal of both kidneys. These data suggest that anesthetic exposures and other clinical outcomes might be optimized by initial bilateral nephrectomy. Level of evidence III.

Background Congenital chylothorax (CC) can have devastating consequences for neonates. We sought to determine the outcomes of cases treated at our institution and evaluate the role of fetal intervention. Materials and methods With Institutional Review Board approval, patients treated at our institution 09/2006-04/2016 with CC were reviewed. History and outcomes were compared between patients undergoing fetal intervention (fetal group) and patients who did not (control group). Results Twenty-three patients were identified. Mean gestational age at birth was 35 wk. Overall mortality was 30% (7 patients). Nineteen patients (83%) were prenatally diagnosed, and 10 patients (43%) underwent fetal intervention. Birth weight was significantly lower in the fetal group compared to the control group (median interquartile range [IQR]; 2.5 [2.3-3.0] versus 3.3 [2.6-3.7] kg, P = 0.02). Apgar scores were significantly higher in the fetal group than the control group at 1 and 5 min (median [IQR]; 6 [4-8] versus 1 [1-2], P = 0.005 and 8 [7-9] versus 2 [2-6], P = 0.008, respectively). For those patients with prenatal diagnosis of CC and hydrops fetalis, thrombosis and lymphopenia were both improved in the fetal group (thrombosis 0% versus 40%, P = 0.03; lymphocyte nadir [median {IQR}] 1.5 [0.6-2.9] versus 0.1 [0.05-0.2], P = 0.02). Duration of support with mechanical ventilation was significantly shorter in the fetal group (median [IQR]; 1 [0-40] versus 41 [29-75] d, P = 0.04). Conclusions Fetal intervention for CC is associated with improved Apgar scores and decreased ventilator days and complications in patients with hydrops fetalis. Fetuses with chylothorax, especially those with hydrops, should be referred to a fetal center for evaluation.

Review current practices and expert opinions on contraindications to extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH) and contraindications to repair of CDH following initiation of ECMO. Modified Delphi method was employed to achieve consensus among members of the American Pediatric Surgical Association Critical Care Committee (APSA-CCC). Overall response rate was 81% including current and former members of the APSA-CCC. An average of 5–15 CDH repairs were reported annually per institution; 26–50% of patients required ECMO. 100% of respondents would not offer ECMO to a patient with a complex or unrepairable cardiac defects or lethal chromosomal abnormality; 94.1% would not in the setting of severe intracranial hemorrhage (ICH). 76.5% and 72.2% of respondents would not offer CDH repair to patients on ECMO with grade III–IV ICH or new diagnosis of lethal genetic or metabolic abnormalities, respectively. There was significant variability in whether or not to repair CDH if unable to wean from ECMO at 4–5 weeks. Significant variability in practice pattern and opinions exist regarding contraindications to ECMO and when to offer repair of CDH for patients on ECMO. Ongoing work to evaluate outcomes is needed to standardize management and minimize potentially futile interventions. V (expert opinion).