Development and Validation of a Scale to Measure Person-Centered Care in Fetal Care Centers.

INTRODUCTION: Fetal care centers (FCCs) in the USA lack a standardized instrument to measure person-centered care. This study aimed to develop and validate the Person-Centered Care in Fetal Care Centers (PCC-FCC) Scale. METHODS: Initial items were developed based on literature and input from clinicians and former patients. A Delphi study involving 16 experts was conducted to validate the content and construct. Through three rounds of online questionnaires using open-ended questions and Likert scales, consensus on item clarity and relevancy was established. The resulting items were then piloted with former fetal care center patients via a web-based survey. The instrument's reliability and validity were validated using Cronbach's α and exploratory factor analysis, respectively. Concurrent validity was assessed by comparing scores with the Revised Patient Perception of Patient-Centeredness (PPPC-R) Questionnaire. RESULTS: 258 participants completed the 48-item pilot PCC-FCC survey, categorized into six domains. Factor analysis yielded a 2-factor, 28-item scale. Internal consistency of the final scale had good reliability (α = 0.969). Data supported content, construct, and concurrent validity. CONCLUSION: The PCC-FCC Scale is a reliable and valid measure of person-centered care in U.S. FCCs. It can be used to enhance services and begin connecting person-centered care to maternal-child health outcomes.

Diagnostic utility of PRAME in distinguishing proliferative nodules from melanoma in giant congenital melanocytic nevi.

We describe a case of a melanocytic proliferation arising in a giant congenital melanocytic nevus (CMN) and outline the potential utility of an immunohistochemical study with PReferentially expressed Antigen in MElanoma (PRAME) in distinguishing benign proliferative nodules (PN) from melanoma in this context. A 15-day-old girl presented with a fibrotic nodule clinically suspicious for melanoma within a giant CMN. Histopathological examination showed a predominantly intradermal melanocytic nevus with congenital features intermixing with an ill-defined proliferation of larger melanocytes demonstrating mild-to-moderate cytologic atypia and increased mitotic activity. Anti-PRAME was diffusely positive within the congenital nevus while negative within the larger proliferating cells. Chromosomal microarray analysis revealed whole chromosomal gains and losses only, consistent with a PN arising in a giant CMN. To our knowledge, PRAME expression in giant CMN, PN, and pediatric melanomas has not been previously described. Based on our experience with this case, we propose that differential patterns of PRAME expression may be present in these three lesions, allowing PRAME immunohistochemistry to potentially serve as a helpful adjunct diagnostic tool for laboratories that do not readily have access to molecular testing in rendering a diagnosis for atypical melanocytic proliferations arising in giant CMN.

Low Transverse versus Midline Abdominal Skin Incisions for in utero Spina Bifida Repair.

OBJECTIVE: The 2 types of maternal skin incisions for in utero spina bifida repair are low transverse (LT) incision perceived to be cosmetic benefit and midline longitudinal (ML) incision, typically associated with a reduction in surgical time and lower blood loss. Our objective was to compare short- and long-term outcomes associated with these 2 types of skin incisions following in utero spina bifida repair. METHODS: Prospective observational cohort of 72 patients undergoing fetal spina bifida repair at a single institution between September 2011 and August 2018. The decision for the type of incision was at the discretion of the surgeons. The primary outcome was total operative time. Secondary outcomes included an analog scale of wound pain score on postoperative day 3, duration of postoperative stay, and postoperative wound complications within the first 4 weeks. The Patient Scar Assessment Questionnaire, a validated questionnaire, was obtained for all patients (≥6 months from delivery) using 4 categories (appearance, consciousness, satisfaction with appearance and with symptoms), with higher scores reflecting a poorer perception of the scar. RESULTS: There were 43 women (59.7%) in the LT group and 29 (40.3%) in the ML group. In all patients, the same incision was used during cesarean delivery. The total operative time was higher in the LT group by 33 min (p < 0.001), primarily due to abdominal wall incision time (open and closure). No significant differences were found between the groups in pain score, length of postoperative stay, or the rate of wound complications. Fifty-three patients (73.6%) responded to the questionnaire, 36/43 from the LT group and 17/29 from the ML group. There was no difference in the scores of appearance, consciousness, and satisfaction with appearance and symptoms between the groups. CONCLUSION: ML incisions shorten operative times without altering long-term incision-related satisfaction when compared to LT incisions.

Barriers to Transition to Home From the Neonatal Intensive Care Unit: A Qualitative Perspectives of Parents and Healthcare Providers.

The objective of this study was to explore the challenges faced by parents of former neonatal intensive care unit (NICU) patients in transitioning home from parents' and healthcare providers' perspective. We conducted semistructured individual and group interviews with parents of former NICU patients and healthcare providers. Themes from the individual interviews framed the group interviews' contents. The group interviews were recorded and transcribed, and thematic analysis was performed to identify themes. We conducted individual and group interviews with 16 parents and 33 inpatient and outpatient providers from November 2017 to June 2018. Individual interview participants identified several barriers experienced by parents when transitioning their infant home from the NICU including parental involvement and engagement during NICU stay and during the discharge process. Further exploration within group interviews revealed opportunities to improve discharge communication and processes, standardization of parental education that was lacking due to NICU resource constraints, support for parents' emotional state, and use of technology for infant care in the home. Parents of NICU patients face serious emotional, logistical, and knowledge challenges when transitioning their infant home from the NICU. Understanding and mitigating the challenges of transitioning infants from NICU to home require multistakeholder input from both parents and providers.

Povidone-iodine Irrigation for Pediatric Perforated Appendicitis May Be Protective: A Bayesian Pilot Randomized Controlled Trial.

OBJECTIVES: A randomized controlled trial was conducted to test the hypothesis that povidone-iodine (PVI) irrigation versus no irrigation (NI) reduces postoperative intra-abdominal abscess (IAA) in children with perforated appendicitis. METHODS: A 100 patient pilot randomized controlled trial was conducted. Consecutive patients with acute perforated appendicitis were randomized (1:1) to PVI or NI from April 2016 to March 2017 and followed for 1 year. Patients and postoperative providers were blinded to allocation. The primary endpoint was 30-day image-confirmed IAA. Secondary outcomes included initial and total 30-day length of stay (LOS), emergency department (ED) visits, and readmissions. Intention-to-treat analyses were performed to estimate the probability of clinical benefit using Bayesian regression models (an optimistic prior for the primary outcome and neutral priors for secondary outcomes). Frequentist statistics were also used. RESULTS: Baseline characteristics were similar between treatment arms. The PVI arm had 12% postoperative IAA versus 16% in the NI arm (relative risk 0.72, 95% credible interval 0.38-1.23). Bayesian analysis estimates 89% probability that PVI reduces IAA. High probability of benefit was seen in all secondary outcomes for the PVI arm: fewer ED visits and readmissions, and shorter initial and total 30-day LOS. The probability of benefit in reduction of total 30-day LOS in PVI patients was 96% and was significant (P = 0.05) on frequentist analysis. CONCLUSIONS: PVI irrigation for perforated appendicitis in children demonstrated a strong probability of reduction in postoperative IAA with a high probability of decreased LOS. With the favorable probability of benefit in all outcomes, this pilot study serves as evidence to continue a definitive trial.

Preoperative Opioids Associated With Increased Postoperative Opioid Use in Pediatric Appendicitis.

BACKGROUND: In light of current opioid-minimization efforts, we aimed to identify factors that predict postoperative opioid requirement in pediatric appendicitis patients. METHODS: A single-center retrospective cohort study was conducted of children (<18 y) who underwent laparoscopic appendectomy for acute appendicitis between January 1, 2018 and April 30, 2019. Patients who underwent open or interval appendectomies were excluded. The primary outcome was morphine milliequivalents (MMEs) per kilogram administered between 2 and 24 h after surgery. Multivariable analyses were performed to evaluate predictors of postoperative opioid use. Clinically sound covariates were chosen a priori: age, weight, simple versus complicated appendicitis, preoperative opioid administration, and receipt of regional or local anesthesia. RESULTS: Of 546 patients, 153 (28%) received postoperative opioids. Patients who received postoperative opioids had a longer median preadmission symptom duration (48 versus 24 h, P < 0.001) and were more likely to have complicated appendicitis (55% versus 21%, P < 0.001). Patients who received postoperative opioids were more likely to have received preoperative opioids (54% versus 31%, P < 0.001). Regional and local anesthesia use was similar between groups. Nearly all patients (99%) received intraoperative opioids. Each preoperative MME per kilogram that a patient received was associated with receipt of 0.29 additional MMEs per kilogram postoperatively (95% confidence interval, 0.19-0.40). CONCLUSIONS: Preoperative opioid administration was independently associated with increased postoperative opioid use in pediatric appendicitis. These findings suggest that preoperative opioids may potentiate increased postoperative pain. Limiting preoperative opioid exposure, through strategies such as multimodal analgesia, may be an important facet of efforts to reduce postoperative opioid use.

Fractional Limb Volume in Spina Bifida Fetuses as an Assessment Tool for Postnatal Ambulation.

BACKGROUND: Prenatal fractional limb volume (FLV) can be used to assess muscle atrophy in fetuses with myelomeningocele. OBJECTIVE: We hypothesize that FLV in fetal myelomeningocele (fMMC) repair is different from postnatal repair (PNR). Assessing intrauterine muscle development can predict ambulation. METHODS: A prospective observational study was performed from July 2012 to April 2016. Demographics, clinical outcomes, and FLV of the fetal thigh were assessed by ultrasound. Ambulation videos were collected from patients over 30 months of age. FLV was compared between the fMMC and PNR groups and between ambulators and non-ambulators. Two-sample t test, ANOVA, Spearman's rho correlation, and Bland-Altman plots were used for analysis. A p value <0.05 was used for statistical significance. RESULTS: Fifty-nine patients were included, 24 had fMMC and 35 had PNR. Videos were obtained in 47 cases (73%). There was no difference in baseline demographics between the groups. There was no significant change in the fMMC group between the FLV at initial presentation and the repeat at 34 weeks gestation (54.5 ± 28.2 and 62.2% ± 16.4; p = 0.6). In contrast, the FLV in the PNR decreased between the initial evaluation and the repeat at 34 weeks (54.1 ± 27.7 to 35.8 ± 34.1%; p = 0.04). FLV at 34 weeks gestation was higher in the fMMC group as compared to the PNR group (62.2 ± 16.4 vs. 35.8 ± 34.1%; p = 0.02). There was no difference in FLV between ambulators and non-ambulators either at initial evaluation (p = 0.8) or at 34 weeks gestation (p = 0.6). CONCLUSION: Lower FLV in the PNR group compared to fMMC may suggest in utero muscle atrophy. No correlation was seen between FLV and subsequent ambulation; however, future larger studies may be needed.

The Impact of Sociodemographic and Hospital Factors on Length of Stay Before and After Pyloromyotomy.

BACKGROUND: The aim of the study was to determine whether racial or ethnic and insurance disparities exist in pre- and post-operative length of stay (LOS) in patients with hypertrophic pyloric stenosis (HPS). MATERIALS AND METHODS: The Healthcare Cost and Utilization Project Kid's Inpatient Database database (years 2006, 2009, and 2012) was analyzed for patients aged <1 y with HPS with a primary procedure of pyloromyotomy. Multivariate logistic regression was performed to determine the association between race or ethnicity and insurance status with the primary outcomes of prolonged pre- and post-operative LOS (defined as >1 d). Odds ratios (ORs) and 95% confidence intervals (CIs) were tabulated using SPSS v24. RESULTS: A total of 13,706 cases were identified: 8503 (62%) non-Hispanic whites, 3143 (23%) Hispanics, 1007 (7%) non-Hispanic blacks (NHB), and 1053 (8%) non-Hispanic other race or ethnicity. NHB and Hispanics were 45% and 37%, respectively, more likely to have prolonged preoperative LOS compared with non-Hispanic whites (OR = 1.45, 95% CI: 1.19-1.77; OR = 1.37, 95% CI: 1.18-1.60, respectively). Children with public insurance had 21% increased odds of increased preoperative LOS (OR = 1.21, 95% CI: 1.06-1.38). All minority groups had increased odds of postoperative LOS (NHB OR 1.36, 95% CI: 1.17-1.54; Hispanic OR 1.14, 95% CI: 1.03-1.26; NHO OR 1.31, 95% CI: 1.15-1.51). CONCLUSIONS: We conclude that NHB, Hispanics, and other race or ethnicity were more likely to have prolonged pre- and post-operative LOS. In addition, children with public insurance were more likely to have prolonged preoperative LOS. Further work is needed to better characterize and eliminate disparities in the management and outcomes of children with HPS.

Early Administration of Magnesium Sulfate during Open Fetal Myelomeningocele Repair Reduces the Dose of Inhalational Anesthesia.

INTRODUCTION: Optimal uterine relaxation is achieved through higher minimum alveolar concentration (MAC) of inhalational anesthetics, increasing risks to mother and fetus. Our objective was to determine if earlier administration of magnesium sulfate would reduce the requirement of inhalational anesthetics in fetal myelomeningocele repair. MATERIALS AND METHODS: Prospective observational study of fetal myelomeningocele repair was performed from September 2011 to August 2017. Groups compared were: (1) magnesium sulfate at uterine closure; (2) magnesium sulfate at maternal skin incision. Maternal demographics, anesthetic agents, intraoperative complications, and pregnancy outcomes were reviewed. A Student t test was used for analysis. RESULTS: There were 30 cases in group 1 and 21 cases in group 2. There was no difference in gestational age at intervention (24.92 ± 0.62 vs. 25.22 ± 0.47 weeks, p = 0.07) or at delivery (34.83 ± 2.77 vs. 33.98 ± 3.83 weeks, p = 0.38) between groups 1 and 2, respectively. The maximum MAC of sevoflurane was significantly lower in group 2 (1.84 ± 0.25 vs. 1.05 ± 0.28, p < 0.0001). There was no difference in the average dose of phenylephrine used. DISCUSSION: Magnesium sulfate infusion initiated earlier in open fetal surgery reduces the total anesthetic exposure to the fetus.

Retroperitoneal lymph node staging in paratesticular rhabdomyosarcoma-are we meeting expectations?

BACKGROUND: Staging retroperitoneal lymph node dissection (RPLND) for paratesticular rhabdomyosarcoma (RMS) is recommended for all patients aged ≥10 y. The purpose of this study was to evaluate adherence with surgical resection guidelines for RPLND in patients with paratesticular RMS as a measure for surgical quality. MATERIALS AND METHODS: All patients with paratesticular RMS were identified in the Surveillance, Epidemiology, and End Results database from 1973 to 2012. Patients were divided into two eras to reflect before (1973-2002) and after (2003-2012) the release and dissemination of the 2001 surgical guidelines for staging ipsilateral RPLND in all patients aged ≥10 y with paratesticular RMS. Survival outcomes associated with lymph node dissection were calculated using the Kaplan-Meier method and Cox proportional hazards analysis. RESULTS: Two hundred thirty-five patients with paratesticular RMS were identified and included in the study, among whom 111 were adolescents aged 10-20. RPLND did not significantly increase after 2003 among adolescents (45%-61%, P = 0.09). The benefit of RPLND on improved 5-y overall survival was evident among adolescents (92% versus 64%, P = 0.003). Adjusting for histology, age, stage at diagnosis, and race/ethnicity, RPLND was associated with improved overall survival among patients aged ≥10 y (hazard ratio 0.37, 95% confidence interval 0.17-0.83). CONCLUSIONS: Despite surgical guidelines recommending RPLND in pediatric patients aged ≥10 y, nearly one-third of adolescent patients did not undergo RPLND. These findings are disturbing considering the survival benefit associated with RPLND among adolescent patients and indicate an opportunity for improvement in surgical quality.

Are we missing the near misses in the OR?-underreporting of safety incidents in pediatric surgery.

BACKGROUND: Electronic hospital variance reporting systems used to report near misses and adverse events are plagued by underreporting. The purpose of this study is to prospectively evaluate directly observed variances that occur in our pediatric operating room and to correlate these with the two established variance reporting systems in our hospital. MATERIALS AND METHODS: Trained individuals directly observed pediatric perioperative patient care for 6 wk to identify near misses and adverse events. These direct observations were compared to the established handwritten perioperative variance cards and the electronic hospital variance reporting system. All observations were analyzed and categorized into an additional six safety domains and five variance categories. The chi-square test was used, and P-values < 0.05 were considered statistically significant. RESULTS: Out of 830 surgical cases, 211 were audited by the safety observers. During this period, 137 (64%) near misses were identified by direct observation, while 57 (7%) handwritten and 8 (1%) electronic variance were reported. Only 1 of 137 observed events was reported in the handwritten variance system. Five directly observed adverse events were not reported in either of the two variance reporting systems. Safety observers were more likely to recognize time-out and equipment variances (P < 0.001). Both variance reporting systems and direct observation identified numerous policy and process issues. CONCLUSIONS: Despite multiple reporting systems, near misses and adverse events remain underreported. Identifying near misses may help address system and process issues before an adverse event occurs. Efforts need to be made to lessen barriers to reporting in order to improve patient safety.

Tunneled central venous catheters in pediatric intestinal failure: a single-center experience.

BACKGROUND: Parenteral nutrition for intestinal failure (IF) often requires a tunneled central venous catheter (CVC). The purpose of this study was to characterize complications after CVC placement and contributors to line loss in pediatric IF patients. METHODS: An institutional review board-approved retrospective review of pediatric (<18 y) IF patients who had a silicone tunneled CVC newly inserted or exchanged from 2012 to 2016 in an IF center was conducted. Patient demographics, procedure service (surgery versus interventional radiology), procedure type (new versus exchange), vessel, and complications related to CVCs were evaluated. Complications included dislodgement, infection, break, occlusion/malfunction, and others. An ethanol-lock protocol for silicone CVCs in IF patients was instituted in January 2012. RESULTS: Twenty-nine IF patients with tunneled CVCs were identified with 182 lines and 18,534 line d. Median age at line insertion was 17.1 mo (interquartile range [IQR] 7.6-31.5) with a median of five catheters (IQR 2-8) per patient. There were 19.2 complications per 1000 line d. Occlusions/malfunctions were the most common complication (6.0/1000 line d) followed by breaks (5.6/1000 line d). Median life of catheters was 51.5 d (IQR 21-129). On regression, adjusting for age, insertion service, and procedure type, shorter line life was associated with younger age (P = 0.04) and placement by interventional radiology (P < 0.01). Dislodgement was associated with newly placed lines relative risk 6.5 (95% CI 2.2-28.8). CONCLUSIONS: CVCs in pediatric IF patients have frequent complications and short line lifetimes. Dislodgement of CVC was an unexpectedly common complication with loss of access in newly placed lines. There may be modifiable processes to mitigate CVC complications.

Increased complications in pediatric surgery are associated with comorbidities and not with Down syndrome itself.

BACKGROUND: Down syndrome (DS) is a genetic condition associated with multiple comorbidities. While physicians may perceive that DS patients have more postoperative complications, the literature remains unclear. This study compared postoperative complications for children with and without DS who underwent abdominal and thoracic procedures. METHODS: The National Surgical Quality Improvement Program Pediatric was queried for patients aged <18 years, who underwent abdominal and noncardiac thoracic operations (by Current Procedural Terminology codes) from 2012 to 2015. The analysis compared patients based on the presence or absence of DS. The primary outcome was a composite of all postoperative complications as defined by the National Surgical Quality Improvement Program Pediatric. The analysis utilized chi-square, Student's t-test, and univariate and multiple logistic regression. RESULTS: There were 91,478 patients included, of which 1476 (1.6%) had a diagnosis of DS. Patients with DS had higher rates of preoperative nutritional support (38.8% versus 15.0%), developmental delay (61.9% versus 10.4%), and cardiac risk factors (76.5% versus 13.8%). The overall rate of postoperative complications was 11.1%, with a greater proportion in DS patients (16.2% versus 10.8%, P < 0.001). On univariate analysis, DS was associated with increased odds of postoperative complications (odds ratio 1.6 95% confidence interval 1.4-1.9) compared with the non-DS group; however, DS was not a risk factor after adjusting for other covariates (adjusted odds ratio 0.86 95% confidence interval 0.7-1.1). CONCLUSIONS: A higher proportion of postoperative complications were observed in patients with DS. However, after adjusting for other risk factors, DS was not an independent risk factor. The increased rate of complications is likely related to the presence of multiple comorbidities in DS.

An overview of disparities in childhood cancer: Report on the Inaugural Symposium on Childhood Cancer Health Disparities, Houston, Texas, 2016.

The Inaugural Symposium on Childhood Cancer Health Disparities was held in Houston, Texas, on November 2, 2016. The symposium was attended by 109 scientists and clinicians from diverse disciplinary backgrounds with interests in pediatric cancer disparities and focused on reviewing our current knowledge of disparities in cancer risk and outcomes for select childhood cancers. Following a full day of topical sessions, everyone participated in a brainstorming session to develop a working strategy for the continued expansion of research in this area. This meeting was designed to serve as a springboard for examination of childhood cancer disparities from a more unified and systematic approach and to enhance awareness of this area of need.

Surgical Feeding Tubes in Pediatric and Adolescent Cancer Patients: A Single-institution Retrospective Review.

The purpose of our study was to evaluate surgical enteric access in pediatric cancer patients to determine factors associated with postoperative complications. We performed a single-institution retrospective review of all patients below 21 years old with a primary cancer diagnosis who underwent surgical procedures for enteral access between 2004 and 2014. Multivariate logistic regression was performed to determine independent predictors of postoperative complications. During the study period, 122 patients had surgically placed feeding tubes, of whom 58% developed ≥1 complication(s) and 16% experienced a major complication. No single factor was significantly associated with developing any complication or major complication. Several trends were noted including increased complications associated with jejunostomy tubes, percutaneous endoscopic gastrostomy tubes, and abdominal radiation. Surgically placed enteric access in pediatric and adolescent cancer patients is associated with an extremely high complication rate emphasizing the importance of careful evaluation of these patients before embarking on surgical feeding access. Future work should evaluate mechanisms to decrease complications and/or explore alternative methods to provide supplemental nutrition in children and adolescents with cancer.

Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee.

PURPOSE: Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. METHODS: Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. RESULTS: 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. CONCLUSIONS: Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.

The Impact of Socioeconomic Status on Appendiceal Perforation in Pediatric Appendicitis.

OBJECTIVE: To assess the impact of socioeconomic status (SES) on pediatric appendicitis outcomes using the validated Agency for Healthcare Research and Quality (AHRQ) SES Index and incorporating block-group data. STUDY DESIGN: We reviewed all patients <18 years old who underwent appendectomy for acute appendicitis from 2009-2013 at our institution. Patient addresses were geocoded and linked to 2010 US Census SES block-group data to determine composite AHRQ SES Index scores based on 7 publically reported SES variables. The primary outcome was appendiceal perforation, and the impact of SES scores, age, race, and insurance status on perforation rates were assessed through regression analyses. RESULTS: Of 1501 patients, 510 (34%) had perforated appendicitis. On bivariate analysis, components of the SES Index associated with an increased perforation rate included lower household income, lower percentage of adults with college education, and higher percentage of adults with <12th grade education (all P < .05). On multivariate analysis, age ≤ 10 years (OR 1.7, 95% CI 1.4-2.2) and public insurance (OR 1.5, 95% CI 1.2-2.0) were associated with increased odds of perforation. CONCLUSIONS: This study used the AHRQ SES scoring system to evaluate SES and its influence on appendiceal perforation. Among our cohort of pediatric patients, the risk of perforation was multifactorial, and younger age and public insurance were stronger predictors of perforation than SES.

Health Disparities Influence Childhood Melanoma Stage at Diagnosis and Outcome.

OBJECTIVE: To identify health disparities in pediatric patients with melanoma that affect disease presentation and outcome. STUDY DESIGN: This was a retrospective cohort study of all persons aged ≤18 years diagnosed with melanoma and enrolled in the Texas Cancer Registry between 1995 and 2009. Socioeconomic status (SES) and driving distance to the nearest pediatric cancer treatment center were calculated for each patient. Logistic regression was used to determine factors associated with advanced-stage disease. Life table methods and Cox regression were used to estimate survival probability and hazard ratios. RESULTS: A total of 185 adolescents (age >10 years) and 50 young children (age ≤10 years) were identified. Hispanics (n = 27; 12%) were 3 times more likely than non-Hispanic whites (n = 177; 75%) to present with advanced disease (OR, 3.8; 95% CI, 1.7-8.8). Young children were twice as likely as adolescents to present with advanced disease (OR, 2.2; 95% CI, 1.1-4.3). Distance to treatment center and SES did not affect stage of disease at presentation. Hispanics and those in the lowest SES quartile had a significantly higher mortality risk (hazard ratios, 3.0 [95% CI, 1.2-7.8] and 4.3 [95% CI, 1.4-13.9], respectively). In the adjusted survival model, only advanced disease was predictive of mortality (P < .001). CONCLUSION: Hispanics and young children with melanoma are more likely to present with advanced disease, and advanced disease is the single most important predictor of survival. Heightened awareness among physicians is needed to facilitate early detection of melanoma within these groups.

Surgical management for Hirschsprung disease: A review for primary care providers.

Primary care providers may encounter infants and children with Hirschsprung disease, a congenital colonic defect. Although primarily a surgical problem, the disease requires extensive supportive care and a multidisciplinary approach that often extends beyond surgical correction. This article reviews the management of Hirschsprung disease.

Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.

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.