Management of the undescended testis in children: An American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee Systematic Review.

PURPOSE: Management of undescended testes (UDT) has evolved over the last decade. While urologic societies in the United States and Europe have established some guidelines for care, management by North American pediatric surgeons remains variable. The aim of this systematic review is to evaluate the published evidence regarding the treatment of (UDT) in children. METHODS: A comprehensive search strategy and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were utilized to identify, review, and report salient articles. Five principal questions were asked regarding imaging standards, medical treatment, surgical technique, timing of operation, and outcomes. A literature search was performed from 2005 to 2020. RESULTS: A total of 825 articles were identified in the initial search, and 260 were included in the final review. CONCLUSIONS: Pre-operative imaging and hormonal therapy are generally not recommended except in specific circumstances. Testicular growth and potential for fertility improves when orchiopexy is performed before one year of age. For a palpable testis, a single incision approach is preferred over a two-incision orchiopexy. Laparoscopic orchiopexy is associated with a slightly lower testicular atrophy rate but a higher rate of long-term testicular retraction. One and two-stage Fowler-Stephens orchiopexy have similar rates of testicular atrophy and retraction. There is a higher relative risk of testicular cancer in UDT which may be lessened by pre-pubertal orchiopexy.

Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

The effects of early anesthesia on neurodevelopment: A systematic review.

BACKGROUND: There is growing concern regarding the impact of general anesthesia on neurodevelopment in children. Pre-clinical animal studies have linked anesthetic exposure to abnormal central nervous system development, but it is unclear whether these results translate into humans. The purpose of this systematic review from the American Pediatric Surgical Association (APSA) Outcomes and Evidence-Based Practice (OEBP) Committee was to review, summarize, and evaluate the evidence regarding the neurodevelopmental impact of general anesthesia on children and identify factors that may affect the risk of neurotoxicity. METHODS: Medline, Cochrane, Embase, Web of Science, and Scopus databases were queried for articles published up to and including December 2017 using the search terms "general anesthesia and neurodevelopment" as well as specific anesthetic agents. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to screen manuscripts for inclusion in the review. A consensus statement of recommendations in response to each study question was synthesized based upon the best available evidence. RESULTS: In total, 493 titles were initially identified, with 56 articles selected for full analysis and 44 included for review. Based on currently available developmental assessment tools, a single exposure to general anesthesia does not appear to have a significant effect on general neurodevelopment, although prolonged or multiple anesthetic exposures may have some adverse effects. Exposure to general anesthesia may affect different domains of development at different ages. Regional anesthetic techniques with the addition of dexmedetomidine and/or some intravenous agents may mitigate the risks of neurotoxicity. This approach may be performed safely in some patients and can be considered as an option in selected short procedures. CONCLUSION: There is no conclusive evidence that a single short anesthetic in infancy has a detectable neurodevelopmental effect. Data do not support waiting until later in childhood to perform general anesthesia for single short procedures. With the complexities and nuances of different anesthetic methods, patients and procedures, the planning and execution of anesthesia for the pediatric patient is generally best accomplished by an anesthesiologist, ideally a pediatric anesthesiologist. TYPE OF STUDY: Systematic review of level 1-4 studies. LEVEL OF EVIDENCE: Level 1-4 (mainly level 3-4).

Management of intussusception 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 develop recommendations for the management of ileocolic intussusception in children. METHODS: The ClinicalTrials.gov, Embase, PubMed, and Scopus databases were queried for literature from January 1988 through December 2018. Search terms were designed to address the following topics in intussusception: prophylactic antibiotic use, repeated enema reductions, outpatient management, and use of minimally invasive techniques for children with intussusception. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Consensus recommendations were derived based on the best available evidence. RESULTS: A total of 83 articles were analyzed and included for review. Prophylactic antibiotic use does not decrease complications after radiologic reduction. Repeated enema reductions may be attempted when clinically appropriate. Patients can be safely observed in the emergency department following enema reduction of ileocolic intussusception, avoiding hospital admission. Laparoscopic reduction is often successful. CONCLUSIONS: Regarding intussusception in hemodynamically stable children without critical illness, pre-reduction antibiotics are unnecessary, non-operative outpatient management should be maximized, and minimally invasive techniques may be used to avoid laparotomy. LEVEL OF EVIDENCE: Level 3-5 (mainly level 3-4) TYPE OF STUDY: Systematic Review of level 1-4 studies.

Accreditation Council for Graduate Medical Education (ACGME) Surgery Resident Operative Logs: The Last Quarter Century.

STUDY OBJECTIVE: To describe secular trends in operative experience for surgical trainees across an extended period using the most comprehensive data available, the Accreditation Council for Graduate Medical Education (ACGME) case logs. BACKGROUND: Some experts have expressed concern that current trainees are inadequately prepared for independent practice. One frequently mentioned factor is whether duty hours' restrictions (DHR) implemented in 2003 and 2004 contributed by reducing time spent in the operating room. METHODS: A dataset was generated from annual ACGME reports. Operative volume for total major cases (TMC), defined categories, and four index laparoscopic procedures was evaluated. RESULTS: TMC dropped after implementation of DHR but rebounded after a transition period (949 vs 946 cases, P = nonsignificance). Abdominal cases increased from 22% of overall cases to 31%. Alimentary cases increased from 21% to 26%. Trauma and vascular surgery substantially decreased. For trauma, this drop took place well before DHR. The decrease in vascular surgery also began before DHR but continued afterward as well: 148 cases/resident in the late 1990s to 107 currently. CONCLUSIONS: Although total operative volume rebounded after implementation of DHR, diversity of operative experienced narrowed. The combined increase in alimentary and abdominal cases is nearly 13%, over a half-year's worth of operating in 5-year training programs. Bedrock general surgery cases-trauma, vascular, pediatrics, and breast-decreased. Laparoscopic operations have steadily increased. If the competence of current graduates has, in fact, diminished. Our analysis suggests that operative volume is not the problem. Rather, changing disease processes, subspecialization, reductions in resident autonomy, and technical innovation challenge how today's general surgeons are trained.

Establishing Equipoise: National Survey of the Treatment of Pediatric Para-Pneumonic Effusion and Empyema.

BACKGROUND: Despite six randomized trials of various treatments for pediatric para-pneumonic effusion (PPE), management approaches differ. The purpose of this study was to gain insight into opinions on PPE treatment with the goal of designing a definitive trial to generate consensus intervention guidelines. METHODS: To evaluate physician opinions regarding PPE management, we developed a survey based on input from a nationwide, multi-disciplinary advisory group that established content validity. The survey was disseminated broadly to six pediatric medicine and interventional radiology groups. Descriptive and χ2 statistics were calculated. RESULTS: There were 741 respondents (response rate 13.1%), of whom 52.2% were surgeons, 15.2% hospitalists, 14.2% pulmonologists, 12.4% intensivists, and 6.0% interventional radiologists. Nearly all respondents (97.3%) reported caring primarily for pediatric patients. Eighty percent reported no written institutional treatment guidelines. Nearly all (90.3%) agreed that patients require antibiotics, but there was disagreement regarding their duration. Respondents also were split as to how often PPE required drainage. There were multiple absolute indications for drainage, including mediastinal shift on chest radiograph (67.2%) and loculations on imaging (47.7%). There were substantial differences in the preferred first-line methods of drainage based on the treating physician's specialty, with surgeons preferring tube thoracostomy and a fibrinolytic agent (42.0%) or video-assisted thoracoscopic surgery (41.6%), whereas interventional radiologists preferred either a tube thoracostomy (46.4%) or a tube thoracostomy with a fibrinolytic agent (39.3%) (p < 0.001). A large majority (75.3%) believed that the published evidence does not identify the optimal intervention. CONCLUSIONS: There is a lack of consensus regarding the optimal treatment of PPE. Respondents believed the published evidence is inconclusive and were willing to participate in a prospective trial. These findings will help inform the design of a randomized, pragmatic clinical trial to optimize PPE management.

Influence of hospital and patient location on early postoperative outcomes after appendectomy and pyloromyotomy.

BACKGROUND: The effects of hospital location and designation on postoperative pediatric outcomes remain unclear. We hypothesized that urban hospital outcomes would be superior to rural hospitals, and that outcomes at urban centers would differ for children from rural versus urban counties. METHODS: Retrospective cohort study of children undergoing appendectomy (n=129,507) and pyloromyotomy (n=13,452) using the 2006/2009 KID databases. Hospitals were characterized by specialty designation and classified as urban/rural. County of residence was classified as urban/rural. Outcomes included complications and length of stay. Multivariate regression models were used to adjust for confounding. RESULTS: Among appendectomy patients, treatment at urban hospitals was associated with reduced odds of any postoperative complication (OR=0.77, 95% C.I. 0.70-0.85) and anesthesia-related complications (OR=0.72, 95% C.I. 0.57-0.91). This association was strongest in the youngest children (<5 years) and at children's hospitals. For pyloromyotomy patients, urban hospitals were associated with reduced odds of any complication (OR=0.43, 95% C.I. 0.24-0.75), anesthesia-related complications (OR=0.14, 95% C.I. 0.05-0.37), and duodenal perforation (OR=0.46, 95% C.I. 0.19-1.07). These associations were most significant at children's hospitals. CONCLUSIONS: Postoperative outcomes appear to be improved at urban specialty hospitals relative to rural hospitals for certain common pediatric procedures. Identification of the factors driving this association may help inform resource optimization efforts in pediatric surgery.

A national review of the frequency of minimally invasive surgery among general surgery residents: assessment of ACGME case logs during 2 decades of general surgery resident training.

IMPORTANCE: Minimally invasive surgery (MIS) has created a shift in how many surgical diseases are treated. Examining the effect on resident operative experience provides valuable insight into trends that may be useful for restructuring the requirements of resident training. OBJECTIVE: To evaluate changes in general surgery resident operative experience regarding MIS. DESIGN, SETTING, AND PARTICIPANTS: Retrospective review of the frequency of MIS relative to open operations among general surgery residents using the Accreditation Council for Graduate Medical Education case logs for academic years 1993-1994 through 2011-2012. EXPOSURES: General surgery residency training among accredited programs in the United States. MAIN OUTCOMES AND MEASURES: We analyzed the difference in the mean number of MIS techniques and corresponding open procedures across training periods using 2-tailed t tests with statistical significance set at P < .05. RESULTS: Of 6,467,708 operations with the option of MIS, 2,393,030 (37.0%) were performed with the MIS approach. Of all MIS operations performed, the 5 most common were cholecystectomy (48.5%), appendectomy (16.2%), groin hernia repair (10.0%), abdominal exploration (nontrauma) (4.4%), and antireflux procedures (3.6%). During the study period, there was a transition from a predominantly open to MIS approach for appendectomy, antireflux procedures, thoracic wedge resection, and partial gastric resection. Cholecystectomy is the only procedure for which MIS was more common than the open technique throughout the study period (P < .001). The open approach is more common for all other procedures, including splenectomy (0.7% MIS), common bile duct exploration (24.9% MIS), gastrostomy (25.9% MIS), abdominal exploration (33.1% MIS), hernia (20.3% MIS), lung resection (22.3% MIS), partial or total colectomy (39.1%), enterolysis (19.0% MIS), ileostomy (9.0% MIS), enterectomy (5.2% MIS), vagotomy (1.8% MIS), and pediatric antireflux procedures (35.9% MIS); P < .001. CONCLUSIONS AND RELEVANCE: Minimally invasive surgery has an increasingly prominent role in contemporary surgical therapy for many common diseases. The open approach, however, still predominates in all but 5 procedures. Residents today must become efficient at performing multiple techniques for a single procedure, which demands a broader skill set than in the past.

Isolated appendiceal typhlitis masquerading as perforated appendicitis in the setting of acute lymphoblastic leukemia.

Abdominal pain is common during chemotherapy for childhood leukemia. Clinically differentiating typhlitis from appendicitis can be difficult. We present an 8-year-old boy with abdominal pain in the setting of acute lymphoblastic leukemia and neutropenia. Following appendectomy for presumed appendicitis, pathology revealed appendiceal typhlitis. Diagnostic and treatment considerations are discussed.

Maternal medical and behavioral risk factors for congenital diaphragmatic hernia.

PURPOSE: Maternal factors contributing to the etiology of congenital diaphragmatic hernia (CDH) remain unclear. We hypothesized that specific maternal medical conditions (pregestational diabetes, hypertension), and behaviors (alcohol, tobacco) would be associated with CDH. METHODS: We conducted a population-based case-control study using Washington State birth certificates linked to hospital discharge records (1987-2009). We identified all infants with CDH (n=492). Controls were randomly selected among non-CDH infants. Maternal data were extracted from the birth record. Logistic regression was used to adjust for covariates. RESULTS: Cases and controls were generally similar regarding demographics, although CDH infants were more likely to be male than controls (58.5% vs. 52.5%). Isolated and complex (multiple-anomaly) CDH had similar characteristics. Each of the exposures of interest was more common among case mothers than among control mothers. In univariate analysis, alcohol use, hypertension, and pregestational diabetes were each significantly associated with the outcome. After multivariate adjustment, only alcohol use (OR=3.65, p=0.01) and pregestational diabetes (OR=12.53, p=0.003) maintained significance. Results were similar for both isolated and complex CDH. CONCLUSIONS: Maternal pregestational diabetes and alcohol use are significantly associated with occurrence of CDH in infants. These are important modifiable risk factors to consider with regard to efforts seeking to impact the incidence of CDH.

Shifts towards pediatric specialists in the treatment of appendicitis and pyloric stenosis: trends and outcomes.

BACKGROUND: Little data exists on temporal changes in the care of children with common surgical conditions. We hypothesized that an increasing proportion of procedures are performed at pediatric hospitals over time, and that outcomes are superior at these centers. METHODS: We conducted a retrospective cohort study using Washington State discharge records for children 0-17years old undergoing appendectomy (n=39,472) or pyloromyotomy (n=3,500). Pediatric hospitals were defined as centers with full-time pediatric surgeons. Outcomes were examined for two time periods (1987-2000, 2001-2009). RESULTS: From 1987 to 2009, the proportion of procedures performed at pediatric hospitals steadily increased. The percentage for appendectomies increased from 17% to 32%, and that for pyloromyotomies increased from 57% to 99%. For pyloromyotomy, care at a pediatric hospital was associated with decreased risk of postoperative complications (OR=0.36, p<0.001) for both time periods. Appendectomy outcomes did not differ significantly in the early time period, but in the later time period specialist care was associated with lower risk of complications in children <5years (OR=0.54, p=0.03). CONCLUSION: There has been a shift towards pediatric hospitals for certain procedures, with a widening disparity in outcomes for younger children. These results suggest that procedures in younger patients may best be performed by providers familiar with these patient populations.

Antireflux procedures for gastroesophageal reflux disease in children: influence of patient age on surgical management.

IMPORTANCE: Gastroesophageal reflux disease (GERD) is a common diagnosis in infants and children, but no objective criteria exist to guide the diagnosis and treatment of this disease in this population. The extent to which age influences decisions about surgical treatment in childhood GERD is unknown. OBJECTIVE: To identify factors associated with progression to antireflux procedures (ARPs) in children hospitalized with GERD. DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study using inpatient data from 41 US children's hospitals in the Pediatric Health Information System database. We included patients younger than 18 years discharged from January 1, 2002, through December 31, 2010, with primary diagnostic codes for GERD (n = 141 190). We evaluated demographics, comorbidities, and diagnostic procedures descriptively and with a multivariate Cox proportional hazards regression model. EXPOSURE: Patient age. MAIN OUTCOMES AND MEASURES: Proportional hazard of progression to ARP during admission. RESULTS: Of the 141,190 patients meeting study criteria, 11,621 (8.2%) underwent ARPs during the study period. More than half of patients undergoing ARPs (52.7%) were 6 months or younger. Although most patients in the ARP group had preoperative upper gastrointestinal tract fluoroscopy (65.0%), these patients did not undergo a uniform workup. The hazard of progression to an ARP was significantly decreased in children aged 7 months to 4 years (hazard ratio, 0.63 [P < .001]) and 5 to 17 years (0.43 [P < .001]) relative to children younger than 2 months. Hazard ratios were independently increased for comorbid diagnoses of failure to thrive, neurodevelopmental delay, cardiopulmonary anomalies, cerebral palsy, and aspiration pneumonia and among patients with tracheoesophageal fistula and diaphragmatic hernia. Each additional GERD-related hospitalization was associated with a 10% increased risk of an ARP. CONCLUSIONS AND REVELANCE: Antireflux procedures are most commonly performed in children during a period of life when regurgitation is normal and physiologic and objective measures of GERD are difficult to interpret. To identify meaningful outcomes after ARP, indications must be clear and standardized. We must clarify the appropriate workup for infants and young children with GERD and better define "failure of medical management" in this population.

Hepatocellular carcinoma in children: epidemiology and the impact of regional lymphadenectomy on surgical outcomes.

BACKGROUND: Factors influencing survival in children with HCC have not been studied. The objective of this study was to identify prognostic factors in pediatric HCC, and to determine whether regional lymphadenectomy is associated with improved survival. METHODS: We performed a retrospective cohort study using the Surveillance, Epidemiology and End Results (SEER) registry. All patients <20 years old diagnosed with HCC from 1973-2009 were included. Disease-specific survival was compared using Kaplan-Meier statistics and Cox proportional-hazards regression. RESULTS: We identified 238 patients (139 Male: 99 Female). Overall, 112 (47%) received an operation (resection/transplantation). Observed mortality and adjusted hazard of disease-specific death was greater for females (HR=2.07, p=0.013) and older children. Among operative patients, 44% were documented to have a regional lymphadenectomy. Although demographic factors did not differ between lymphadenectomy and non-lymphadenectomy groups, patients who underwent lymphadenectomy had a greater proportion of metastatic disease (24% vs. 15%) and fibrolamellar HCC (53% vs. 31%). Five-year survival for lymphadenectomy patients was superior to non-lymphadenectomy (70% vs. 57%). Adjusted mortality for lymphadenectomy was also improved relative to non-lymphadenectomy (HR=0.26, p=0.013). CONCLUSIONS: HCC in children is associated with poor survival, especially among children older than 4 years and girls. In surgical candidates, regional lymphadenectomy may be associated with improved survival.

Role of bottle feeding in the etiology of hypertrophic pyloric stenosis.

IMPORTANCE Bottle feeding has been implicated in the etiology of hypertrophic pyloric stenosis (HPS). Further data are needed to define the nature of this relationship and the clinical variables that influence it. OBJECTIVE To determine if bottle feeding after birth is associated with the development of HPS in infants. We hypothesized that bottle feeding is associated with an increased risk of HPS and that this risk is modified by other risk factors. DESIGN, SETTING, AND PARTICIPANTS Population-based case-control study of births from January 1, 2003, to December 31, 2009, using Washington State birth certificates linked to hospital discharge data. Cases included all singleton infants born within the study period and subsequently admitted with both a diagnostic code for HPS and a procedure code for pyloromyotomy (n = 714). Controls were randomly chosen among singleton infants who did not develop HPS and were frequency matched to cases by birth year. EXPOSURE Feeding status (breast vs bottle) was coded on the birth certificate as the type of feeding the infant was receiving at birth discharge. MAIN OUTCOME AND MEASURE Diagnosis of HPS. RESULTS Hypertrophic pyloric stenosis incidence decreased over time, from 14 per 10,000 births in 2003 to 9 per 10,000 in 2009. Simultaneously, breastfeeding prevalence increased from 80% in 2003 to 94% in 2009. Compared with controls, cases were more likely to be bottle feeding after birth (19.5% vs 9.1%). After adjustment, bottle feeding was associated with an increased risk of HPS (odds ratio [OR], 2.31; 95% CI, 1.81-2.95). This association did not differ according to sex or maternal smoking status but was significantly modified by maternal age (<20 years OR, 0.98; 95% CI, 0.51-1.88; ≥35 years OR, 6.07; 95% CI, 2.81-13.10) and parity (nulliparous OR, 1.60; 95% CI, 1.07-2.38; multiparous OR, 3.42; 95% CI, 2.23-5.24). CONCLUSIONS AND RELEVANCE Bottle feeding is associated with an increased risk of HPS, and this effect seems to be most important in older and multiparous women. These data suggest that bottle feeding may play a role in HPS etiology, and further investigations may help to elucidate the mechanisms underlying the observed effect modification by age and parity.

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.

Predictors of survival in pediatric adrenocortical carcinoma: a Surveillance, Epidemiology, and End Results (SEER) program study.

BACKGROUND: Adrenocortical carcinoma (ACC) is rarely described in children. There is variation in incidence worldwide. This study sought to identify national incidence rates and independent prognostic indicators for children. METHODS: The SEER database was queried for the years 1973 through 2008 for all patients with ACC less than 20 years of age. Incidence rates and survival were analyzed accounting for clinical and demographic factors. Cox proportional-hazards regression was used to identify factors associated with disease-specific survival. RESULTS: Eighty-five patients (57 F: 28 M) were identified. Annual ACC incidence was 0.21 per million. Young patients (≤ 4 years) were noted to have more favorable features than older patients (5-19 years) and more likely to have local disease (76% vs. 31%, p < 0.001), tumor size < 10 cm (69% vs. 31%, p = 0.007), and better 5-year survival (91.1% vs. 29.8%, p < 0.001). After adjustment, the most significant predictors of cancer-specific death were age 5-19 years (HR 8.6, p = 0.001) and distant disease (HR 3.3, p = 0.01). After accounting for tumor size, only age maintained statistical significance (HR 9.9, p = 0.009). CONCLUSIONS: Our study represents one of the largest reviews of pediatric ACC. An age of ≤ 4 years was associated with better outcome. Potential factors responsible for this include patient and tumor related factors.

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.

Pediatric specialist care is associated with a lower risk of bowel resection in children with intussusception: a population-based analysis.

BACKGROUND: Although previous studies have shown that radiologic intussusception reduction is more likely at children's hospitals, no study to date has compared outcomes among children advancing to surgical intervention. We hypothesized that rates of bowel resection would differ between hospitals with and without pediatric surgeons. STUDY DESIGN: We conducted a population-based retrospective cohort study using Washington State discharge records. All children younger than 18 years undergoing operative intussusception reduction between 1999 and 2009 were included (n = 327). Data were collected on demographics, disease severity, comorbidities, and concomitant gastrointestinal pathology. Multivariate logistic regression was used to estimate odds of intestinal resection during operative intussusception reduction. RESULTS: Pediatric hospitals treated a smaller proportion of children older than 4 years of age (12.1% vs 44.4%), as well as a greater proportion of Medicaid patients (50.9% vs 42.6%). Patients at pediatric hospitals had a lower prevalence of underlying intestinal anomalies or identifiable mass lesions (14.3% vs 16.7%). "Severe disease" (perforation, ischemia, acidosis) was more common at pediatric hospitals (17.6% vs 9.3%). Overall, bowel resection was more commonly performed at nonpediatric hospitals (59.3% vs 33.0%). On multivariate analysis, the odds of bowel resection were significantly lower at pediatric compared with nonpediatric hospitals (odds ratio [OR] 0.20, p < 0.001), and this association was strongest in younger patients. Adjusted odds of postoperative complications were greater for bowel resection patients (OR 2.83, p < 0.001). CONCLUSIONS: Bowel resection during operative intussusception reduction is more likely at hospitals without pediatric surgeons, and is associated with increased complications. Improved outcomes may be achieved by efforts aimed at standardizing care and decreasing variability in the treatment of pediatric intussusception.

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.

Influence of surgeon experience, hospital volume, and specialty designation on outcomes in pediatric surgery: a systematic review.

IMPORTANCE: Analyses of volume-outcome relationships in adult surgery have found that hospital and physician characteristics affect patient outcomes, such as length of stay, hospital charges, complications, and mortality. Similar investigations in children's surgical specialties are fewer in number, and their conclusions are less clear. OBJECTIVE: To review the evidence regarding surgeon or hospital experience and their influence on outcomes in children's surgery. EVIDENCE REVIEW: A MEDLINE and EMBASE search was conducted for English-language studies published from January 1, 1980, through April 13, 2012. Titles and abstracts were screened in a standardized manner by 2 reviewers. Studies selected for inclusion had to use a measure of hospital or surgeon experience as a predictor variable and had to report postoperative outcomes as dependent response variables. Included studies were reviewed with regard to methodologic quality, and study results were extracted. FINDINGS: Sixty-three studies were reviewed. Significant heterogeneity was detected in exposure definitions, outcome measures, and risk adjustment, with the greatest heterogeneity seen in appendectomy studies. Various exposure levels were examined: hospital level in 48 (68%) studies, surgeon level in 11 (17%), and both in 9 (14%). Nineteen percent of studies did not adjust for confounding, and 57% did not adjust for sample clustering. The most consistent methods and reproducible results were seen in the pediatric cardiac surgical literature. Forty-nine studies (78%) showed positive correlation between experience and most primary outcomes, but differences in outcomes and exposure definitions made comparisons between studies difficult. In general, hospital-level factors tended to correlate with outcomes for high-complexity procedures, whereas surgeon-level factors tended to correlate with outcomes for more common procedures. CONCLUSIONS AND RELEVANCE: Data on experience-related outcomes in children's surgery are limited in number and vary widely in methodologic quality. Future studies should seek both to standardize definitions, making results more applicable, and to differentiate procedures affected by surgeon experience from those more affected by hospital resources and system-level variables.