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.

Incidence and Operative Factors Associated With Discretional Postoperative Mechanical Ventilation After General Surgery.

BACKGROUND: Mechanical ventilation after general surgery is associated with worse outcomes, prolonged hospital stay, and increased health care cost. Postoperatively, patients admitted to the intensive care unit (ICU) may be categorized into 1 of 3 groups: extubated patients (EXT), patients with objective medical indications to remain ventilated (MED), and patients not meeting these criteria, called "discretional postoperative mechanical ventilation" (DPMV). The objectives of this study were to determine the incidence of DPMV in general surgery patients and identify the associated operative factors. METHODS: At a large, tertiary medical center, we reviewed all surgical cases performed under general anesthesia from April 1, 2008 to February 28, 2015 and admitted to the ICU postoperatively. Patients were categorized into 1 of 3 cohorts: EXT, MED, or DPMV. Operative factors related to the American Society of Anesthesiologists Physical Status (ASA PS), duration of surgery, surgery end time, difficult airway management, intraoperative blood and fluid administration, vasopressor infusions, intraoperative arterial blood gasses, and ventilation data were collected. Additionally, anesthesia records were reviewed for notes indicating a reason or rationale for postoperative ventilation. Categorical variables were compared by χ test, and continuous variables by analysis of variance or Kruskal-Wallis H test. Categorical variables are presented as n (%), and continuous variables as mean ± standard deviation or median (interquartile range) as appropriate. Significance level was set at P≤ .05. RESULTS: Sixteen percent of the 3555 patients were categorized as DPMV and 12.2% as MED. Compared to EXT patients, those classified as DPMV had received significantly less fluid (2757 ± 2728 mL vs 3868 ± 1885 mL; P < .001), lost less blood during surgery (150 [20-625] mL vs 300 [150-600] mL; P< .001), underwent a shorter surgery (199 ± 215 minutes vs 276 ± 143 minutes; P< .001), but received more blood products, 900 (600-1800) mL vs 600 (300-900) mL. The DPMV group had more patients with high ASA PS (ASA III-V) than the EXT group: 508 (90.4%) vs 1934 (75.6%); P< .001. Emergency surgery (ASA E modifier) was more common in the DPMV group than the EXT group: 145 (25.8%) vs 306 (12%), P< .001, respectively. Surgery end after regular working hours was not significantly higher with DPMV status compared to EXT. DPMV cohort had fewer cases with difficult airway when compared to EXT or MED. When compared to MED patients, those classified as DPMV received less fluid (2757 ± 2728 mL vs 4499 ± 2830 mL; P< .001), lost less blood (150 [20-625] mL vs 500 [200-1350] mL; P < .001), but did not differ in blood products transfused or duration of surgery. CONCLUSIONS: In our tertiary medical center, patients often admitted to the ICU on mechanical ventilation without an objective medical indication. When compared to patients admitted to the ICU extubated, those mechanically ventilated but without an objective indication had a higher ASA PS class and were more likely to have an ASA E modifier. A surgery end time after regular working hours or difficult airway management was not associated with higher incidence of DPMV.

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.

Waardenburg Syndrome Type IV De Novo SOX10 Variant Causing Chronic Intestinal Pseudo-Obstruction.

Waardenburg syndrome (WS) type IV is characterized by pigmentary abnormalities, deafness and Hirschsprung's disease. This syndrome can be triggered by dysregulation of the SOX10 gene, which belongs to the SOX (SRY-related high-mobility group-box) family of genes. We discuss the first known case of a SOX10 frameshift mutation variant defined as c.895delC causing WS type IV without Hirschsprung's disease. This female patient of unrelated Kuwaiti parents, who tested negative for cystic fibrosis and Hirschsprung's disease, was born with meconium ileus and malrotation and had multiple surgical complications likely due to chronic intestinal pseudo-obstruction. These complications included small intestinal necrosis requiring resection, development of a spontaneous fistula between the duodenum and jejunum after being left in discontinuity, and short gut syndrome. This case and previously reported cases demonstrate that SOX10 gene sequencing is a consideration in WS patients without aganglionosis but with intestinal dysfunction.

Disparities in pediatric gonadal torsion: Does gender, race and insurance status affect outcomes?

PURPOSE: Ovarian and testicular torsions are emergencies requiring prompt surgical treatment to preserve gonadal function. However, diagnosis in females is often delayed owing to nonspecific symptoms. We sought to assess disparities in management and outcomes between males and females with torsion. METHODS: The National Inpatient Sample was queried for pediatric patients with "emergent", "urgent", or "trauma center" admission and ICD-9 codes for ovarian torsion and testicular torsion. Demographic data, operative procedure, gonadal loss, length of stay (LOS), total charges (TC), and mortality were recorded. RESULTS: There were 2254 unweighted encounters. The average age was 11.56±5.30years for males and 12.55±3.72years for females (p<0.001). Among males, 90% underwent surgery (p<0.001), of which 40% required orchiectomy. Conversely, 73% of females had surgery (p<0.001), of which 78% had oophorectomy. Subsequent analysis with only patients who underwent surgery showed that insurance status (p=0.012), race (p<0.001), and U.S. region (p<0.001) were significantly different between males and females. Gender specific analyses showed that hospital control, hospital location/teaching status, and treatment year were also significant. As such, these six factors in addition to age and gender were used for propensity score matching (PSM). PSM produced two gender cohorts of 755 encounters each. Females had longer LOS (2.44±1.84days vs. 1.28±2.27days for males, p<0.001) and had higher TC ($20,058.44±13,420.82) compared to males ($12,386.58±12,793.34), p<0.001. Logistic regression revealed that males (OR 0.163 [0.130-0.206]) and older patients (age OR 0.924 [0.903-0.946]) were less likely to undergo gonadal loss. Compared to those with private insurance, those with Medicare/Medicaid were more likely to have gonadal loss (1.401 [1.101-1.783]). CONCLUSION: Disparities exist in the management of torsion based on gender. Overall, females had higher charges, had longer hospitalization, and were more likely to have gonadal loss despite current data supporting gonadal preservation for nearly all cases of ovarian torsion. CLINICAL STUDY: Level III Evidence.

Gastroduodenal artery coiling to curb upper gastrointestinal bleeding.

BACKGROUND: Peptic ulcers in pediatric populations are uncommon and can present with upper gastrointestinal bleeding and shock on presentation. An endoscopy is done initially to identify bleeding source. However, definitive treatment is achieved with angiography. The use of coiling is effective in achieving hemorrhagic control in duodenal ulcers or gastric ulcers, particularly in adults. However, the use in pediatric populations is unknown. We present a case of peptic ulcer disease treated with a gastroduodenal artery coil in a pediatric patient that has never been reported in the literature. CASE PRESENTATION: A 15-year-old male with a five-year history of peptic ulcer disease was admitted with an upper gastrointestinal bleed. Angiographic imaging was done to isolate and locate the bleeding, and coil embolization of the gastroduodenal artery was performed. Coils eroded into intestinal lumen but bleeding was controlled. CONCLUSION: Peptic ulcers in pediatric populations are rare and complex in nature. Besides aggressive resuscitation and endoscopy, other methods to control bleeding such as coil embolization can be performed. Further investigations are needed to understand long-term effects of coil embolization in pediatric peptic ulcer patients. LEVEL OF EVIDENCE: 5/Case Report/.

Effectiveness of a Perioperative Transthoracic Ultrasound Training Program for Students and Residents.

OBJECTIVE: Focused ultrasound (US) is being incorporated across all levels of medical education. Although many comprehensive US courses exist, their scope is broad, requiring expert instructors, access to simulation, and extensive time commitment by the learner. We aim to compare learning across levels of training and specialties using a goal-directed, web-based course without live skills training. DESIGN: A prospective observational study of students and residents from medicine, surgery, and anesthesiology. Analysis compared pretests and posttests assessing 3 competencies. Individual mean score improvement (MSI) was compared by paired-sample t-tests and MSI among cohorts by analysis of variance, with significance set at p ≤ 0.05. McNemar test compared those who agreed or strongly agreed with survey items with those who did not before and after intervention. SETTING: Jackson Memorial Hospital, Miami, FL residency training programs in Medicine, Surgery, and Anesthesiology. RESULTS: A total of 180 trainees participated. A significant MSI was noted in each of 3 competencies in all 3 cohorts. Students' (S) MSI was significantly higher than residents' (R) and interns' (I) in US "knobology" and window recognition [S = 2.28 ± 1.29/5 vs R = 1.63 ± 1.21/5 (p = 0.014); vs I = 1.59 ± 1.12/5 (p = 0.032)]; students' total score MSI was significantly higher than residents [7.60 ± 3.43/20 vs 5.78 ± 3.08/20 (p < 0.008)]. All cohorts reported improved comfort in using transthoracic US and improved ability to recognize indications for use. More than 81% of all participants reported improved confidence in performing transthoracic US; more than 91% reported interest in additional training; and more than 88% believed course length was appropriate. CONCLUSIONS: Learners across levels of medical training and specialties can benefit from a brief, goal-directed, web-based training with early incorporation producing maximal yield.