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Controversy persists regarding the ideal surgical approach for repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). We examined complications and outcomes of infants undergoing thoracoscopy and thoracotomy for repair of Type C EA/TEF using propensity score-based overlap weights to minimize the effects of selection bias.Secondary analysis of two databases from multicenter retrospective and prospective studies examining outcomes of infants with proximal EA and distal TEF who underwent repair at 11 institutions was performed based on surgical approach. Regression analysis using propensity score-based overlap weights was utilized to evaluate outcomes of patients undergoing thoracotomy or thoracoscopy for Type C EA/TEF repair.Of 504 patients included, 448 (89%) underwent thoracotomy and 56 (11%) thoracoscopy. Patients undergoing thoracoscopy were more likely to be full term (37.9 vs. 36.3 weeks estimated gestational age, p 0.001), have a higher weight at operative repair (2.9 vs. 2.6 kg, p 0.001), and less likely to have congenital heart disease (16% vs. 39%, p 0.001). Postoperative stricture rate did not differ by approach, 29 (52%) thoracoscopy and 198 (44%) thoracotomy (p = 0.42). Similarly, there was no significant difference in time from surgery to stricture formation (p 0.26). Regression analysis using propensity score-based overlap weighting found no significant difference in the odds of vocal cord paresis or paralysis (OR 1.087 p = 0.885), odds of anastomotic leak (OR 1.683 p = 0.123), the hazard of time to anastomotic stricture (HR 1.204 p = 0.378), or the number of dilations (IRR 1.182 p = 0.519) between thoracoscopy and thoracotomy.Infants undergoing thoracoscopic repair of Type C EA/TEF are more commonly full term, with higher weight at repair, and without congenital heart disease as compared to infants repaired via thoracotomy. Utilizing propensity score-based overlap weighting to minimize the effects of selection bias, we found no significant difference in complications based on surgical approach. However, our study may be underpowered to detect such outcome differences owing to the small number of infants undergoing thoracoscopic repair.Level III.

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.

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.

Background Overuse and misuse of opioids is a continuing crisis. The most common reason for children to receive opioids is postoperative pain, and they are often prescribed more than needed. The amount of opioids prescribed varies widely, even for minor ambulatory procedures. This study uses a large national sample to describe filled opioid prescriptions to preteen patients after all ambulatory surgical procedures and common standard procedures. Methods We analyzed Truven Health MarketScan data for July 2012 through December 2016 to perform descriptive analyses of opioid fills by age and geographic area, change over time, second opioid fills in opioid-naive patients, and variation in the types and amount of medication prescribed for 18 common and standard procedures in otolaryngology, urology, general surgery, ophthalmology, and orthopedics. Results Over 10% of preteen children filled perioperative opioid prescriptions for ambulatory surgery in the period 2012 to 2016. The amount prescribed varied widely (median 5 days’ supply, IQR 3–8, range 1–90), even for the most minor procedures, for example, frenotomy (median 4 days’ supply, IQR 2–5, range 1–60). Codeine fills were common despite safety concerns. Second opioid prescriptions were filled by opioid-naive patients after almost all procedures studied. The rate of prescribing declined significantly over time and varied substantially by age and across census regions. Conclusions We identified opioid prescribing outside of the norms of standard practice in all of the specialties studied. Standardizing perioperative opioid prescribing and developing guidelines on appropriate prescribing for children may reduce the opioids available for misuse and diversion.

Objective The purpose of this study was to evaluate the safety of a water-soluble contrast challenge as part of a nonoperative management algorithm in children with an adhesive small bowel obstruction (ASBO). Background Predicting which children will successfully resolve their ASBO with non-operative management at the time of admission remains difficult. Additionally, the safety of a water-soluble contrast challenge for children with ASBO has not been established in the literature. Methods A retrospective review was performed of patients who underwent non-operative management for an ASBO and received a contrast challenge across 5 children's hospitals between 2012 and 2020. Safety was assessed by comparing the complication rate associated with a contrast challenge against a pre-specified maximum acceptable level of 5%. Sensitivity, specificity, negative (NPV) and positive (PPV) predictive values of a contrast challenge to identify successful nonoperative management were calculated. Results Of 82 children who received a contrast challenge, 65% were successfully managed nonoperatively. The most common surgical indications were failure of the contrast challenge or failure to progress after initially passing the contrast challenge. There were no complications related to contrast administration (0%; 95% confidence interval: 0-3.6%, P = 0.03). The contrast challenge was highly reliable in determining which patients would require surgery and which could be successfully managed non-operatively (sensitivity 100%, specificity 86%, NPV 100%, PPV 93%). Conclusion A contrast challenge is safe in children with ASBO and has a high predictive value to assist in clinical decision-making.

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.

Background Codeine and tramadol are commonly used analgesics in surgery. In 2013, the Food and Drug Administration (FDA) issued a contraindication to the use of codeine in tonsillectomy and adenoidectomy patients aged below 18 y. This warning was expanded in April 2017 to include tramadol and all children aged below 12 y. We sought to describe the prescribing of codeine and tramadol to contraindicated populations in Wisconsin before and after the release of the expanded FDA warning. Materials and methods Using a statewide Wisconsin claims database, we identified common pediatric ambulatory surgical procedures across the specialties of otolaryngology, urology, general surgery, orthopedics, and ophthalmology. For these procedures, we examined the rates of perioperative codeine and tramadol prescription fills and change in prescribing after the FDA contraindication. Results Surgeons in all of the specialties studied continued to prescribe codeine to pediatric patients after the contraindication, but tramadol was rarely prescribed. Procedures with relatively high rates of codeine fills were strabismus repair (65% of opioid fills), circumcision >1 yo (22%), and laparoscopic appendectomy (15%). Codeine fills significantly declined after the contraindication to 6% for circumcision >1 yo and 5% for orchiopexy and inguinal hernia repair. Otolaryngology, which was subject to the 2013 codeine contraindication, has low rates of codeine fills (under 2.5%) for the whole period studied. Codeine prescribing for strabismus repair showed no significant decline. Conclusions Codeine, and to a lesser extent tramadol, continue to be prescribed to contraindicated populations of children. This represents a target for future de-implementation interventions.

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.

Nearly 75% of clinical trials fail to enroll enough participants, and cohorts often fail to reflect the clinical and demographic diversity of at-risk populations. Effective recruitment strategies are critically important for successful clinical trials. Framing treatment risks are known to affect medical decision-making for both physicians and patients but has not been rigorously studied in surgical trials. We sought to examine the impact of a high-quality video-based consent tool and the effect of risk-benefit framing on patient willingness to participate in a surgical clinical trial.A standardized video consent was shown to all potential participants in the Comparison of Outcomes of antibiotic Drugs and Appendectomy (CODA) trial, a randomized controlled trial comparing antibiotics and surgery for acute appendicitis. We report (1) differences in recruitment between two versions of a video-based tool that differed in production quality and (2) the impact of risk-benefit framing on participant randomization rates. The reasons for declining randomization were also assessed.Of 4697 eligible patients approached to participate in the CODA trial, 1535 (33% [95% confidence interval (CI): 31%-34%]) agreed to randomization; this did not change from video version 1 to version 2. There was no difference in participation between positively framed videos (32% [95% CI: 30%-34%]) versus negatively framed videos (33.0% [95% CI: 30.8-35.2]). The most common reason for declining participation was treatment preference (72% for surgery and 18% for antibiotics).Neither the change from video 1 to video 2 nor the positive versus negative framing affected participant willingness to randomize. The stakeholder-informed video-based consenting tool used in CODA was an effective strategy for the recruitment of a heterogeneous patient population within the proposed study period.

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.

New communication technologies and generational differences in communication techniques create ethical challenges for pediatric surgeons. Using two hypothetical cases we explore the ethics of modern communication in pediatric surgery. The first case explores the ethics of text messaging with patients and families and of social media posts, both of which have useful ethical analogues in older communication technologies. The second case explores ways that generational experiential differences in learning can foster misunderstandings between team members at different levels of training and potentially impact important medical care decisions. The ethical rules that govern the delivery of patient care also apply to what we say and how we say it. Effective, ethical and compassionate communication will often be the aspect of therapy most appreciated by the patient and family.

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.

Pediatric umbilical hernia repair is a common procedure that requires minimal tissue disruption. We examined variation in opioid prescription fills after repair of uncomplicated umbilical hernias to characterize the types and doses of medication used and persistent postsurgical use.Using the Truven Health Analytics MarketScan© Research Database for June 2012-September 2015, we identified pediatric patients undergoing umbilical hernia repair. We excluded patients with obstruction, gangrene, an earlier repair or a concurrent surgical procedure, and those without available pharmacy claim data. Analyses describe filled outpatient prescriptions by age, geographic region, drug type, quantity, and second prescriptions/refills.Of 4,407 procedures performed, 2,292 patients (52%) filled a prescription for postoperative opioids (age 0-1 years: 21.6%, age 2-3 years: 51.5%, age 4-5 years: 54.3%, 6 years or older: 57.9% [P.0001]). In the northeast United States, 42% of patients filled narcotic prescriptions, compared with 59% of patients in the south (P.0001). Hydrocodone/acetaminophen was most commonly prescribed (51%), followed by codeine/acetaminophen (30%). Durations were ≤3 days (50%), 4-10 days (46%), and10 days (4%). A total of 6% of patients filled a second opioid prescription within 30 days.Although many patients do not require opioids for umbilical hernia repair, most pediatric patients fill opioid prescriptions, including for prolonged courses and refills. Guidelines for appropriate prescribing of opioids after common, simple procedures, such as umbilical hernia repair, could improve the quality of care for children and impact the US epidemic of opioid abuse.

Background/purpose We examined outcomes before and after implementing an enteral water-soluble contrast protocol for management of pediatric adhesive small bowel obstruction (ASBO). Methods Medical records were reviewed retrospectively for all children admitted with ASBO between November 2010 and June 2017. Those admitted between November 2010 and October 2013 received nasogastric decompression with decision for surgery determined by surgeon judgment (preprotocol). Patients admitted after October 2013 (postprotocol) received water-soluble contrast early after admission, were monitored with serial examinations and radiographs, and underwent surgery if contrast was not visualized in the cecum by 24 h. Group outcomes were compared. Results Twenty-six patients experienced 29 admissions preprotocol, and 11 patients experienced 12 admissions postprotocol. Thirteen (45%) patients admitted preprotocol underwent surgery, versus 2 (17%) postprotocol patients (p = 0.04). Contrast study diagnostic sensitivity as a predictor for ASBO resolution was 100%, with 90% specificity. Median overall hospital LOS trended shorter in the postprotocol group, though was not statistically significant (6.2 days (preprotocol) vs 3.6 days (postprotocol) p = 0.12). Pre- vs. postprotocol net operating cost per admission yielded a savings of $8885.42. Conclusions Administration of water-soluble contrast after hospitalization for pediatric ASBO may play a dual diagnostic and therapeutic role in management with decreases in surgical intervention, LOS, and cost. Type of study Retrospective comparative study. Level of evidence Level III.

Umbilical hernias are common in young children. Many resolve spontaneously by age four with very low risk of symptoms or incarceration. Complications associated with surgical repair of asymptomatic umbilical hernias have not been well elucidated. We analyzed data from one hospital to test the hypothesis that repair at younger ages is associated with increased complication rates.A retrospective chart review of all umbilical hernia repairs performed during 2007-2015 was conducted at a tertiary care children's hospital. Patients undergoing repairs as a single procedure for asymptomatic hernia were evaluated for post-operative complications by age, demographics, and co-morbidities.Of 308 umbilical hernia repairs performed, 204 were isolated and asymptomatic. Postoperative complications were more frequent in children 4 years (12.3%) compared to 4 years (3.1%, p = 0.034). All respiratory complications (N = 4) and readmissions (N = 1) were in children 4 years.Age of umbilical hernia repair in children varied widely even within a single institution, demonstrating that timing of repair may be a surgeon-dependent decision. Patients 4 years were more likely to experience post-operative complications. Umbilical hernias often resolve over time and can safely be monitored with watchful waiting. Formal guidelines are needed to support delayed repair and prevent unnecessary, potentially harmful operations.

Prior data suggest that infants with gastroschisis are at high risk for hypothermia and infectious complications (ICs). This study evaluated the associations between perioperative hypothermia (PH) and ICs in gastroschisis using a multi-institutional cohort.Retrospective review of infants with gastroschisis who underwent abdominal closure from 2013-2017 was performed at 7 children's hospitals. Any-IC and surgical site infection (SSI) were stratified against the presence or absence of PH, and perioperative characteristics associated with PH and SSI were determined using multivariable logistic regression.Of 256 gastroschisis neonates, 42% developed PH, with 18% classified as mild hypothermia (35.5-35.9 °C), 10.5% as moderate (35.0-35.4 °C), and 13% severe (35 °C). There were 82 (32%) ICs with 50 (19.5%) being SSIs. No associations between PH and any-IC (p = 0.7) or SSI (p = 0.98) were found. Pulmonary comorbidities (odds ratio (OR)=3.76, 95%CI:1.42-10, p = 0.008) and primary closure (OR=0.21, 95%CI:0.12-0.39, p0.001) were associated with PH, while silo placement (OR=2.62, 95%CI:1.1-6.3, p = 0.03) and prosthetic patch (OR=3.42, 95%CI:1.4-8.3, p = 0.007) were associated with SSI on multivariable logistic regression.Primary abdominal closure and pulmonary comorbidities are associated with PH in gastroschisis, however PH was not associated with increased risk of ICs. Independent risk factors for SSI include silo placement and prosthetic patch closure.

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.

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.

This study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM).We performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 children's hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed.Among 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥1 abnormal finding on any screening test varied by type of ARM (p0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%).Screening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients.Multi-institutional retrospective cohort study.III.

Patient: Male, 6 Final Diagnosis: Colonic perforation secondary to vascular Ehlers Danlos Syndrome Symptoms: Abdominal pain • constipation Medication: — Clinical Procedure: Loop colostomy followed by total colectomy and ileostomy Specialty: Surgery Objective: Unusual clinical course Background: Ehlers-Danlos Syndrome (EDS) is a group of connective tissue disorders with heterogeneous clinical features associated with varying genetic mutations. EDS type IV, also known as vascular EDS (vEDS), is the rarest type but has fatal complications, including rupture of major vasculature and intestinal and uterine perforation. Intestinal perforation can be spontaneous or a consequence of long-standing constipation, a common symptom among patients with EDS. Case Report: We present a case of a 6-year-old boy with the previous diagnosis of vEDS who presented with colonic perforation from a stercoral ulcer. He underwent diagnostic laparoscopy and loop colostomy, with an uneventful postoperative course. Unfortunately, he developed a second colonic perforation 14 months after the initial episode and underwent total abdominal colectomy with end ileostomy. Conclusions: Intestinal perforation is a well-documented and devastating complication of vEDS. However, spontaneous intestinal perforation is extremely rare in a young child. Therefore, the diagnosis of vEDS should be included in the differential diagnosis if a child presents with intestinal perforation. There is no clear guideline available for surgical management of colonic perforation in patients with vEDS, but total abdominal colectomy appears to provide the best chance of preventing recurrent perforation.

To evaluate trends in procedures used to treat children hospitalized in the US with empyema during a period that included the release of guidelines endorsing chest tube placement as an acceptable first-line alternative to video-assisted thoracoscopic surgery.We used National Inpatient Samples to describe empyema-related discharges of children ages 0-17 years during 2008-2014. We evaluated trends using inverse variance weighted linear regression and characterized treatment failure using multivariable logistic regression to identify factors associated with having more than 1 procedure.Empyema-related discharges declined from 3 in 100 000 children to 2 in 100 000 during 2008-2014 (P = .04, linear trend). There was no significant change in the proportion of discharges having 1 procedure (66.1% to 64.1%) or in the proportion having 2 or more procedures (22.1% to 21.6%). The proportion coded for video-assisted thoracoscopic surgery as the only procedure declined (41.4% to 36.2%; P = .03), and the proportions coded for 1 chest tube (14.6% to 20.9%; P = .04) and 2 chest tube procedures (0.9% to 3.5%; P .01) both increased. The median length of stay for empyema-related discharges remained unchanged (9.3 days to 9.8 days; P = .053). Having more than 1 procedure was associated with continuous mechanical ventilation (adjusted OR, 2.7; 95% CI, 1.8-4.1) but not with age, sex, payer, chronic conditions, transfer admission, hospital size, or census region.The use of video-assisted thoracoscopic surgery to treat children in the US hospitalized with empyema seems to be decreasing without associated increases in length of stay or need for additional drainage procedures.

Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF.A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up.In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54-2.65) vs. 2.57 kg (2.00-3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32-37) vs. 37 weeks (35-39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p 0.29).RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach.Level III.

Background The ability of ultrasound to identify specific features relevant to nonoperative management of pediatric appendicitis, such as the presence of complicated appendicitis (CA) or an appendicolith, is unknown. Our objective was to determine the reliability of ultrasound in identifying these features. Methods We performed a retrospective study of children who underwent appendectomy after an ultrasound at four children's hospitals. Imaging, operative, and pathology reports were reviewed. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of ultrasound for identifying CA based on pathology and intraoperative findings and an appendicolith based on pathology were calculated. CA was defined as a perforation of the appendix. Equivocal ultrasounds were considered as not indicating CA. Results Of 1027 patients, 77.5% had simple appendicitis, 16.2% had CA, 5.4% had no evidence of appendicitis, and 15.6% had an appendicolith. Sensitivity and specificity of ultrasound for detecting CA based on pathology were 42.2% and 90.4%; the PPV and NPV were 45.8% and 89.0%, respectively. Sensitivity and specificity of ultrasound for detecting CA based on intraoperative findings were 37.3% and 92.7%; the PPV and NPV were 63.4% and 81.4%, respectively. Sensitivity and specificity of ultrasound for detecting an appendicolith based on pathology were 58.1% and 78.3%; the PPV and NPV were 33.1% and 91.0%, respectively. Results were similar when equivocal ultrasound and negative appendectomies were excluded. Conclusions The high specificity and NPV suggest that ultrasound is a reliable test to exclude CA and an appendicolith in patients being considered for nonoperative management of simple appendicitis.

Introduction Uncomplicated pediatric umbilical hernias are common and most close spontaneously. No formal practice guidelines exist regarding the optimal timing and indications for repair. The objective of this review is to examine the existing literature on the natural history of pediatric umbilical hernias, known complications of repair and non-operative approaches, and management recommendations. Study design A systematic literature search was performed to identify publications relating to pediatric umbilical hernias. Inclusion criteria comprised studies addressing recommendations for optimal timing of repair, evidence examining complications from hernias not operatively repaired, and research exploring the likelihood of pediatric umbilical hernias to close spontaneously. In addition, the websites of all pediatric hospitals in the United States were examined for recommendations on operative timing. Results A total of 787 manuscripts were reviewed, and 28 met criteria for inclusion in the analysis. Studies examined the likelihood of spontaneous closure based on child's age and size of hernia defect, complications of unrepaired umbilical hernias including incarceration, strangulation and evisceration based on child's age and size of defect, incidence of postoperative complications and current recommendations for timing of repair. In addition, 63 (27.5%) of the United States pediatric hospital websites published a wide range of management recommendations. Conclusion Despite the high prevalence of pediatric umbilical hernias, there is a paucity of high quality data to guide management. The literature does suggest that expectant management of asymptomatic hernias until age 4–5years, regardless of size of hernia defect, is both safe and the standard practice of many pediatric hospitals. Type of study Review Article. Level of evidence Level IV.

Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF.The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers.292 patients with proximal EA and distal TEF who underwent primary repair were reviewed. The overall mortality was 6% and was significantly associated with the presence of congenital heart disease (OR 4.82, p=0.005). Postoperative complications occurred in 181 (62%) infants, including: anastomotic stricture requiring intervention (n=127; 43%); anastomotic leak (n=54; 18%); recurrent fistula (n=15; 5%); vocal cord paralysis/paresis (n=14; 5%); and esophageal dehiscence (n=5; 2%). Placement of a transanastomotic tube was associated with an increase in esophageal stricture formation (OR 2.2, p=0.01). Acid suppression was not associated with altered rates of stricture, leak or pneumonia (all p0.1). Placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased leak rate (OR 4.7, p0.001), but no difference in the incidence of recurrent fistula (p=0.3). Empiric postoperative antibiotics for24h were used in 193 patients (66%) with no difference in rates of infection, shock or death when compared to antibiotic use ≤24h (all p0.3). Hospital volume was not associated with postoperative complication rates (p0.08). Routine postoperative esophagram obtained on day 5 resulted in no delayed/missed anastomotic leaks or a difference in anastomotic leak rate as compared to esophagrams obtained on day 7.Morbidity after primary repair of proximal EA and distal TEF patients is substantial, and many common practices do not appear to reduce complications. Specifically, this large retrospective series does not support the use of prophylactic antibiotics beyond 24h and empiric acid suppression may not prevent complications. Use of a transanastomotic tube was associated with higher rates of stricture, and interposition of prosthetic material was associated with higher leak rates. Routine postoperative esophagram can be safely obtained on day 5 resulting in earlier initiation of oral feeds.Treatment study.III.

Injury to the solid abdominal organs—liver, spleen, kidney, and pancreas—is one of the most common injury patterns in pediatric blunt trauma. Pediatric trauma centers are becoming increasingly successful in managing these injuries without operative intervention. Well-validated guidelines have been established for liver and spleen injury management, and operative intervention is reserved for patients who show evidence of active bleeding after resuscitation. No such guidelines yet exist for the management of traumatic injury of the kidney or pancreas. Exploratory laparotomy remains the treatment of choice in patients suffering hemodynamic collapse, but interventional radiologic or endoscopic procedures are increasingly used to manage all but the most devastating solid organ injuries. [ Pediatr Ann . 2016;45(7):e241–e246.]

Operating in small spaces presents physical constraints that can be even more challenging in minimally invasive operations. Recently, a 5-mm stapler was approved for use in general surgery and pediatric surgery. Here, we present our initial experience using the 5-mm stapler in pediatric general surgery.A retrospective chart review was conducted to identify cases using the 5-mm stapler at our institution. Demographic data included age (in months) and weight (in kilograms). Operative data included indication for use, number of loads used, complications related to 5-mm stapler use, and interventions to address complications. A second review focused on patients undergoing the same operations, but using a 10-mm stapler.A total of 60 staple loads were deployed in 32 procedures. There were four adverse outcomes, all recognized intraoperatively. One bleed resulted from application on irradiated tissue and another bleed from application to a small noninflamed mesoappendix. A bronchial staple line leak resulted from improper stapler loading, and a bowel anastomosis leak was oversewn with a single stitch. When compared with 32 matched cases using a 10-mm stapler, there was no difference in age (5-mm = 39.11 months, 10-mm = 50.21 months, P = .49) or weight (5-mm = 16.34 kg, 10-mm = 19.93 kg, P = .51). A total of 60 staple applications were used, with one bleed noted. There was no significant difference in overall complication rate (5-mm rate = 4/60, 10-mm rate = 1/60; P = .36).Our initial experience suggests that although there were more complications with the 5-mm stapler, there is no statistically significant difference in complication rates when compared with the 10-mm stapler. Furthermore, the 5-mm stapler complications can be corrected with device training and proper patient selection. In appropriately selected pediatric surgery cases with size limitations, the 5-mm stapler can be used to minimize the invasiveness of the operation.

Necrotizing fasciitis caused by Klebsiella pneumoniae is rare, with 11 documented cases in the literature, all occurring in Asia and the Middle East. These reports have become more frequent in recent years in association with the emergence of the highly virulent K1 capsular serotype of Klebsiella. We report a case of Klebsiella necrotizing fasciitis and con-current septic arthritis in the Western hemisphere.Case report and review of the literature.Our patient, a 50-year-old Cambodian man with advanced hepatitis B-associated cirrhosis, who had last visited an endemic area six months prior to presentation, was hospitalized after several days of left knee and thigh pain. He was hypotensive, tachycardic, and bacteremic with K. pneumoniae. A computed tomography scan showed gas in the tissues of the thigh and left knee joint. Despite aggressive surgical debridement and antibiotic therapy, the patient died on day three. Serotyping of blood, wound, and synovial cultures revealed K1-type K. pneumoniae.Although K. pneumoniae is common, monomicrobial necrotizing fasciitis and septic arthritis caused by this pathogen has not been reported previously outside Asia, where it is rare. The appearance of this infection in the Western hemisphere may reflect geographic spread of the aggressive K1 phenotype. Physicians treating patients with symptoms and signs of necrotizing fasciitis or septic arthritis, particularly in the setting of underlying chronic illness or recent travel to Asia, should consider K. pneumoniae as a potential cause and treat accordingly.

Trichobezoar is a mass of hair retained in the stomach, usually associated with trichotillomania. Large trichobezoars may extend into the small bowel and cause small bowel obstruction or pancreatic dysfunction, a condition known as Rapunzel syndrome. Here we report the first North American case of Rapunzel syndrome in a male patient.

Background Caspase-3, a pro-apoptotic enzyme, represents a class of proteins in which the active site contains reduced thiol (S-H) groups and is modulated by heavy metal cations, such as Zn 2+ . We explored the effects of the thiol oxidant monochloramine (NH 2 Cl) on caspase-3 activity within cells of isolated rabbit gastric glands. In addition, we tested the hypothesis that NH 2 Cl-induced alterations of caspase-3 activity are modulated by oxidant-induced accumulation of Zn 2+ within the cytoplasm. Materials and methods Isolated gastric glands were prepared from rabbit mucosa by collagenase digestion. Caspase-3 activity was measured colorimetrically in suspensions of healthy rabbit gastric glands, following exposure to various concentrations of NH 2 Cl with or without the zinc chelator TPEN [tetrakis-(2-pyridylmethyl)ethylene diamine] for 1 h, and re-equilibration in Ringer's solution for 5 h. Conversion of procaspase-3 to active caspase-3 was monitored by Western blot. Results Monochloramine inhibited caspase-3 activity in a dose-dependent fashion. At concentrations of NH 2 Cl up to 100 μ m , these effects were prevented if TPEN was given concurrently and were partly reversed if TPEN was given 1 h later. Caspase-3 activity was preserved by concurrent treatment with a thiol-reducing agent, dithiothreitol. Conclusions At pathologically relevant concentrations, NH 2 Cl impairs caspase-3 activity through oxidation of its thiol groups. Independently from its thiol oxidant effects on the enzyme, NH 2 Cl-induced accumulation of Zn 2+ in the cytoplasm is sufficient to restrain endogenous caspase-3 activity. Our studies suggest that some bacterially generated oxidants, such as NH 2 Cl, impair host pathways of apoptosis through release of Zn 2+ from endogenous pools.

Introduction. Although there is a significant body of evidence that bacteria activate a variety of pro-inflammatory signals following their interaction with human epithelial cells, there is virtually no information on signaling in the opposite direction. In this study we sought to determine whether host cell factors released by intestinal epithelial cells subjected to stress could signal the opportunistic pathogen, P. aeruginosa, to express a key virulence related protein, the PA-I lectin. Methods. Human intestinal epithelia (Caco-2bbe) were grown to confluence in HDMEM media. To expose cells to a clinically relevant stress, hyperthermic conditions were created by immersing sealed culture dishes in a 42°C water bath for 23 min, followed by 2 h of recovery at 37°C and 5% CO2. Control cells were maintained in 37°C at 5% CO2.. Media were then filtered through a 0.22-μm filter before serial centrifugal membrane filtration using 100, 50, 30, 10, and 3 kDa filters and resuspended in HDMEM. GFP reporter strains of P. aeruginosa carrying a fusion construct for the PA-I lectin were then exposed to the epithelial cell derived filtrates from heat shock stressed Caco-2 cells. Dynamic fluorimetry was used to measure fluorescence (i.e. PA-I activity) over time. To determine if inducing compounds were proteins, fractions capable of activating PA-I were then heat inactivated by heating to 70°C for 30 min and tested against active fractions by the same method. Results. A 30- to 50-kDa media fraction from heat-stressed cells induced a 10-fold increase in PA-I promoter activity over controls, as measured by fluorescence (1082 ± 216%, P < 0.01), while all other fractions showed no effect. Heat inactivation completely eliminated this effect (−156% of control ± 293%, P = NS). Conclusion. Human intestinal epithelial cells release a soluble 30- to 50-kDa compound(s) that can activate a key virulence protein in P. aeruginosa. We speculate that the molecular dialogue between a pathogen and the intestinal epithelium is bidirectional and can be initiated by host stress conditions such as hyperthermia.

Low molecular weight heparin (LMWH) is frequently employed in children to prevent extension of intravascular thrombosis. However, this therapy can result in pathologic bleeding, including spontaneous intramural hematoma of the intestinal wall. In this report, we describe two cases of intestinal obstruction resulting from intramural hematomas during therapeutic LMWH therapy in children. The diagnostic studies and management of spontaneous intramural hematoma in children are discussed.