Changing Landscape of Routine Pediatric Surgery for Rural and Urban Children: A Report From the Child Health Evaluation of Surgical Services (CHESS) Group.

OBJECTIVE: To describe the changes to routine pediatric surgical care over the past 2 decades for children living in urban and rural environments. BACKGROUND: A knowledge gaps exists regarding trends in the location where routine pediatric surgical care is provided to children from urban and rural environments over time. METHODS: Children (age 0-18) undergoing 7 common surgeries were identified using State Inpatient Databases (SID, 2002-2017). Rural-Urban Commuting Area codes were used to classify patient and hospital zip codes. Multivariable regression models for distance traveled >60 miles and transfer status were used to compare rural and urban populations, adjusting for year, age, sex, race, and insurance status. RESULTS: Among 143,467 children, 13% lived in rural zip codes. The distance traveled for care increased for both rural and urban children for all procedures but significantly more for the rural cohort (eg, 102% vs 30%, P <0.001, cholecystectomy). Transfers also increased for rural children (eg, transfers for appendectomy increased from 1% in 2002 to 23% in 2017, P <0.001). Factors associated with the need to travel >60 miles included year [adjusted odds ratio (aOR)=2.18, 95% CI: 1.94-2.46: 2017 vs 2002], rural residence (aOR=6.55, 95% CI: 6.11-7.01), age less than 5 years (aOR=2.17, 95% CI: 1.92-2.46), and Medicaid insurance (aOR=1.35, 95% CI: 1.26-1.45). Factors associated with transfer included year (aOR=5.77, 95% CI: 5.26-6.33: 2017 vs 2002), rural residence (aOR=1.47, 95% CI: 1.39-1.56), age less than 10 years (aOR=2.34, 95% CI: 2.15-2.54), and Medicaid insurance (aOR=1.49, 95% CI: 1.42-1.46). CONCLUSION: Rural children, younger age, and those on Medicaid disproportionately traveled greater distances and were more frequently transferred for common pediatric surgical procedures.

Leveraging the Incidence, Burden, and Fiscal Implications of Unplanned Hospital Revisits for the Prioritization of Prevention Efforts in Pediatric Surgery.

OBJECTIVE: To characterize procedure-level burden of revisit-associated resource utilization in pediatric surgery with the goal of establishing a prioritization framework for prevention efforts. SUMMARY OF BACKGROUND DATA: Unplanned hospital revisits are costly to the health care system and associated with lost productivity on behalf of patients and their families. Limited objective data exist to guide the prioritization of prevention efforts within pediatric surgery. METHODS: Using the Pediatric Health Information System (PHIS) database, 30-day unplanned revisits for the 30 most commonly performed pediatric surgical procedures were reviewed from 47 children's hospitals between January 1, 2012 and March 31, 2015. The relative contribution of each procedure to the cumulative burden of revisit-associated length of stay and cost from all procedures was calculated as an estimate of public health relevance if prevention efforts were successfully applied (higher relative contribution = greater potential public health relevance). RESULTS: 159,675 index encounters were analyzed with an aggregate 30-day revisit rate of 10.8%. Four procedures contributed more than half of the revisit-associated length of stay burden from all procedures, with the highest relative contributions attributable to complicated appendicitis (18.4%), gastrostomy (13.4%), uncomplicated appendicitis (13.0%), and fundoplication (9.4%). Four procedures contributed more than half of the revisit-associated cost burden from all procedures, with the highest relative contributions attributable to complicated appendicitis (18.8%), gastrostomy (14.6%), fundoplication (10.4%), and uncomplicated appendicitis (10.2%). CONCLUSIONS AND RELEVANCE: A small number of procedures account for a disproportionate burden of revisit-associated resource utilization in pediatric surgery. Gastrostomy, fundoplication, and appendectomy should be considered high-priority targets for prevention efforts within pediatric surgery.

Parent Perspectives on Readiness for Discharge Home after Neonatal Intensive Care Unit Admission.

OBJECTIVE: To explore the parent perspective on discharge home from the neonatal intensive care unit (NICU). STUDY DESIGN: We interviewed parents of NICU graduates with a range of demographic characteristics and medical complexities to explore parent perspectives on readiness for discharge. Interviews were transcribed and coded by a 6-member team. We performed content analysis to identify themes and develop a family-centered conceptual framework around readiness for NICU discharge. RESULTS: We interviewed a total of 15 parents who experienced NICU stays with 18 infants. Parents who have experienced NICU discharge have a spectrum of needs that evolve from the time the child is in the NICU, at time of discharge, and at home afterward. These needs consistently centered around 5 themes-communication, parent role clarity, emotional support, knowledge sources, and financial resources. CONCLUSIONS: Parents described many ways the system could have better prepared them and connected them with essential resources. Summarizing the voices of the parents who participated in this study, we have compiled a series of practical recommendations for clinicians to use in daily practice to help parents feel prepared and confident for the transition home from the NICU.

Disparities in fertility-sparing surgery in adolescent and young women with stage I ovarian dysgerminoma.

BACKGROUND: In many cancers, racial and socioeconomic disparities exist regarding the extent of surgery. For ovarian dysgerminoma, fertility-sparing (FS) surgery is recommended whenever possible. The aim of this study was to investigate rates of FS versus non-fertility-sparing (NFS) procedures for stage I ovarian dysgerminoma in adolescents and young adults (AYAs) by ethnicity/race and socioeconomic status. MATERIALS AND METHODS: The National Cancer Data Base was queried for patients with ovarian dysgerminoma from 1998 to 2012. After selecting patients aged 15-39 y with stage I disease, a multivariate regression analysis was performed, and rates of FS and NFS procedures were compared, first according to ethnicity/race, and then by socioeconomic surrogate variables. RESULTS: Among the 687 AYAs with stage I ovarian dysgerminoma, there was no significant difference in rates of FS and NFS procedures based on ethnicity/race alone (P = 0.17), but there was a significant difference in procedure type for all three socioeconomic surrogates. The uninsured had higher NFS rates (30%) than those with government (21%) or private (19%) insurance (P = 0.036). Those in the poorest ZIP codes had almost twice the rate of NFS procedures (31%) compared with those in the most affluent ZIP codes (17%). For those in the least-educated regions, 24% underwent NFS procedures compared to 14% in the most-educated areas (P = 0.027). CONCLUSIONS: AYAs with stage I ovarian dysgerminoma in lower socioeconomic groups were more likely to undergo NFS procedures than those in higher socioeconomic groups, but there was no difference in rates of FS versus NFS procedures by ethnicity/race. Approaches aimed at reducing socioeconomic disparities require further examination.

Socioeconomic disparities affect survival in malignant ovarian germ cell tumors in AYA population.

BACKGROUND: Malignant ovarian germ cell tumors (MOGCTs) are a rare form of ovarian malignancy. Socioeconomic status (SES) has been shown to affect survival in several gynecologic cancers. We examined whether SES impacted survival in adolescent and young adults (AYAs) with MOGCT. MATERIALS AND METHODS: The National Cancer Data Base was used to identify AYAs (aged 15-39 years) with MOGCT from 1998 to 2012. Three SES surrogate variables identified were as follows: insurance type, income quartile, and education quartile. Pooled variance t-tests and chi-square tests were used to compare tumor characteristics, the time from diagnosis to staging/treatment, and clinical outcome variables for each SES surrogate variable, while controlling for age and race/ethnicity in a multivariate model. Kaplan-Meier survival estimates were calculated using the log-rank test. RESULTS: A total of 3125 AYAs with MOGCT were identified. Subjects with lower SES measures had higher overall stage and T-stage MOGCTs at presentation. There was no significant difference in the time to staging/treatment, extent of surgery, or use of chemotherapy by SES. Subjects from a lower education background, from a lower income quartile, and without insurance had decreased survival (P ≤ 0.02 for all). Controlling for overall stage and T-stage, the difference in survival was no longer significant. CONCLUSIONS: AYAs with MOGCT from lower SES backgrounds presented with more advanced stage disease. Further studies that focus on the underlying reasons for this difference are needed to address these disparities.

Breast Malignancies in Children: Presentation, Management, and Survival.

PURPOSE: Pediatric breast malignancies are rare, and descriptions in the literature are limited. The purpose of our study was to compare pediatric and adult breast malignancy. METHODS: We performed a retrospective cohort study using the National Cancer Data Base comparing patients ≤21 years to those >21 years at diagnosis (1998-2012). Generalized linear models estimated differences in demographic, tumor, and treatment characteristics. Cox regression was used to compare overall survival. RESULTS: Of 1,999,181 cases of invasive breast malignancies, 477 (0.02%) occurred in patients ≤21 years. Ninety-nine percent of adult patients had invasive carcinoma compared with 64.8% of pediatric patients with the remaining patients having sarcoma, malignant phyllodes, or malignancy not otherwise specified (p < 0.001). Pediatric patients were twice as likely to have an undifferentiated malignancy [relative risk (RR) 2.19; 95% confidence interval (CI) 1.72-3.79]. Half of adults presented with Stage I disease compared with only 22.7% of pediatric patients (p < 0.001). Pediatric patients were 40% more likely to have positive axillary nodes (RR 1.42; 95% CI 1.10-1.84). Among patients with invasive carcinoma, pediatric patients were more than four times as likely to receive a bilateral than a unilateral mastectomy compared with adults (RR 4.56; 95% CI 3.19-6.53). There was no difference in overall survival between children and adults. CONCLUSIONS: Pediatric breast malignancies are more advanced at presentation, and there is variability in treatment practices. Adult and pediatric patients with invasive carcinoma have similar overall survival.

Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database.

OBJECTIVE: To examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi-institutional database. SUMMARY BACKGROUND DATA: UESL is a rare disease (incidence is one per million). Therefore, the current literature is mostly limited to small case series. METHODS: The National Cancer Database was queried for primary UESL diagnosed between 1998 and 2012. RESULTS: A total of 103 patients (<18 years) were identified. The 5-year overall survival of the entire group was 86%. The best outcomes were seen in children who had tumors smaller than 15 cm and were able to undergo surgical resection with or without chemotherapy. Margin status did not appear to significantly affect survival. The most common type of resection was hemihepatectomy (37%), followed by sectionectomy (10%) and trisectionectomy (10%). Orthotopic liver transplant was performed in 10 children, all of whom survived to 5 years. CONCLUSION: Surgical resection with or without chemotherapy should be the mainstay of treatment in children with UESL, and is associated with very favorable outcomes. Negative surgical margins were not associated with improved survival. Orthotopic liver transplantation may be a viable method of attaining local control in tumors, which would otherwise be unresectable.

Case Volume and Revisits in Children Undergoing Gastrostomy Tube Placement.

OBJECTIVES: Emergency department (ED) visits and hospital readmissions are common after gastrostomy tube (GT) placement in children. We sought to characterize interhospital variation in revisit rates and explore the association between this outcome and hospital-specific GT case volume. PATIENTS AND METHODS: We conducted a retrospective cohort study from 38 hospitals using the Pediatric Health Information System database. Patients younger than 18 years who had a GT placed in 2010 to 2012 were assessed for a GT-related (mechanical or infectious) ED visit or inpatient readmission at 30 and 90 days after discharge from GT placement. Risk-adjusted rates were calculated using generalized linear mixed-effects models accounting for hospital clustering and relevant demographic and clinical attributes, then compared across hospitals. RESULTS: A total of 15,642 patients were included. A median of 468 GTs were placed in all the 38 hospitals during 3 years (range: 83-891), with a median of 11.4 GT placed per 1000 discharges (range: 2.4-16.7). Median ED visit for each hospital at 30 days after discharge was 8.2% (range: 3.7%-17.2%) and 14.8% at 90 days (range: 6.3%-26.1%). Median inpatient readmissions for each hospital at 30 days after discharge was 3.5% (range: 0.5%-10.5%) and 5.9% at 90 days (range: 1.0%-18.5%). Hospital-specific GT placement per 1000 discharges (rate of GT placement) was inversely correlated with ED visit rates at 30 (P = 0.007) and 90 days (P = 0.020). The adjusted 30- and 90-day readmission rate and the adjusted 30- and 90-day ED return rates decreased with increasing GT insertion rate (P < 0.001). CONCLUSION: Higher hospital GT insertion rates are associated with lower ED revisit rates but not inpatient readmissions.

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.

Regionalization of Pediatric Surgery: Trends Already Underway.

INTRODUCTION: This study aims to characterize the delivery of pediatric surgical care based on hospital volume stratified by disease severity, geography, and specialty. Longitudinal regionalization over the 10-year study period is noted and further explored. METHODS: The Kids' Inpatient Database (KID) was queried from 2000 to 2009 for patients <18 years undergoing noncardiac surgery. Hospitals nationwide were grouped into commutable regions and identified as high-volume centers (HVCs) if they had more than 1000 weighted procedures per year. Regions that had at least one HVC and one or more additional lower volume center were included for analysis. Low-risk, high-risk neonatal, and surgical subspecialties were analyzed separately. RESULTS: A total of 385,242 weighted pediatric surgical admissions in 33 geographical regions and 224 hospitals were analyzed. Overall, HVCs comprised 33 (14.7%) hospitals, medium-volume center (MVC) 33 (14.7%), and low-volume center (LVC) 158 (70.5%). The four low-risk procedures analyzed were increasingly regionalized: appendectomy (52% in HVCs in 2000 to 60% in 2009, P < 0.001), fracture reduction (63% to 68%, P < 0.001), cholecystectomy (54% to 63%, P < 0.001), and pyloromyotomy (65% to 85%, P < 0.001). Neonatal surgery showed significant regionalization trends for tracheoesophageal fistula (66% to 87%, P < 0.001) and gastroschisis (76% to 89%, P < 0.001). CONCLUSIONS: This is the first large-scale, multi-region analysis to demonstrate that pediatric surgical care has transitioned to HVCs over a recent decade, particularly for low-risk patients. It is important for practitioners and policymakers alike to understand such volume trends in order to ensure hospital capacity while maintaining an optimal quality of care.

Emergency Department Visits and Readmissions among Children after Gastrostomy Tube Placement.

OBJECTIVES: To define the incidence of 30-day postdischarge emergency department (ED) visits and hospital readmissions following pediatric gastrostomy tube (GT) placement across all procedural services (Surgery, Interventional-Radiology, Gastroenterology) in 38 freestanding Children's Hospitals. STUDY DESIGN: This retrospective cohort study evaluated patients <18 years of age discharged between 2010 and 2012 after GT placement. Factors significantly associated with ED revisits and hospital readmissions within 30 days of hospital discharge were identified using multivariable logistic regression. A subgroup analysis was performed comparing patients having the GT placed on the date of admission or later in the hospital course. RESULTS: Of 15 642 identified patients, 8.6% had an ED visit within 30 days of hospital discharge, and 3.9% were readmitted through the ED with a GT-related issue. GT-related events associated with these visits included infection (27%), mechanical complication (22%), and replacement (19%). In multivariable analysis, Hispanic ethnicity, non-Hispanic black race, and the presence of ≥3 chronic conditions were independently associated with ED revisits; gastroesophageal reflux and not having a concomitant fundoplication at time of GT placement were independently associated with hospital readmission. Timing of GT placement (scheduled vs late) was not associated with either ED revisits or hospital readmission. CONCLUSIONS: GT placement is associated with high rates of ED revisits and hospital readmissions in the first 30 days after hospital discharge. The association of nonmodifiable risk factors such as race/ethnicity and medical complexity is an initial step toward understanding this population so that interventions can be developed to decrease these potentially preventable occurrences given their importance among accountable care organizations.

Ultrasound-guided rectus sheath block or wound infiltration in children: a randomized blinded study of analgesia and bupivacaine absorption.

BACKGROUND: Rectus sheath block can provide analgesia following umbilical hernia repair. However, conflicting reports on its analgesic effectiveness exist. No study has investigated plasma local anesthetic concentration following ultrasound-guided rectus sheath block (USGRSB) in children. OBJECTIVES: Compare the effectiveness and bupivacaine absorption following USGRSB or wound infiltration (WI) for umbilical hernia repair in children. METHODS: A randomized blinded study comparing WI with USGRSB in 40 children undergoing umbilical hernia repair was performed. Group WI (n = 20) received wound infiltration 1 mg·kg(-1) 0.25% bupivacaine. Group RS (n = 20) received USGRSB 0.5 mg·kg(-1) 0.25% bupivacaine per side in the posterior rectus sheath compartment. Pain scores and rescue analgesia were recorded. Blood samples were drawn at 0, 10, 20, 30, 45, and 60 min. RESULTS: Patients in the WI group had a twofold increased risk of requiring morphine (hazard ratio 2.06, 95% CI 1.01, 4.20, P = 0.05). When required, median time to first morphine dose was longer in the USGRSB group (65.5 min vs. 47.5 min, P = 0.049). Peak plasma bupivacaine concentration was higher following USGRSB than WI (median: 631.9 ng·ml(-1) IQR: 553.9-784.1 vs. 389.7 ng·ml(-1) IQR: 250.5-502.7, P = 0.002). Tmax was longer in the USGRSB group (median 45 min IQR: 30-60 vs. 20 min IQR: 20-45, P = 0.006). CONCLUSIONS: USGRSB provides more effective analgesia than WI for umbilical hernia repair. USGRSB with 1 mg·kg(-1) 0.25% bupivacaine is associated with safe plasma bupivacaine concentration that peaks higher and later than WI. Caution against using larger volumes of higher concentration local anesthetic for USGRSB is advised.

Interfacility Transfer of Children With Time-Sensitive Surgical Conditions, 2002-2017.

Importance: Surgical care for children in the United States has become increasingly regionalized among fewer centers over time. The degree to which regionalization may be associated with access to urgent surgical care for time-sensitive conditions is not clear. Objective: To investigate whether transfers and travel distance have increased for 4 surgical conditions, and whether changes in transfers and travel distance have been more pronounced for rural vs urban children. Design, Setting, and Participants: This retrospective cross-sectional study analyzed data from 9 State Inpatient Databases from 2002 to 2017. Participants included children aged younger than 18 years undergoing urgent or emergent procedures for malrotation with volvulus, esophageal foreign body, and ovarian and testicular torsion. Exposure: Residential and hospital zip codes were categorized as rural or urban. Hospitals were categorized as pediatric hospitals, adult hospitals with pediatric services, and adult hospitals without pediatric services. Main Outcomes and Measures: Primary outcomes were transfer for care and travel distance between patients' home residences and the hospitals where care was provided. Transfer and travel distance were analyzed using multivariable regression models. Results: Among the 5865 children younger than 18 years undergoing procedures for malrotation with volvulus, esophageal foreign body, ovarian torsion, or testicular torsion, 461 (7.9%) resided in a rural area; 1097 (20.5%) were Hispanic, 1334 (24.9%) were non-Hispanic Black, and 2255 (42.0%) were non-Hispanic White; 2763 (47.1%) were covered by private insurance and 2535 (43.2%) were covered by Medicaid; and the median (IQR) age was 9 (2-14) years. Most care was provided at adult hospitals (73.4% with and 16.9% without pediatric services); the number of hospitals providing this care decreased from 493 to 292 hospitals (2002 vs 2017). Transfer was associated with rural residence (adjusted odds ratio [aRR], 2.3 [95% CI, 1.8-3.0]; P < .001) and increased over time (2017 vs 2002: aOR, 2.8 [95% CI, 2.0-3.8]; P < .001). Similarly, travel distance was associated with rural residence (adjusted risk ratio [aRR], 4.4 [95% CI, 3.9-4.8]; P < .001) and increased over time (2017 vs 2002: aRR, 1.3 [95% CI, 1.2-1.4]; P < .001). Rural children were more frequently transferred (2017 vs 2002) for esophageal foreign body (48.0% [12 of 25] vs 7.3% [4 of 55]; P < .001), ovarian torsion (26.7% [4 of 15] vs 0% [0 of 18]; P = .01), and testicular torsion (18.2% [2 of 11] vs 0% [0 of 16]; P = .04). Travel distance for rural children increased the most for torsions, from a median (IQR) of 19.1 (2.3-35.4) to 43.0 (21.6-98.8) miles (P = .03) for ovarian torsion and from 7.3 (0.4-23.7) to 44.5 (33.1-48.8) miles (P < .001) for testicular torsion. Conclusions and Relevance: In this cross-sectional study of children with time-sensitive surgical conditions, the number of hospitals providing urgent surgical care to children decreased over time. Transfers of care, especially among rural children, and travel distance, especially for those with ovarian and testicular torsion, increased over time.

Classification and Surgical Management of Anorectal Malformations: A Systematic Review and Evidence-based Guideline From the APSA Outcomes and Evidence-based Practice Committee.

OBJECTIVE: Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs. These questions were related to categorization of ARMs and optimal methods and timing of surgical management. A comprehensive search strategy was performed, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to perform the systematic review to attempt to answer five questions related to surgical care of ARM. RESULTS: A total of 10,843 publications were reviewed, of which 90 were included in final recommendations, and some publications addressed more than one question (question: 1 n = 6, 2 n = 63, n = 15, 4 n = 44). Studies contained largely heterogenous groups of ARMs, making direct comparison for each subtype challenging and therefore, no specific recommendation for optimal surgical approach based on outcomes can be made. Both loop and divided colostomy may be acceptable methods of fecal diversion for patients with a diagnosis of anorectal malformation, however, loop colostomies have higher rates of prolapse in the literature reviewed. In terms of timing of repair, there did not appear to be significant differences in outcomes between early and late repair groups. Clear and uniform definitions are needed in order to ensure similar populations of patients are compared moving forward. Recommendations are provided based primarily on A-D levels of evidence. CONCLUSIONS: Evidence-based best practices for ARMs are lacking for many aspects of care. Multi-institutional registries have made progress to address some of these gaps. Further prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

Surgical treatment of primary liver tumors in children: outcomes analysis of resection and transplantation in the SEER database.

Adjusted survival outcomes following hepatic resection and transplantation for pediatric liver tumors have not been compared. To address this question, we conducted a retrospective cohort study using the SEER registry. While SEER lacks certain specifics regarding staging, chemotherapy, comorbidities, and recurrence, important hypothesis-generating data are available and were analyzed using Kaplan-Meier statistics and Cox proportional hazards regression. All SEER patients under the age of 20 yr undergoing surgery for HB (n = 318) or HCC (n = 80) between 1998 and 2009 were included. Of HB patients, 83.3% underwent resection and 16.7% transplantation. Advanced disease, vascular invasion, and satellite lesions were more common among transplant patients. Unadjusted five-yr survival was equivalent, as was the adjusted hazard of death for transplant relative to resection (HR = 0.58, p = 0.63). Of HCC patients, 75.0% underwent resection and 25.0% transplantation. Transplant patients had a higher prevalence of vascular invasion and satellite lesions. Five-yr survival was 53.4% after resection and 85.3% after transplant, and the adjusted hazard of death was significantly lower after transplantation (HR = 0.05, p = 0.045). While transplantation is generally reserved for unresectable tumors, the favorable survival seen in HCC patients suggests that liberalized transplant criteria might improve survival, although further prospective data are needed.

Health-related quality of life in children with intestinal failure.

OBJECTIVES: Survival of infants with intestinal failure (IF) has increased in the past decade; however, data on their health-related quality of life (HRQOL) are lacking. We hypothesized that HRQOL would be lower among children with IF compared with that of healthy children. METHODS: We performed a cross-sectional study of the HRQOL of children enrolled in the outpatient intestinal rehabilitation program at Seattle Children's Hospital using the PedsQL 4.0 Generic Core Scales parent proxy-report and the Family Impact Module questionnaires. Parents were asked 2 open-ended questions pertaining to the suitability and completeness of the PedsQL to assess their and their child's HRQOL. RESULTS: Parents of 23 children with IF completed the questionnaires. Compared with norms for healthy children, parents reported significantly lower total PedsQL scores for children ages 1 to 2 years (mean difference -13.16, 95% confidence interval [CI] -21.86 to -4.46; P = 0.003) and 2 to 6 years (mean difference -15.57, 95% CI -22.66 to -8.48; P < 0.001). Scores were also lower for children younger than 1 year (mean difference -6.43, 95% CI -13.93 to 1.07), although this test was not statistically significant. No measured demographic or clinical characteristics were associated with HRQOL. The majority of parents (65%) said the PedsQL failed to address important effects of IF on children and their families. CONCLUSIONS: Children with IF and their parents have a decreased HRQOL compared with healthy children as measured by the PedsQL survey. A disease-specific module or separate HRQOL questionnaire is needed for a more comprehensive assessment of HRQOL in children with IF.

Total colectomy for ulcerative colitis in children: when are we operating?

PURPOSE: Ulcerative colitis (UC) in children is frequently severe and treatment-refractory. While medical therapy is well standardized, little is known regarding factors that contribute to surgical indications. Our aim was to identify factors associated with progression to colectomy in a large cohort of pediatric UC patients. METHODS: We conducted a retrospective cohort study using the Pediatric Health Information System database. We identified all patients under age 18 discharged between January 1, 2004 and September 30, 2011 with a primary diagnosis of UC. Primary outcome was odds of total colectomy. RESULTS: Of 8,688 patients, 240 (2.8 %) underwent colectomy. Compared with non-operative patients, a greater proportion of colectomy patients received advanced therapies during admission, including corticosteroids (84.2 vs. 71.3 %) and biological therapy (25.4 vs. 13.6 %). Odds of colectomy were increased with malnutrition (OR 1.86), anemia (OR 2.17), electrolyte imbalance (OR 2.31), and Clostridium difficile infection (OR 1.69). TPN requirement also independently predicted colectomy (OR 3.86). Each successive UC admission significantly increased the odds of colectomy (OR 1.08). CONCLUSION: These data identify factors associated with progression to colectomy in children hospitalized with UC. Our findings help to identify factors that should be incorporated into future studies aiming to reduce the variability in surgical treatment of childhood UC.

Outcomes in pediatric surgery by hospital volume: a population-based comparison.

PURPOSE: The volume-outcome relationship has not been well-defined in pediatric surgery. Our aim was to determine the association between hospital-volume and outcomes for common procedures in children. METHODS: Retrospective population-based cohort study of patients <18 years of age hospitalized between 1989 and 2009 for common surgical procedures in Washington State. The association between annual hospital case volume and post-operative outcomes (readmission and reoperation within 30-days, post-operative complications) was assessed using multivariate logistic regression. RESULTS: The three most common procedures over the study period were appendectomy (n = 36,525), skin and soft tissue debridement (n = 9,813), and pyloromyotomy (n = 3,323). A greater proportion of patients with comorbidities were treated at higher-volume hospitals. After adjustment, outcomes did not differ significantly across hospital-volume quartiles except that debridement patients had lower odds of readmission (OR = 0.63, 95 % CI 0.46-0.88) and re-operation (OR = 0.53, 95 % CI 0.35-0.81) at medium-high-volume compared with high-volume centers. CONCLUSIONS: This work suggests that risks of readmission and post-operative complications for common procedures may be similar across hospital-volume categories, but appropriate risk-stratification is essential. In order to optimize safety, we must identify the resources required for low-, medium-, and high-risk surgical patients, and implement these standards into practice.

Extensive congenital abdominal aortic aneurysm and renovascular disease in the neonate.

Primary congenital abdominal aortic aneurysm is an extremely rare entity, with only 15 patients reported in the literature. Options for repair are often limited secondary to branch vessel size and other anatomic limitations. We present a neonate diagnosed with an abdominal aortic aneurysm on prenatal ultrasound. A postpartum computed tomography angiogram revealed an extensive type IV thoracoabdominal aortic aneurysm extending to the aortic bifurcation and resulting in bilateral renal artery stenosis. The unique features of this patient and challenges in management are discussed.

Anesthesia for pheochromocytoma resection in a child with Fontan circulation.

PURPOSE: To report the anesthetic management of a successful resection of a pheochromocytoma in a child with a completed Fontan circulation. CLINICAL FEATURES: The patient was an 11-yr-old boy with Ivemark syndrome who had undergone Fontan palliation at three years of age. Six weeks earlier, he had been diagnosed with a norepinephrine-producing pheochromocytoma, and he had been pretreated with oral propranolol and phenoxybenzamine. During surgery, intravenous administration of magnesium sulphate, esmolol, and phentolamine provided good hemodynamic control. Postoperatively, the patient tended to be hypotensive, and treatment with fluid administration resulted in prolonged intensive care. CONCLUSION: Although intraoperative management was not problematic, postoperative care of this 11-yr old child with pheochromocytoma was complicated by residual sympathetic blockade.