Radical Resection of Pediatric Fibrosarcoma Is Not Associated With Survival Benefit: A Nationwide Database Analysis.

INTRODUCTION: Fibrosarcoma is one of the most common nonrhabdomyosarcoma soft tissue sarcomas in the pediatric population. Surgical resection is the cornerstone of treatment; however, optimal factors regarding the extent of fibrosarcoma resection in localized versus regional disease is poorly understood. METHODS: The Surveillance, Epidemiology, and End Results database was queried for patients who are 19 y old or younger with a diagnosis of fibrosarcoma from 1975 to 2016. Kaplan-Meier analyses were performed for disease-specific survival on clinical and pathologic variables. Multivariate analysis was performed based on significant predictors of disease-specific survival. RESULTS: There were 1290 patients (median age 13 [7-17] y) identified with fibrosarcoma. The overall survival rate at 20 y was 93%. Radical resection was performed on 22%, 40%, and 52% of patients with localized, regional, and distant Surveillance, Epidemiology, and End Results disease stage, respectively. Chemotherapy (P < 0.001), radiation (P < 0.001), histology (P < 0.001), and stage (P = 0.004) were significant predictors of increased mortality using univariate Kaplan-Meier analysis. Most patients (86%) with localized or regional stage who underwent radiation had fibrosarcomas of nondermatofibrosarcoma histology (P < 0.001). Dermatofibrosarcoma subtype comprised most radical resections (41%). Excluding distant stage, nondermatofibroma histology (hazard ratio 16.94, 95% confidence interval 3.77-76.01) and regional stage (hazard ratio 8.15, 95% confidence interval 2.93-22.69) were independent prognostic factors of mortality (both P < 0.001). Radical resection was not a significant independent prognostic indicator of survival. CONCLUSIONS: Nondermatofibrosarcoma subtype is independently associated with increased mortality. Although surgery remains the mainstay of treatment, the extent of resection is not a predictor of survival for patients with the localized and regional stage of disease.

National burden of pediatric abusive injuries: patterns vary by age.

PURPOSE: Child abuse is often unrecognized by healthcare practitioners. This study sought to characterize pediatric abuse injury patterns and associated mortality rates in a nationwide cohort. METHODS: The Kids' Inpatient Database (1997-2012) was queried for patients < 18 years old with a diagnosis of child abuse (utilizing ICD-9 codes for abusive head trauma [AHT], neglect, physical, emotional, sexual, and other abuse). Demographic factors, mortality, and injury patterns were compared with demographic factors using standard statistical tests. RESULTS: > 39,000 children were hospitalized for abuse from 1997 to 2012. The majority were Caucasian (36%), male (51%) and < 4 years old (70%). Most sustained physical abuse (53%), followed by AHT (14%), sexual (9%) and emotional abuse (2%). Multiple injuries were sustained by 44% of patients. Mortality was 4% (n = 1476). Burns (2%) and intrathoracic (2%) injuries had the highest mortality compared to other injuries (21% and 19%, respectively; both P < 0.001). Emotional abuse (5%) and neglect (1%) were highest in those ≥ 13 years old (both P  = 0.001). CONCLUSION: Physical injuries are less common in adolescents (13-18 years) hospitalized for abuse. However, they are more likely to report emotional abuse and neglect, which has not been compared in previous studies. Intrathoracic injuries and AHT are associated with significant mortality.

Liquid Nitrogen Applied at Point of Sale: A New Presentation of Gastric Perforation in Children.

ABSTRACT: The August 30, 2018, US Food and Drug Administration advisory warned consumers to avoid eating, drinking, or handling food products prepared with liquid nitrogen (LN) (US FDA. Safety Alerts & Advisories: FDA Advises Consumers to Avoid Eating, Drinking, or Handling Food Products Prepared with Liquid Nitrogen at the Point of Sale. US Food and Drug Administration). We report on the case of a pediatric patient sustaining gastric perforation after ingestion of LN applied to food at the point of sale in the United States. "Dragon's Breath" is a popular snack of cereal puffs coated in LN giving the allusion of breathing smoke on ingestion. Instructions provided by vendors include avoidance of touching or drinking the liquid in the bottom of the cup. We report on a case of a 9-year-old girl presenting with peritonitis and gross pneumoperitoneum after consumption of Dragon's Breath with injury conferred secondary to LN ingestion. Intraoperative finding of a large perforation along the lesser curvature of the stomach was repaired primarily with an omental overlay. Her postoperative course was complicated by pneumonia and a surgical site infection. She was discharged 13 days after admission. A review of the literature of previous case reports of LN ingestion by intentional or accidental means is provided. This case report and review of the literature bring awareness of the dangers posed to pediatric patients exposed to LN applied at the point of sale.

Protocol-Driven Surgical Care of Necrotizing Enterocolitis and Spontaneous Intestinal Perforation.

BACKGROUND: There is no clear consensus on the optimal operative management of premature infants with surgical necrotizing enterocolitis (sNEC) or spontaneous intestinal perforation (SIP); thus, a protocol was developed to guide surgical decision making regarding initial peritoneal drainage (PD) versus initial laparotomy (LAP). We sought to evaluate outcomes after implementation of the protocol. METHODS: Pre-post study including multiple urban hospitals. Premature infants with sNEC/SIP were accrued after implementation of surgical protocol-directed care (June 2014-June 2019). Patients with a birth weight of <750 g and less than 2 wk of age without pneumatosis or portal venous gas were treated with PD on perforation. PD patients received subsequent LAP for clinical deterioration or continued meconium/bilious drainage. Postprotocol characteristics and outcomes were compared with institutional historical controls. Significance set at P < 0.05. RESULTS: Preprotocol and postprotocol cohorts comprise 35 and 73 patients, respectively. There was a statistically significant difference in age at intervention between historical control PD (14 ± 13 d) and postprotocol PD (9 ± 4 d) groups (P = 0.01), PD patient's birth weight (716 ± 212 g versus 610 ± 141 g, P = 0.02) and estimated gestational age of LAP patients (27 ± 1.7 wk versus 31 ± 4 wk, P = 0.002). PD was definitive surgery in 27% (12 of 44) of postprotocol patients compared with 13% (3 of 23) historical controls. A trend in improved survival postprotocol occurred in all PD infants (73% versus 65%), all LAP (75% versus 70%), and for initial PD and subsequent LAP (82% versus 67%). CONCLUSIONS: Utilization of a surgical protocol in sNEC/SIP is associated with improved success of PD as definitive surgery and improved survival.

Lymph Node Sampling and Survival in Child and Adolescent Extremity Soft-Tissue Sarcoma.

BACKGROUND: The significance of lymph node sampling (LNS) on disease-specific survival (DSS) of extremity soft tissue sarcomas (STS) is unknown. We investigated the effect of LNS on DSS in child and adolescent extremity STS. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results registry was queried for patients aged <20 y with extremity STS who underwent surgery. Patient demographics were collected and analyzed. RESULTS: A total of 1550 patients were included, with findings of 10-y DSS of 74% for all extremity STS and 49% for rhabdoymyosarcoma (RMS) (P < 0.005). LNS was associated with worse DSS in patients with extremity nonrhabdomyosacrcoma soft tissue sarcomas (79% versus 84%, P = 0.036). Conversely, LNS was associated with an improved DSS in patients with extremity RMS (64% versus 49%, P = 0.005). CONCLUSIONS: LNS is positively associated with an improved DSS in child and adolescent extremity RMS. Multivariate analysis found no correlation between DSS and LNS in child and adolescent extremity nonrhabdomyosarcoma soft tissue sarcomas.

Endoscopic Management of a Double Duodenal Web: A Case Report of a Rare Alimentary Anomaly.

Duodenal webs are a rare clinical entity with the presentation of a double duodenal web being exceedingly uncommon. Management of duodenal webs traditionally involves duodenal web excision with duodenoduodenostomy, which is usually performed via a laparoscopic or an open approach. We report the case of a 6-month-old child who presented with progressively worsening bilious emesis with imaging findings concerning for a duodenal web. Endoscopic evaluation was performed that identified 2 webs in the fourth portion of the duodenum. These were managed completely endoscopically with balloon dilation. Although surgery is the mainstay of treatment of duodenal webs, this patient was successfully managed by endoscopic intervention without the need for open or laparoscopic excision, which has not been previously described for double duodenal webs. This work demonstrates the safety and efficacy of endoscopic management for infants with this anomaly.

Pediatric Pedestrian Injuries: Striking Too Close to Home.

BACKGROUND: Pediatric pedestrian injuries (PPI) are a major public health concern. This study utilized geospatial analysis to characterize the risk and injury severity of PPI. METHODS: A retrospective chart review of PPI patients (age < 18) from a level 1 trauma center was performed (2013-2020). A geographic information system geocoded injury location to home and other public landmarks. Incidents were aggregated to zip codes and the Local Indicators of Spatial Association statistic tested for spatial clustering of injury rates per 10,000 children. Predictors for increased injury severity were assessed by logistic regression. RESULTS: PPI encompassed 6% (n = 188) of pediatric traumas. Most patients were black (54%), male (58%), >13 years (56%), and with Medicaid insurance (68%). Nine zip codes comprised a statistically significant cluster of PPI. Nearly half (40%) occurred within a quarter mile of home; 7% occurred at home. Most (65%) PPI occurred within 1 mile of a school, and 45% occurred within a quarter mile of a park. Nearly all (99%) PPI occurred within a quarter mile of a major intersection and/or roadway. Using admission to ICU as a marker for injury severity, farther distance from home (OR 1.060, 95% CI 1.001-1.121, p = 0.045) and age <13 years (3.662, 95% CI 1.854-7.231, p < 0.001) were independent predictors of injury severity. CONCLUSIONS: There are significant sociodemographic disparities in PPI. Most injuries occur near patients' homes and other public landmarks. Multidisciplinary injury prevention collaboration can help inform policymakers, direct local safety programs, and provide a model for PPI prevention at the national level. LEVEL OF EVIDENCE: Level IV.

The limited role of ultrasound in the surgical assessment of solid pediatric breast lesions.

INTRODUCTION: Ultrasound (US) is an adjunct to history and clinical exam (CE) in the assessment of pediatric breast lesions. We sought to investigate the reliability of US and CE to predict final pathologic diameter (P). METHODS: A single institutional retrospective analysis of patients aged ≤18 years who underwent breast mass resection was performed. Data was collected and analyzed using SPSS. RESULTS: 88 patients met inclusion criteria with an average age at surgery of 16 ± 1.5 years. No malignancies were encountered. The largest mean diameter measured by final pathology (MPØ) for all lesions was 4.1 ± 2.6 cm. Pathology encountered were fibroadenoma (83%, MPØ 3.7 ± 1.7 cm), juvenile fibroadenoma (10%, MPØ 7.0 ± 5.4 cm), and low-grade phyllodes tumor (3%, MPØ 6.2 ± 3.8 cm). 67 patients had documented CE measurement with a mean diameter of 3.4 ± 1.8 cm. 62 patients underwent US with a mean diameter of 3.3 ± 1.6 cm. US and CE were accurate in determining P by Cronbach Alpha reliability testing. CONCLUSION: US and CE are reliable measurements of P. The surgical utility of US when considering pediatric breast lesions is limited and should be individualized following pediatric surgical evaluation and CE.

AAMC Data Shows Effect of Surgery Faculty Diversity on General Surgery Resident Attrition Rate at Programs Sponsored by LCME-Accredited Medical Schools.

OBJECTIVE: General surgery resident (GSR) 5-year attrition rates of 12% to 20% are currently reported. This study explores the impact of full-time surgery faculty (FSF) diversity on GSR attrition. DESIGN: Deidentified data were obtained from the Association of American Medical Colleges (AAMC) for FSF at US Liaison Committee on Medical Education (LCME)-accredited medical schools and GSR at the affiliated general surgery residency programs (2001-2016). Data included annual GSR attrition rate and the number, gender, and race of FSF and GSR. Data were analyzed using linear and logarithmic regression. SETTING: The study was conducted at the University of Miami Leonard M. Miller School of Medicine in Miami, Florida. PARTICIPANTS: The data obtained included FSF from US LCME-accredited medical schools and GSR from those residency programs affiliated with US LCME-accredited medical schools. Data were included only if available for both FSF and GSR at a single institution. There were 107,300 annual FSF positions and 39,504 annual GSR positions from 61 U.S. LCME-accredited medical schools included in the analysis. RESULTS: Data included 107,300 FSF positions (26% non-white; 20% female) and 39,504 GSR positions (41% non-white; 33% female) summed across 1034 institution years. Increased female FSF is associated with decreased GSR attrition (R2 = 0.009, p = 0.002, Fig. 1). For every 1% increase in female FSF, GSR programs were 4% less likely to have an attrition rate in the top quartile (odds ratio 0.96, confidence interval 0.94-0.98). CONCLUSIONS: Gender diversity of FSF has an impact on GSR attrition; more female FSF correlates with lower GSR attrition rates.

A retrospective comparison of outcomes for open vs. laparoscopic surgical techniques in pediatric ulcerative colitis.

BACKGROUND: Ulcerative colitis (UC) is an aggressive disease in the pediatric population and a cause of significant, lifelong morbidity. The aim of this study is to compare surgical complications in pediatric patients undergoing laparoscopic vs. open surgical treatment for UC. METHODS: We queried the Kids' Inpatient Database (KID) for all cases of UC undergoing surgical treatment in 2009 and 2012. We identified patients who received total colectomy without proctectomy (n=413) or total proctocolectomy (n=196) and performed univariate and multivariate analyses comparing laparoscopic vs. open procedures. RESULTS: In pediatric UC patients undergoing total colectomy without proctectomy, open procedures were associated with more complications than laparoscopic, including fluid and electrolyte disorders (40% vs. 28%), surgical wound dehiscence (6% vs. 2%), septicemia (18% vs. 2%), and gastrointestinal disorders (16% vs. 7%) among others, all P<0.05. Likewise, in patients with UC undergoing total proctocolectomy, there were more complications in open vs. laparoscopic technique, including increased transfusion requirements (25% vs. 7%, P=0.001) and significantly more gastrointestinal upset, including nausea, vomiting, and diarrhea (11% vs. 1%, P=0.003). In multivariate analysis, patients who underwent total colectomy with or without proctectomy had an increased risk of experiencing any complication when their procedure was performed in an open or non-elective fashion (all odds ratio >2.4; all P<0.001). CONCLUSIONS: The laparoscopic approach was associated with significantly lower rates of surgical complications in pediatric patients undergoing total colectomy with or without proctectomy for UC. These findings demonstrate that laparoscopic technique compares favorably, and may be preferable, to the open approach in selected pediatric patients with UC.

Laparoscopic-assisted Open Pyloroduodenostomy Repair of Pyloric Atresia in an Infant: A Rare Congenital Anomaly.

PURPOSE: Pyloric atresia is an uncommon congenital anomaly that may present with emesis and dehydration early in infancy. Definitive therapy consists of pyloroplasty or gastroduodenostomy depending on the subtype of atresia involved. CASE PRESENTATION: We report the case of a 2-day-old girl infant who presented with nonbilious emesis with feeds. Abdominal ultrasound and upper gastrointestinal series were performed and demonstrated a dilated, air-filled stomach with no distal air or passage of contrast. The patient was successfully treated by laparoscopic-assisted open pyloroduodenostomy of a type 2 pyloric atresia. CONCLUSION: Type 2 pyloric atresia is a rare clinical entity that can present with symptoms of gastric outlet obstruction. The patient described was treated by laparoscopic-assisted open pyloroduodenostomy, which has not been previously described for pyloric atresia. This work demonstrates an example of the safety and efficacy of laparoscopic surgery for infants with this anomaly.

Increased lymph node ratio predicts poor survival in pediatric rhabdomyosarcoma.

BACKGROUND/PURPOSE: Lymph Node Ratio (LNR) is defined as the number of positive lymph nodes (pLN) divided by total lymph nodes (LN) examined. LNR correlates with recurrence and/or overall survival in many adult cancers but has not previously been described in pediatric oncologic disease. We hypothesized that LNR correlates with worse disease specific survival (DSS) in pediatric rhabdomyosarcoma (RMS). METHODS: Patients <20 years who underwent surgery for RMS between 1988 and 2013 in the SEER database were analyzed. RESULTS: 188 patients with a mean age at diagnosis of 8.8 ±â€¯6 years and a mean LNR of 0.13 ±â€¯0.27 were identified. Univariate analysis found that alveolar type, positive lymph node (pLN), stage, site, LNR, and age had significantly worse survival. Cox regression analysis identified LNR > 0.75 (HR 4.32, P = 0.015), alveolar histology (HR 4.797, P < 0.003), age < 1 year (HR 4.402, P = 0.004), and distant disease (HR 5.738, P < 0.001), as independent determinants of worse DSS. pLN and site were not statistically significant determinant of DSS on multivariate analysis. DSS for the entire cohort was 83% at 5 and 78% at 10 and 15 years. DSS at 5, 10, and 15 year for LNR ≤ 0.75 was 79% while patients with LNR > 0.75 had a 22% 5 year and 0% 10 year survival (P <0.001). CONCLUSIONS: LNR is superior to pLN status as an independent prognostic indicator of DSS in pediatric RMS. LEVEL OF EVIDENCE: Level III.

Predictors of in-hospital mortality in newborn conjoined twins.

BACKGROUND: Conjoined twins are rare developmental anomalies. There is a paucity of literature other than case reports and small case series. The aim of this study was to examine national outcomes and identify predictors of mortality in newborn conjoined twins. METHODS: We reviewed data on newborn conjoined twins from the Kids' Inpatient Database (1997-2012). RESULTS: A total of 240 patients were identified for a nationally weighted incidence of 1 per 100,000 live births. The majority of conjoined twins were female (n = 190 [81%]). The most commonly associated anomalies were cardiac (n = 87 [36%]), gastrointestinal (n = 41 [17%]), and abdominal wall (n = 32 [13%]) defects. Fifty-six (23%) patients underwent operative procedures, including 28 (12%) neonatal separation surgeries. The overall mortality rate was 61%; most deaths occurred within 24 hours (99 of 146 [68%]) to 48 hours (129 of 146 [88%]) after birth. Mortality was higher in female compared with male children (66% vs 38%, P = .025), premature compared with full-term children (72% vs 44%, P = .007), and in children with extremely low birth weight (95% vs 59%, P = .002). Congenital diaphragmatic hernias were seen in 15 (6%) patients and were uniformly fatal (100% vs 58%, P = .029). Mortality was highest in hospitals not designated as children's hospitals (72%) compared with children's hospitals (44%) (P = .007). CONCLUSION: Conjoined twins are rare anomalies who are susceptible to extremely high perinatal mortality, especially in female children, those who are premature, or those who have low birth weight. These data support caring for these complex patients at hospitals equipped to care for this fragile population.

Suction rectal biopsy yields adequate tissue in children.

BACKGROUND/PURPOSE: Hirschsprung disease (HD) is diagnosed by rectal biopsy, with suction rectal biopsy (SRB), the preferred technique in neonates. Reported SRB adequacy has varied overall with concern for decreased diagnostic yield in older children. The study aim was to assess SRB adequacy by age in children with the current device used at our institution. METHODS: Following IRB approval, a retrospective cohort of children (1 to 18years) evaluated by SRB for HD was identified through billing records. Data regarding demographics, procedure, results, and complications were collected and analyzed using SPSS. RESULTS: 56 children (median age 3.9years) underwent SRB with an 80.4% overall success rate. Patients older than 5years had 90.5% adequacy rate compared to 74.3% in those younger. Univariate analysis revealed weak association of inadequate specimens with younger age and males, and no association with insurance, race/ethnicity, weight-height or BMI percentile, sedation type, or procedure location. SRB under general anesthesia (GA) had 100% adequacy (n=6). Patients with inadequate initial biopsy achieved diagnosis by SRB with increased sedation (n=5) or full thickness biopsy under GA (n=5). CONCLUSION: With adequacy of 80.4% overall and 90.5% for patients greater than 5years, SRB is effective in evaluating the older child for HD.

Synthesis of macroporous poly(dimethylsiloxane) scaffolds for tissue engineering applications.

Macroporous, biostable scaffolds with controlled porous architecture were prepared from poly(dimethylsiloxane) (PDMS) using sodium chloride particles and a solvent casting and particulate leaching technique. The effect of particulate size range and overall porosity on the resulting structure was evaluated. Results found 90% v/v scaffolds and particulate ranges above 100 µm to have the most optimal open framework and porosity. Resulting hydrophobic PDMS scaffolds were coated with fibronectin and evaluated as a platform for adherent cell culture using human mesenchymal stem cells. Biocompatibility of PDMS scaffolds was also evaluated in a rodent model, where implants were found to be highly biocompatible and biostable, with positive extracellular matrix deposition throughout the scaffold. These results demonstrate the suitability of macroporous PDMS scaffolds for tissue engineering applications where strong integration with the host is desired.

Proangiogenic hydrogels within macroporous scaffolds enhance islet engraftment in an extrahepatic site.

The transplantation of allogeneic islets in recent clinical trials has shown substantial promise as a therapy for type 1 diabetes; however, long-term insulin independence remains inadequate. This has been largely attributed to the current intravascular, hepatic transplant site, which exposes islets to mechanical and inflammatory stresses. A highly macroporous scaffold, housed within an alternative transplant site, can support an ideal environment for islet transplantation by providing three-dimensional distribution of islets, while permitting the infiltration of host vasculature. In the present study, we sought to evaluate the synergistic effect of a proangiogenic hydrogel loaded within the void space of a macroporous poly(dimethylsiloxane) (PDMS) scaffold on islet engraftment. The fibrin-based proangiogenic hydrogel tested presents platelet derived growth factor (PDGF-BB), via a fibronectin (FN) fragment containing growth factor and major integrin binding sites in close proximity. The combination of the proangiogenic hydrogel with PDMS scaffolds resulted in a significant decrease in the time to normoglycemia for syngeneic mouse islet transplants. This benefit was associated with an observed increase in competent vessel branching, as well as mature intraislet vessels. Overall, the addition of the proangiogenic factor PDGF-BB, delivered via the FN fragment-functionalized hydrogel, positively influenced the efficiency of engraftment. These characteristics, along with its ease of retrieval, make this combination of a biostable macroporous scaffold and a degradable proangiogenic hydrogel a supportive structure for insulin-producing cells implanted in extrahepatic sites.

Macroporous three-dimensional PDMS scaffolds for extrahepatic islet transplantation.

Clinical islet transplantation has demonstrated success in treating type 1 diabetes. A current limitation is the intrahepatic portal vein transplant site, which is prone to mechanical stress and inflammation. Transplantation of pancreatic islets into alternative sites is preferable, but challenging, as it may require a three-dimensional vehicle to confer mechanical protection and to confine islets to a well-defined, retrievable space where islet neovascularization can occur. We have fabricated biostable, macroporous scaffolds from poly(dimethylsiloxane) (PDMS) and investigated islet retention and distribution, metabolic function, and glucose-dependent insulin secretion within these scaffolds. Islets from multiple sources, including rodents, nonhuman primates, and humans, were tested in vitro. We observed high islet retention and distribution within PDMS scaffolds, with retention of small islets (< 100 µm) improved through the postloading addition of fibrin gel. Islets loaded within PDMS scaffolds exhibited viability and function comparable to standard culture conditions when incubated under normal oxygen tensions, but displayed improved viability compared to standard two-dimensional culture controls under low oxygen tensions. In vivo efficacy of scaffolds to support islet grafts was evaluated after transplantation in the omental pouch of chemically induced diabetic syngeneic rats, which promptly achieved normoglycemia. Collectively, these results are promising in that they indicate the potential for transplanting islets into a clinically relevant, extrahepatic site that provides spatial distribution of islets as well as intradevice vascularization.