Characterization of long-term outcomes for pediatric patients with epithelioid hemangioma.

Epithelioid hemangioma (EH) is a rare benign vascular tumor that occurs in soft tissues and bone and presents between the third and sixth decades of life. Little is known about the clinical course and outcomes of pediatric EH. We report 11 patients diagnosed with EH at a median age of 14.4 years. One patient treated with interferon and one with sirolimus exhibited partial response for >2 years. Although a benign neoplasm, EH is difficult to manage without standard protocols and portends considerable morbidity. Our findings suggest medical management, particularly sirolimus, may benefit these patients; however, long-term follow-up is needed.

A systematic review and individual patient data meta-analysis of published randomized clinical trials comparing early versus interval appendectomy for children with perforated appendicitis.

PURPOSE: Our objective was to perform a meta-analysis on RCTs that compared outcomes in children with perforated appendicitis (PA) who underwent either early appendectomy (EA) or interval appendectomy (IA). We also sought to determine if the presence of an intra-abdominal abscess (IAA) at admission impacted treatment strategy and outcomes. METHODS: We identified two RCTs comparing EA versus IA in children with PA. A meta-analysis was performed using regression models and the overall adverse event rate was analyzed. The treatment effect variation depending on the presence of IAA at admission was also evaluated. RESULTS: EA significantly reduced the odds of an adverse event (OR 0.28, 95 % CI 0.1-0.77) and an unplanned readmission (OR 0.08, 95 % CI 0.01-0.67), as well as the total charges (79 % of the IA, 95 % CI 63-100) for those who did not have an IAA at admission. In children with an IAA, there was no difference between EA and IA. However, heterogeneity of treatment effect was present regarding IAA at presentation. CONCLUSIONS: While EA appears to improve outcomes in patients without an abscess, the published data support no significant difference in outcomes between EA and IA in patients with an abscess.

The 2002-2007 trends of prevalence of asthma, allergic rhinitis and eczema in Irish schoolchildren.

BACKGROUND: Irish adolescents have the third highest asthma prevalence in the world. Few data examine trends of prevalence of asthma and allergic disorders in younger Irish children. We compared the prevalence of asthma, allergic rhinitis and eczema in school children, aged 6-9 in 2002 and 2007. METHODS: In two cross-sectional studies, parents of children aged 6-9, completed identical ISAAC-based questionnaires in the same 24 Cork City schools in 2002 (n = 1474, response rate = 74.8%) and in 2007 (n = 1535, response rate = 76.2%). RESULTS: The prevalence of asthma in our study population of schoolchildren, aged 6-9 remained stable in 2007 (23.5%) since the 2002 finding of 21.7% (p = 0.27). Significant increases were found in the prevalence of rhino-conjunctivitis (7.6-10.6%, p = 0.005) and eczema (8.9-13.5%, p < 0.001). In 2007, male preponderance for suffering the symptoms of current asthma existed (M:F 1.2:1, p = 0.075), although it had lost its significance since 2002 (M:F 1.5:1, p < 0.001). M:F ratio for most allergic rhinitis related symptoms moved from equal sex distribution to male predominance (nasal problems ever: 2002 - M:F, 1.1:1, p = 0.117; 2007 - M:F, 1.2:1, p = 0.012, current rhinitis: 2002 - M:F 1.2:1, p = 0.98; 2007 M:F 1.3:1, p = 0.009, hay fever ever: 2002 - M:F 1:1, p = 0.57; 2007 - M:F 1.5:1, p = 0.007). The sex-specific prevalence of rhino-conjunctivitis and the severity of symptoms suffered, remained equally sex distributed in both timeframes. From 2002 to 2007, the prevalence of all the reported symptoms of eczema were equally distributed between the sexes, while lifetime prevalence of eczema moved from male predominance to equal distribution. CONCLUSION: The prevalence of asthma in 6-9 yr old Cork schoolchildren remained static between 2002 and 2007; however, rhino-conjunctivitis and eczema have become increasingly prevalent. Co-morbidity of allergic conditions continues to pose a considerable health burden in this young population. We also demonstrated an alteration in the sex-specific profile of current asthma and lifetime allergic rhinitis towards equalization in distribution.

Thoracoscopic esophagomyotomy for achalasia in the pediatric population: A retrospective cohort study.

BACKGROUND/PURPOSE: Achalasia is an extremely rare disease in children (0.1 per 100,000 individuals). Standard treatments for this include pneumatic dilation and esophagomyotomy. Minimally invasive esophagomyotomies have increasingly been used owing to improved postoperative pain and length of stay. We describe our experience with thoracoscopic esophagomyotomy in this population. METHODS: This is a retrospective cohort study of all patients at our institution who underwent thoracoscopic esophagomyotomy for achalasia from 1995 to 2016. We used endoscopic guidance during all procedures. No fundoplication was performed. RESULTS: Thirty-one patients were included in this study. Thirteen patients underwent pneumatic dilations prior to their operation with a median of 3 dilations. Two patients had a mucosal injury during the case. There were no conversions to an open procedure. Median length of stay was 2 days. After the procedure, 97% of patients had initial symptom relief. Eight patients (26%) required postoperative pneumatic dilations for recurrent symptoms; there was a greater chance of this (OR 8.5) if they had a preoperative dilation. No patients required a fundoplication for reflux postoperatively. CONCLUSIONS: Thoracoscopic esophagomyotomy is a safe and effective procedure for achalasia in the pediatric population. It should be considered as an alternative to the laparoscopic approach for these patients. LEVEL OF EVIDENCE: IV.

Reality check: What happens when patients with anorectal malformations grow up? A pilot study of medical care transition from the adult patient perspective.

BACKGROUND: There has been an increased focus on transition of pediatric and adolescent patients to adult centered care. For patients with rare and complex conditions, such as anorectal malformations (ARM), difficulties are compounded by the absence of structured transition protocols and lack of adult provider expertise. We sought to explore the actual experiences of adult patients with ARM and distinguish if there are factors that influence the ability to achieve satisfactory transition to adult centered medical care. METHODS: Patients older than 18years with ARM were identified through a hospital database and social support networks, and invited to participate in an anonymous questionnaire. Patients were asked to describe their transition experience as well as report their demographics, and functional outcomes (bowel and bladder function). RESULTS: Twenty-six surveys were completed. Comparison of those respondents (53%) who had transitioned to adult providers to those that had not yet transferred care demonstrated no difference in satisfaction with bowel and bladder function, presence of ostomy, and education level. There was a slight trend for patients with more complex malformations to seek further medical care with adult providers. Of those who transitioned, 67% reported no discussion prior to their transition and 71% received no recommendations for follow-up providers. CONCLUSIONS: This pilot project suggests that a significant number of adult patients with ARM lack transition preparation and fail to find adult providers with expertise. Future studies will need to find optimal ways to address these issues. TYPE OF STUDY: Survey. Retrospective Study LEVELS OF EVIDENCE: Level II.

Improvements in Antimicrobial Prescribing and Outcomes in Pediatric Complicated Appendicitis.

BACKGROUND: Complicated appendicitis, characterized by perforation and/or peritonitis, is common in children, and late infectious complications are frequent. The best antibiotic treatment approach is unknown, resulting in substantial variation in care. We evaluated the effects of 2 successive interventions, an antimicrobial stewardship program (ASP) and a condition-specific clinical practice guideline (CPG), on antimicrobial utilization and patient outcomes in these patients. METHODS: The ASP at our institution was begun in March 2012. The CPG, a standardized antibiotic treatment, was implemented in July 2013. We reviewed every case of complicated appendicitis managed with early appendectomy between January 2011 and October 2014. Patients were thus divided into 3 eras based on their exposure to the following: (1) neither intervention, (2) ASP only or (3) both ASP and CPG. We compared measures of antibiotic utilization and clinical outcomes among the 3 eras. RESULTS: A total of 313 patients were included in the study: 91 exposed to neither intervention; 100 exposed to only the ASP; and 122 exposed to both interventions. With ASP implementation, there were declines in the use of unnecessarily broad or toxic antibiotic regimens. With CPG implementation, there was a decrease in total antibiotic utilization and discharges with intravenous antibiotics. Compliance with CPG-recommended antibiotics exceeded 90%. There was no significant change in overall adverse events; there was a decline in the incidence of surgical-site infections in patients exposed to both interventions. CONCLUSIONS: Complicated appendicitis is an important target for antimicrobial stewardship and quality improvement efforts. A condition-specific CPG can improve both antimicrobial utilization and clinical outcomes.

Does the American College of Surgeons National Surgical Quality Improvement Program pediatric provide actionable quality improvement data for surgical neonates?

BACKGROUND/PURPOSE: The purpose of this project was to examine the American College of Surgeons National Surgical Quality Improvement Program Pediatric (ACSNSQIP-P) Participant Use File (PUF) to compare risk-adjusted outcomes of neonates versus other pediatric surgical patients. METHODS: In the ACS-NSQIP-P 2012-2013 PUF, patients were classified as preterm neonate, term neonate, or nonneonate at the time of surgery. The primary outcomes were 30-day mortality and composite morbidity. Patient characteristics significantly associated with the primary outcomes were used to build a multivariate logistic regression model. RESULTS: The overall 30-day mortality rate for preterm neonates, term neonate, and nonneonates was 4.9%, 2.0%, 0.1%, respectively (p<0.0001). The overall 30-day morbidity rate for preterm neonates, term neonates, and nonneonates was 27.0%, 17.4%, 6.4%, respectively (p<0.0001). After adjustment for preoperative and operative risk factors, both preterm (adjusted odds ratio, 95% CI: 2.0, 1.4-3.0) and term neonates (aOR, 95% CI: 1.9, 1.2-3.1) had a significantly increased odds of 30-day mortality compared to nonneonates. CONCLUSION: Surgical neonates are a cohort who are particularity susceptible to postoperative morbidity and mortality after adjusting for preoperative and operative risk factors. Collaborative efforts focusing on surgical neonates are needed to understand the unique characteristics of this cohort and identify the areas where the morbidity and mortality can be improved.

Effect of a Clinical Practice Guideline for Pediatric Complicated Appendicitis.

IMPORTANCE: Complicated appendicitis is a common condition in children that causes substantial morbidity. Significant variation in practice exists within and between centers. We observed highly variable practices within our hospital and hypothesized that a clinical practice guideline (CPG) would standardize care and be associated with improved patient outcomes. OBJECTIVE: To determine whether a CPG for complicated appendicitis could be associated with improved clinical outcomes. DESIGN, SETTING, AND PARTICIPANTS: A comprehensive CPG was developed for all children with complicated appendicitis at Monroe Carell Jr Children's Hospital at Vanderbilt, a freestanding children's hospital in Nashville, Tennessee, and was implemented in July 2013. All patients with complicated appendicitis who were treated with early appendectomy during the study period were included in the study. Patients were divided into 2 cohorts, based on whether they were treated before or after CPG implementation. Clinical characteristics and outcomes were recorded for 30 months prior to and 16 months following CPG implementation. EXPOSURE: Clinical practice guideline developed for all children with complicated appendicitis at Monroe Carell Jr Children's Hospital at Vanderbilt. MAIN OUTCOMES AND MEASURES: The primary outcome measure was the occurrence of any adverse event such as readmission or surgical site infection. In addition, resource use, practice variation, and CPG adherence were assessed. RESULTS: Of the 313 patients included in the study, 183 were boys (58.5%) and 234 were white (74.8%). Complete CPG adherence occurred in 78.7% of cases (n = 96). The pre-CPG group included 191 patients with a mean (SD) age of 8.8 (4.0) years, and the post-CPG group included 122 patients with a mean (SD) age of 8.7 (4.1) years. Compared with the pre-CPG group, patients in the post-CPG group were less likely to receive a peripherally inserted central catheter (2.5%, n = 3 vs 30.4%, n = 58; P < .001) or require a postoperative computed tomographic scan (13.1%, n = 16 vs 29.3%, n = 56; P = .001), and length of hospital stay was significantly reduced (4.6 days post-CPG vs 5.1 days pre-CPG, P < .05). Patients in the post-CPG group were less likely to have a surgical site infection (relative risk [RR], 0.41; 95% CI, 0.27-0.74) or require a second operation (RR, 0.35; 95% CI, 0.12-1.00). In the pre-CPG group, 30.9% of patients (n = 59) experienced any adverse event, while 22.1% of post-CPG patients (n = 27) experienced any adverse event (RR, 0.72; 95% CI, 0.48-1.06). CONCLUSIONS AND RELEVANCE: Significant practice variation exists among surgeons in the management of pediatric complicated appendicitis. In our institution, a CPG that standardized practice patterns was associated with reduced resource use and improved patient outcomes. Most surgeons had very high compliance with the CPG.

Inguinal hernia repair in premature infants: more questions than answers.

This review shows that there are many single institution studies reviewing outcomes of premature infants with IH. However, the numbers of patients in these studies are often small and most studies were retrospective, therefore, these studies were subject to the limitations inherent to observational studies for identifying best treatment methods. Nevertheless, the studies show that risks are high in this population and that outcomes may vary with the timing of repair. There have been calls for multicentre randomised trials comparing early versus later IH repair from all over the world and for a very long time. Yet, despite the frequency of IH repair in premature infants, this issue remains unstudied in a high-quality manner. A large, multicentre randomised trial is currently underway to address the effect of timing on the short-term and long-term safety and efficacy of IH repair in this population so that we may be able to deliver safe surgical care to this vulnerable population.

Neonatal carotid repair at ECMO decannulation: patency rates and early neurologic outcomes.

PURPOSE: Neonates placed on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) undergo either carotid repair or ligation at decannulation. Study aims were to evaluate carotid patency rates after repair and to compare early neurologic outcomes between repaired and ligated patients. METHODS: A retrospective study of all neonates without congenital heart disease (CHD) who had VA-ECMO between 1989 and 2012 was completed using our institutional ECMO Registry. Carotid patency after repair, neuroimaging studies, and auditory brainstem response (ABR) testing at time of discharge were examined. RESULTS: 140 neonates were placed on VA-ECMO during the study period. Among survivors, 84% of carotids repaired and imaged remained patent at last study. No significant differences were observed between infants in the repaired and ligated groups regarding diagnosis, ECMO duration, or length of stay. A large proportion (43%) developed a severe brain lesion after VA-ECMO, but few failed their ABR testing. Differences in early neurologic outcomes between the two groups of survivors were not significant. CONCLUSIONS: At this single institution, carotid patency is excellent following repair at ECMO decannulation. No increased incidence of severe brain lesions or greater neurosensory impairment in the repair group was observed. Further studies are needed to investigate the effects of ligation on longer-term neurocognitive outcomes.

Early versus delayed surgical correction of malrotation in children with critical congenital heart disease.

PURPOSE: The purpose of this study was to compare outcomes between early and delayed surgical correction of malrotation in children with critical congenital heart disease (CHD). METHODS: Patients with CHD who underwent cardiac surgery by 1 year of age and had malrotation diagnosed during their initial admission at 34 hospitals contributing to the Pediatric Health Information System in 2004-2009 were included. Ladd's procedures performed during the first admission were considered early correction, and those at a subsequent admission were considered delayed. Interhospital variability in the proportion of patients undergoing delayed correction was assessed, and outcomes were compared between the groups. RESULTS: Of the 324 patients identified, 85.2% underwent early correction. Significant variability existed in the proportion of patients undergoing delayed correction across hospitals (p<0.0001). Baseline characteristics, including severity of CHD, were similar between the groups. In the delayed group, 27% of patients underwent a Ladd's procedure during an urgent or emergent admission, but none had volvulus or underwent intestinal resection. Rates of mortality and readmission within 1 year of malrotation diagnosis were similar in both groups. Chart validation confirmed 100% accuracy of diagnosis and treatment group assignment. CONCLUSIONS: In patients with critical CHD, delayed operative intervention for malrotation without volvulus may be a reasonable alternative.

Does timing of neonatal inguinal hernia repair affect outcomes?

PURPOSE: The purpose of this study was to examine practice variability and compare outcomes between early and delayed neonatal inguinal hernia repair (IHR). METHODS: Patients admitted to neonatal intensive care units with a diagnosis of IH who underwent IHR by age 1 year in the Pediatric Health Information System from 1999 to 2011 were included. IHR after the index hospitalization was considered delayed. Inter-hospital variability in the proportion of delayed repairs and differences in outcomes for each group were compared. A propensity score matched analysis was performed to account for baseline differences between treatment groups. RESULTS: Of the 2030 patients identified, 32.9% underwent delayed IHR with significant variability in the proportion of patients having delayed repair across hospitals (p<0.0001). More patients in the delayed group had a congenital anomaly or received life supportive measures prior to IHR (all p<0.01), and 8.2% of patients undergoing delayed repair had a diagnosis of incarceration at repair. More patients in the early group underwent reoperation for hernia within 1 year (5.9% vs. 3.7%, p=0.02). Results were similar after performing a propensity score matched analysis. CONCLUSIONS: Significant variability in practice exists between children's hospitals in the timing of IHR, with delayed repair associated with incarceration and early repair with a higher rate of reoperation.

Is NSQIP Pediatric review representative of total institutional experience for children undergoing appendectomy?

BACKGROUND/PURPOSE: NSQIP Pediatric (NSQIP-P) is a robust quality improvement effort. A limitation of the NSQIP process lies in capturing a small proportion of the total case volume. This study examines whether appendectomies captured by NSQIP-P are concordant with all appendectomies, the most commonly captured procedure in 2011. METHODS: We compared case mix and 30-day outcomes between children undergoing an appendectomy who were included in NSQIP (n=80) and children not captured by NSQIP (n=276) during 2011 at a tertiary referral children's hospital. A single surgical case reviewer reviewed all cases using NSQIP-P methodology. RESULTS: NSQIP-P captured 80 of a total of 356 appendectomies (22%). The case mix was similar between NSQIP and non-NSQIP groups (e.g., 31% of each group had complicated appendicitis). Outcomes were also similar; post-operative occurrences, readmissions and return to the operation room occurred at rates of 7.5% vs. 7.6%, 5% vs. 4.7%, and 3.8% vs. 4.3% respectively. CONCLUSION: Although NSQIP-P captured a minority of the total patient population that had an appendectomy, the case mix and outcomes were similar. Our results offer reassurance that NSQIP-P data are representative of the larger population for this procedure. Whether this concordance exists for procedures less commonly performed is unknown and a focus of ongoing work.

Improving gastroschisis outcomes: does birth place matter?

PURPOSE: Babies born in the hospital where they obtain definitive surgical care do not require transportation between institutions and may have shorter time to surgical intervention. Whether these differences result in meaningful improvement in outcomes has been debated. A multi-institutional retrospective study was performed comparing outcomes based on birthplace. METHODS: Six institutions within the PedSRC reviewed infants born with gastroschisis from 2008 to 2013. Birthplace, perinatal, and postoperative data were collected. Based on the P-NSQIP definition, inborn was defined as birth at the pediatric hospital where repair occurred. The primary outcome was days to full enteral nutrition (FEN; 120kcal/kg/day). RESULTS: 528 patients with gastroschisis were identified: 286 inborn, 242 outborn. Days to FEN, time to bowel coverage and abdominal wall closure, primary closure rate, and length of stay significantly favored inborn patients. In multivariable analysis, birthplace was not a significant predictor of time to FEN. Gestational age, presence of atresia or necrosis, primary closure rate, and time to abdominal wall closure were significant predictors. CONCLUSIONS: Inborn patients had bowel coverage and definitive closure sooner with fewer days to full feeds and shorter length of stay. Birthplace appears to be important and should be considered in efforts to improve outcomes in patients with gastroschisis.

Composite tissue allotransplantation: a review of relevant immunological issues for plastic surgeons.

BACKGROUND: Composite tissue allotransplantation of hand, facial and other tissues is now a clinical reality. The terminology, treatment principles, drug combinations, dosage schedules and mechanisms of the immunosuppression medications on which contemporary transplant surgery is based are unfamiliar to plastic surgeons and most healthcare providers outside the field of transplantation medicine. With this in mind, the purpose of this manuscript is to provide plastic surgeons with a comprehensive and understandable review of key immunological principles relevant to composite tissue allotransplantation. METHODS: We present an overview of the immunological basis of composite tissue allotransplantation aimed at the plastic surgery readership, based on our own experience plus manuscripts sourced from MEDLINE, EMBASE, text books, ancient manuscripts and illustrations. RESULTS: In this manuscript we provide the reader with a brief history of composite tissue allotransplantation (CTA), a concise description of the immunological terminology, treatment approaches, risks associated with immunosuppressive therapy, risk acceptance, and current research avenues relating to contemporary CTA. CONCLUSION: Today, as transplant and reconstructive surgeons join forces to move hand and facial tissue allotransplantation into the clinical arena, it is important that plastic surgeons have an understanding of the major immunological principles upon which this new treatment is based.