Mary T. Austin

Melanoma incidence rises for children and adolescents: An epidemiologic review of pediatric melanoma in the United States

BACKGROUND/PURPOSE This study was conducted to determine the influence of age on disease presentation and evaluate the change in pediatric melanoma incidence between 1998 and 2007. METHODS We performed a retrospective review of all children ≤18 years with cutaneous melanoma who were included in the 2007 National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database between 1988 and 2007. RESULTS We identified a total of 1447 patients with cutaneous melanoma. The overall average annual melanoma incidence was 5.4 per 1 million children and adolescents in the U.S., which increased throughout the study period. Most patients (89%) were at least 10 years of age (average age 15 years). Melanoma in situ (21%), thin (<1 mm) lesions (37%), stage I disease (46%), and superficial spreading histology (25%) were common at presentation. Only 1% of patients presented with distant metastases. Preadolescents younger than age 10 were ethnically more diverse and more likely to present with non-truncal primaries and advanced disease (P<.01) compared to adolescents. CONCLUSIONS The incidence of pediatric melanoma in the U.S. is increasing. There are significant differences between children and adolescents which suggest age-based inherent differences in the biology of the disease may exist.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

OBJECTIVE Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Health disparities are important determinants of outcome for children with solid tumor malignancies

PURPOSE The purpose of this study was to identify health disparities in children with non-CNS solid tumor malignancies and examine their impact on disease presentation and outcome. METHODS We examined the records of all children (age≤18years) diagnosed with a non-CNS solid tumor malignancy and enrolled in the Texas Cancer Registry between 1995 and 2009 (n=4603). The primary outcome measures were disease stage and overall survival (OS). Covariates included gender, age, race/ethnicity, year of diagnosis, socioeconomic status (SES), and driving distance to the nearest pediatric cancer treatment facility. Statistical analyses included life table methods, logistic, and Cox regression. Statistical significance was defined as p<0.05. RESULTS Children with advanced-stage disease were more likely to be male, <10years old, and Hispanic or non-Hispanic Blacks (all p<0.05). Distance to treatment and SES did not impact stage of disease at presentation. However, Hispanic and non-Hispanic Blacks and patients in the lowest SES quartile had the worst 1- and 5-year survival (all p<0.05). The adjusted OS differed by age, race, and stage, but not SES or distance to the nearest treatment facility. CONCLUSIONS Race/ethnicity plays an important role in survival for children with non-CNS solid tumor malignancies. Future work should better define these differences to establish mechanisms to decrease their impact.

Health disparities and impact on outcomes in children with primary central nervous system solid tumors.

OBJECTIVE Health disparities in access to care, early detection, and survival exist among adult patients with cancer. However, there have been few reports assessing how health disparities impact pediatric patients with malignancies. The objective in this study was to examine the impact of racial/ethnic and social factors on disease presentation and outcome for children with primary CNS solid tumors. METHODS The authors examined all children (age ≤ 18 years) in whom CNS solid tumors were diagnosed and who were enrolled in the Texas Cancer Registry between 1995 and 2009 (n = 2421). Geocoded information was used to calculate the driving distance between a patients home and the nearest pediatric cancer treatment center. Socioeconomic status (SES) was determined using the Agency for Healthcare Research and Quality formula and 2007-2011 US Census block group data. Logistic regression was used to determine factors associated with advanced-stage disease. Survival probability and hazard ratios were calculated using life table methods and Cox regression. RESULTS Children with advanced-stage CNS solid tumors were more likely to be < 1 year old, Hispanic, and in the lowest SES quartile (all p < 0.05). The adjusted odds ratios of presenting with advanced-stage disease were higher in children < 1 year old compared with children > 10 years old (OR 1.71, 95% CI 1.06-2.75), and in Hispanic patients compared with non-Hispanic white patients (OR 1.56, 95% CI 1.19-2.04). Distance to treatment and SES did not impact disease stage at presentation in the adjusted analysis. Furthermore, 1- and 5-year survival probability were worst in children 1-10 years old, Hispanic patients, non-Hispanic black patients, and those in the lowest SES quartile (p < 0.05). In the adjusted survival model, only advanced disease and malignant behavior were predictive of mortality. CONCLUSIONS Racial/ethnic disparities are associated with advanced-stage disease presentation for children with CNS solid tumors. Disease stage at presentation and tumor behavior are the most important predictors of survival.

Surgical management of gastroesophageal reflux disease (GERD) in children: A systematic review

OBJECTIVE The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the surgical treatment of pediatric gastroesophageal reflux disease (GERD). METHODS Five questions were addressed by searching the MEDLINE, Cochrane, Embase, Central, and National Guideline Clearinghouse databases using relevant search terms. Consensus recommendations were derived for each question based on the best available evidence. RESULTS There was insufficient evidence to formulate recommendations for all questions. Fundoplication does not affect the rate of hospitalization for aspiration pneumonia, apnea, or reflux-related symptoms. Fundoplication is effective in reducing all parameters of esophageal acid exposure without altering esophageal motility. Laparoscopic fundoplication may be comparable to open fundoplication with regard to short-term clinical outcomes. Partial fundoplication and complete fundoplication are comparable in effectiveness for subjective control of GERD. Fundoplication may benefit GERD patients with asthma, but may not improve outcomes in patients with neurologic impairment or esophageal atresia. Overall GERD recurrence rates are likely below 20%. CONCLUSIONS High-quality evidence is lacking regarding the surgical management of GERD in the pediatric population. Definitive conclusions regarding the effectiveness of fundoplication are limited by patient heterogeneity and lack of a standardized outcomes reporting framework. TYPE OF STUDY Systematic review of level 1-4 studies. LEVEL OF EVIDENCE Level 1-4 (mainly level 3-4).

Adherence to surgical antibiotic prophylaxis remains a challenge despite multifaceted interventions

BACKGROUND Adherence to prophylactic antibiotics guidelines is challenging and poorly documented. We hypothesized that a multiphase, multifaceted quality improvement initiative would engage relevant stakeholders, address known barriers to adoption, and improve overall adherence. METHODS From 2011 to 2014, a series of interventions were introduced in the pediatric operating rooms. After each interventional period, prospective assessments were performed to record the antibiotic type, dose, timing, and redosing according to the guidelines. Perioperative factors that may influence guideline adherence were analyzed. Spearmans rank correlation, analysis of variance, and χ(2) tests were performed. RESULTS A total of 1,052 operations were observed, and 629 (60%) required prophylactic antibiotics. Adherence to all 4 guideline components remained unchanged (54-55%, P = .38). Redosing significantly improved (7-53%, P = .02), but correct type decreased (98-70%, P < .01). The percentage of cases in which only one antibiotic guideline component was missed remained unchanged (35-34%, P = .46). Adherence to guidelines was not significantly associated with American Society of Anesthesiologists class, surgical specialty, patient weight, anesthesia provider, or surgical wound class. CONCLUSION Despite multiple interventions to improve antibiotic prophylaxis, overall adherence did not improve. Most interventions were directed at the point of administration in the operating room; future implementation strategies should focus on the perioperative setting.

Prevention of infectious complications after elective colorectal surgery in children: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee comprehensive review

OBJECTIVE This goal of this review was to examine the clinical evidence in support of commonly utilized measures intended to reduce complications following elective colorectal surgery. DATA SOURCE Literature searches were performed to identify relevant studies from Medline, PubMed, and Cochrane databases. STUDY SELECTION The American Pediatric Surgery Association Outcomes and Clinical Trials Committee selected eight questions to address this topic systematically in the context of three management areas: 1) appropriate utilization of systemic antibiotics for colorectal procedures, 2) reduction of stool burden through mechanical bowel preparation, and 3) intraluminal gut decontamination through use of enteral nonabsorbable antibiotics. Primary outcomes of interest included the occurrence of infectious and mechanical complications related to stool burden and intraluminal bacterial concentration (incisional surgical site infection, anastomotic leakage, and intraabdominal abscess). RESULTS The evidence in support of each management category was systematically reviewed, graded, and summarized in the context of the reviews primary outcomes. Practice recommendations were made as deemed appropriate by the committee. CONCLUSIONS Clinical evidence in support of interventions to reduce infectious complications following colorectal surgery is derived almost exclusively from the adult literature. High-quality evidence to guide clinical practice in children is sorely needed, as the available data may have only limited relevance to pediatric colorectal diseases.

Complications in the surgical treatment of pediatric melanoma

PURPOSE The purpose of this study was to characterize the complications associated with surgical treatment of pediatric melanoma. METHODS We retrospectively reviewed all pediatric patients who received surgical treatment for melanoma at our institution between 1992 and 2010. We compared complications between three groups: wide local excision only (WLE), WLE and sentinel lymph node biopsy (SLNB), and WLE and completion lymph node dissection (CLND). RESULTS One hundred twenty-five patients were identified: 37 patients received WLE only, 47 received WLE and SLNB, and 41 patients had WLE and CLND. Complication rates differed between the three groups: 19% in WLE, 11% in WLE+SLNB, and 39% in WLE+CLND (P=.006). The risk of complications was significantly lower among patients having WLE+SLNB versus WLE+CLND (OR 0.19, 95% CI 0.06-0.57, P=.0032). Lymphedema was a common complication with a higher incidence in the CLND group compared to the SLNB group (19.5% vs. 2.1%, P=.01). Complications were more frequent in inguinal compared to axillary dissections (52.0% vs. 17.1%, P=.006). CONCLUSIONS In the surgical treatment of pediatric melanoma, the addition of a completion lymph node dissection significantly increases complication risk. Thus, it is critical to determine which patients truly benefit from this procedure.

Effect of concurrent metastatic disease on survival in children and adolescents undergoing lung resection for metastatic osteosarcoma

PURPOSE To evaluate the impact of treated extra-pulmonary metastatic disease on overall (OS) and event-free survival (EFS) for pediatric osteosarcoma patients undergoing pulmonary metastatectomy. METHODS We retrospectively reviewed pediatric patients who were treated for osteosarcoma at our institution from 2001 to 2011 and received pulmonary metastatectomy (n=76). We compared OS and EFS between patients with metastases limited to the lungs (Group A, n=58) to those with treated extra-pulmonary metastases (Group B, n=18) at the time of first pulmonary metastatectomy. RESULTS The estimated median OS and EFS from first pulmonary metastatectomy were 2.0years (95% CI 1.5-2.8years) and 5.5months (95% CI 3.0-8.1months), respectively. Median OS was significantly greater for Group A (2.6years, 95% CI 1.9-3.8) compared to Group B (0.9years, 95% CI 0.6-1.5) (log rank p=0.0001). Median EFS was significantly greater for Group A (7.9months, 95% CI 5.0-10.7) compared to Group B (1.6months, 95% CI 0.8-2.7) (log rank p<0.0001). Independent predictors of OS included extra-pulmonary metastatic disease at the time of first thoracotomy, bilateral pulmonary metastases, and >4 nodules resected at first thoracotomy (all p<0.001). CONCLUSIONS Osteosarcoma patients with treated extra-pulmonary metastatic disease at the time of pulmonary metastatectomy have significantly worse survival compared to those with disease limited to the lungs.

Clinical outcomes of splenectomy in children

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short‐term adverse events (AEs) (≤30 days after surgery), and long‐term AEs (31–365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short‐term AEs and 11% rate of long‐term AEs. As we accumulate more subjects and longer follow‐up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision‐making process. Am. J. Hematol. 90:187–192, 2015.