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Purpose Recent studies have identified transanastomotic tubes (TATs) as a risk factor for the development of anastomotic strictures after repair of esophageal atresia with tracheoesophageal fistula (EATEF). We further investigated these findings in a multicenter study. Methods We conducted a retrospective cohort study at three university-affiliated hospitals in the province of Quebec. All patients with types C and D EATEF who underwent primary repair between January 1993 and August 2018 were included. Anastomotic stricture was defined as clinical symptoms of stricture with confirmation on esophagram or endoscopy. Multivariate logistic regression and the Wilcoxon Rank-Sum test were used to evaluate the primary outcome of stricture within one year of surgery and secondary outcome of duration of postoperative total parenteral nutrition (TPN). Results 244 patients were included, of which 234 (96%) were type C and 10 (4%) were type D. The anastomotic stricture rate at 1 year was 30%. TATs were utilized in 61% of patients. Thirty-six percent of patients with TATs developed a stricture within one year, as compared to 19% of patients without TATs (p = 0.005). TATs were associated with stricture on univariate analysis (OR 2.49, p = 0.004, 95% CI: 1.37-4.69). On multivariate analysis, after adjusting for gestational age, birth weight, leak, long gap, anastomotic tension, and daily acid suppression, patients with TATs had 2.72 times higher odds of developing a stricture as compared to patients without TATs (p = 0.006, 95% CI: 1.35-5.74). The median duration of TPN was 9 days in both groups (p = 0.139, IQR 6-14 in patients with TATs versus IQR 7-16 in patients without). Conclusion Transanastomotic tubes are associated with a significantly higher risk of postoperative stricture following repair of esophageal atresia with tracheoesophageal fistula and do not shorten the duration of total parenteral nutrition. Level of evidence III.

Conservative treatment of pectus excavatum with a vacuum bell device may be an attractive alternative to surgical repair. We describe an early North American experience with this device.Prospectively maintained chest wall clinic registries from two institutions were reviewed to identify pectus excavatum patients ≤21 years treated with the vacuum bell from 2013 to 2017. Multivariate linear regression was used to compare mean improvements in deformity-depth and Haller Index between groups of patients based on age and usage metrics (hours/day and days/week).Thirty-one patients with a median age of 14 years received treatment with the device. Mean follow-up duration was 18 months. Median depth and Haller Index at treatment onset were 2.3 cm and 3.9, respectively. Improvements in deformity-depth were superior with device usage2 h/day (p 0.01) and daily use (p 0.01). After adjusting for compliance, younger age of treatment onset was associated with greater improvement in Haller Index but not deformity depth.Our prospective early North American experience found the vacuum bell to be a potential alternative to surgical treatment for pectus excavatum. Longer usage periods in a daily frequency are associated with best results.Treatment study; case series with no comparison group.Level IV.

Purpose Postoperative pain remains a considerable concern for patients and families. We assessed whether nebulized ropivacaine reduces morphine consumption and pain after laparoscopic appendectomy for uncomplicated appendicitis in children. Methods Patients 7–17 years old with uncomplicated appendicitis were randomized to ropivacaine (intervention arm) or saline nebulization (placebo arm) at the onset of laparoscopy. Nonconsenting individuals were treated with standard care and invited to provide clinical data (baseline arm). The primary outcome was in-patient morphine utilization. Secondary outcomes included pain scores at multiple time-points, markers of recovery, operative times, and surgeon satisfaction. The trial was registered ( NCT02624089 ). Results Study enrollment was 116 patients over a 1-year period: Intervention (n = 43), Placebo (n = 39), Baseline (n = 34). No differences in baseline characteristics were noted between groups. No difference was noted in overall in-patient morphine consumption between randomized groups (0.31 vs. 0.35 mg/kg, p = 0.42) or between ropivacaine and baseline (0.31 vs. 0.277 mg/kg, p = 0.62). Although operative times were comparable between groups, 63% of surgeon respondents felt that nebulization obscured visualization. Conclusion Nebulized ropivacaine did not reduce postoperative morphine consumption or pain scores after laparoscopic appendectomy for simple appendicitis in children. Given that it decreases visualization and likely increases costs, nebulized administration of intraperitoneal analgesia does not appear warranted in this context. Type of study Treatment study. Level of evidence Level I.

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p

Purpose This study reports the incidence, severity, and predictors of musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, following thoracic procedures in children. Methods Children younger than 14 years who had thoracic surgery between 1997 and 2012 and had no other predispositions to MD, underwent longitudinal follow-ups with dedicated musculoskeletal examination performed in an esophageal atresia, orthopedic, or research clinic. Incidence of MD was calculated, and logistic regression methods were used to determine independent predictors, including sex, gestational age, age at procedure, serratus anterior muscle division, and chest tube placement. Results The study cohort consisted of 104 patients followed for a median of 10.8 years (range 3–21). A total of 56 MD developed in 41 patients (39%), including scapular winging (24; 23%), scoliosis (17; 16%), and chest wall anomalies (15; 14%). The majority of MD were subclinical, with only 8 patients [8% (6 thoracotomies, 2 thoracoscopies)] requiring intervention. Among patients who underwent thoracotomies (93, 89%), serratus anterior muscle division was the only significant predictor of the development of MD [OR 8.9; 95% CI 2.8–32.6]. Conclusion Musculoskeletal deformities develop in a significant proportion of children following thoracic surgery, but most are subclinical. A muscle-sparing technique decreases the incidence of these deformities. Type of Study Prospective Cohort Study. Level of Evidence Level II.

Purpose Despite a wide spectrum of severity, perforated appendicitis in children is typically considered a single entity in outcomes studies. We performed a prospective cohort study to define a risk stratification system that correlates with outcomes and resource utilization. Methods A prospective study was conducted of all children operated for perforated appendicitis between May 2015 and December 2016 at a tertiary free-standing university children's hospital. Surgical findings were classified into one of four grades of perforation: I. localized or contained perforation, II. Contained abscess with no generalized peritonitis, III. Generalized peritonitis with no dominant abscess, IV. Generalized peritonitis with one or more dominant abscesses. All patients were treated on a clinical pathway that involved all points of care from admission to final follow-up. Outcomes and resource utilization measures were analyzed using Fisher's exact test, Kruskal–Wallis test, One-way ANOVA, and logistic regression. Results During the study period, 122 patients completed treatment, and 100% had documented follow-up at a median of 25days after operation. Grades of perforation were: I, 20.5%; II, 37.7%; III, 10.7%; IV, 31.1%. Postoperative abscesses occurred in 12 (9.8%) of patients, almost exclusively in Grade IV perforations. Hospital stay, duration of antibiotics, TPN utilization, and the incidence of postoperative imaging significantly increased with increasing grade of perforation. Conclusion Outcomes and resource utilization strongly correlate with increasing grade of perforated appendicitis. Postoperative abscesses, additional imaging, and additional invasive procedures occur disproportionately in patients who present with diffuse peritonitis and abscess formation. The current stratification allows risk-adjusted outcome reporting and appropriate assignment of resource burden. Level of evidence I (Prognosis Study).

Background The treatment of perforated appendicitis in children is characterized by significant variability in care, morbidity, resource utilization, and outcomes. We prospectively studied how minimization of care variability affects outcomes. Methods A clinical pathway for perforated appendicitis, in use for three decades, was further standardized in May 2015 by initiation of a disease severity classification, refinement of discharge criteria, standardization of the operation, and establishment of criteria for use of postoperative total parenteral nutrition, imaging, and invasive procedures. Prospective evaluation of all children treated for 20months on the new fully standardized protocol was conducted and compared to a retrospective cohort treated over 58months prior to standardization. Differences between outcomes before and after standardization were analyzed using regression analysis techniques to adjust for disease severity. Results Median follow-up time post discharge was 25 and 14days in the post- and prestandardization groups, respectively. Standardization significantly reduced postoperative abscess (9.8% vs. 17.4%, p=0.001) and hospital stay (p=0.002). Standardization reduced the odds of developing a postoperative abscess by four fold. Conclusion Minimizing variability of care at all points in the treatment of perforated appendicitis significantly improves outcomes. Type of study Prospective Cohort Study. Level of evidence Level II.

The GPS enables risk stratification for gastroschisis and helps discriminate low from high morbidity groups. The purpose of this study was to revalidate GPS's characterization of a high morbidity group and to quantify relationships between the GPS and outcomes.With REB approval, complete survivor data from a national gastroschisis registry was collected. GPS bowel injury scoring was revalidated excluding the initial inception/validation cohorts (2011). Length of stay (LOS), 1st enteral feed days (dFPO), TPN days (dTPN), and aggregate complications (COMP) were compared between low and high morbidity risk groups. Mathematical relationships between outcomes and integer increases in GPS were explored using the entire cohort (2005-present).Median (range) LOS, dPO, and dTPN for the entire cohort (n=849) was 36 (26,62), 13 (9,18), and 27 (20,46) days, respectively. High-risk patients (GPS≥2; n=80) experienced significantly worse outcomes than low risk patients (n=263). Each integer increase in GPS was associated with increases in LOS and dTPN by 16.9 and 12.7days, respectively (p0.01). COMP rate was also increased in the high-risk cohort (46.3% vs. 22.8%; p0.01).The GPS effectively discriminates low from high morbidity risk groups. Within the high risk group, integer increases in GPS produce quantitatively differentiated outcomes which may guide initial counseling and resource allocation.IIb.

Purpose Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers. Methods Prenatal lung–head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. Results Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values > 45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. Conclusions This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review. Level of evidence IV.

Low- and middle-income countries (LMICs) have only 19% of the global surgical workforce yet see 80% of worldwide deaths from noncommunicable diseases. We aimed to interrogate the correlation between pediatric surgical workforce density (PSWD) and survival from pediatric surgical conditions worldwide.A systematic review of online databases identified outcome studies for key pediatric surgical conditions (gastroschisis, esophageal atresia, intestinal atresia, and typhoid perforation) as well as PSWD data across low-income (LICs), middle-income (MICs), and high-income countries (HICs). PSWD was expressed as the number of PSs/million children under 15 years of age and we correlated this to surgical outcomes for our case series.PSWD ranged between zero (Burundi, The Gambia, and Mauritania) and 125.2 (Poland) across 86 countries. Outcomes for at least one condition were obtained in 61 countries: 50 outcomes in HICs, 52 in MICs and 8 in LICs. The mean survival in our case series was 42.3%, 69.4% and 91.6% for LICs, MICs, and HICs, respectively. A PSWD ≥4 PSs/million children under 15 years of age significantly correlated to odds of survival ≥80% (OR 16.8, p 0.0001, 95% CI 5.66-49.88). Specifically in the studied LICs and MICs, increasing the PSWD to 4 would require training 1427 additional surgeons.Using a novel approach, we have established a benchmark for the scale-up of pediatric surgical workforce, which may support broader efforts to reduce childhood deaths from congenital disease.2c - Outcomes Research.

Background Optimal timing of delivery in fetuses with gastroschisis (GS) is unknown. Some favor early induced delivery to prevent bowel injury. This study evaluates the correlation between bowel injury and the gestational age at birth using the Gastroschisis Prognostic Score (GPS). Methods A national database was analyzed from 2005 to 2013. Patients were pooled based on their gestational age at birth. The mean GPS and % of patients with severe bowel matting were tabulated for each week in utero. Regression modeling was used to evaluate the relationship between the dependent (severe matting and GPS) and independent (gestational age) variables and the R 2 coefficient of determination was derived to evaluate model strength. Additional factors influencing the timing of delivery were evaluated. Results Of 780 cases, 88 were excluded because of missing data. A linear relationship is seen between increasing gestational age and decreasing bowel matting ( R 2 =0.66) and GPS ( R 2 =0.72). For every week in utero, the % of patients with severe matting decreases by 3.6%. Conclusion Early induced delivery simply to protect the bowel from ongoing in utero damage appears unfounded and should be reserved for evidence of closing gastroschisis or traditional obstetrical/fetal indications.

Postoperative abscesses after perforated appendicitis have no clear risk factors or indications for percutaneous drainage. Our study addressed these two issues.A logistic regression model was used to delineate risk factors for postoperative abscess in children with perforated appendicitis treated during a recent 5-year period. Drainage of abscess was compared to antibiotic treatment.Postoperative abscess occurred in 42 (14.8%) of 284 patients. Higher WBC count, presence of bowel obstruction at presentation, diffuse peritonitis with a dominant abscess at surgery, and one specific surgeon were significantly associated with postoperative abscess, while fever or pain requiring narcotics at the time of abscess diagnosis was significantly associated with drainage. Compared to non-drainage, those drained had longer hospital stay including readmissions (15.9 ± 5.3 vs. 12.2 ± 4.6 days, p0.005) and less readmissions (9.5 vs. 33.3%, p = 0.06). Over the 5-year period, there was no increased trend in abscess occurrence (p = 0.56), but there was an increased trend in the use of percutaneous drainage (p = 0.02).The risk of a postoperative abscess can be predicted by specific clinical characteristics, surgical findings, and treatment-related factors. Percutaneous drainage was associated with longer hospital stays, but less readmissions.

The management of congenital lung malformations is controversial both in the prenatal and postnatal periods. This article attempts to inform best practice by reviewing the level of evidence with regard to prenatal diagnosis, prognosis, and management and postnatal management, including imaging, surgical indication, surgical approach, and risk of malignancy. We present a series of clinically relevant statements along those topics and analyze the evidence for each. In the end, we make a plea for an adequate description of the lesions, both before and after birth, which will allow future comparisons between management options and the initiation of prospective registries.

Background Orofacial clefts are usually isolated cases but can be associated with other congenital malformations that are either recognised or unrecognised syndromes. The reported prevalence and pattern of such associated malformations, however, vary among studies. Objectives: To assess the frequencies and aetiologies of congenital malformations and associated medical conditions in children with orofacial clefts in Burkina Faso (Western Africa). Methods A retrospective descriptive study was carried out at the El Fateh-Suka Clinic in Ouagadougou, Burkina Faso. All children who attended surgery for the repair of a cleft lip and/or palate were included in this study. Results The frequency of congenital malformations associated with cleft lip and/or palate was 39/185 (21.1%). In the group with multiple congenital malformations of unknown origin (34 patients; 18.4%), 66.7% had cleft lip and palate, followed by isolated cleft lip (27.4%) and isolated cleft palate (5.9%). The digestive system (35.3%), the musculoskeletal system (19.6%), and eye, ear, face, and neck (15.7%) were the most affected systems. In the group of syndromic malformations (five patients; 2.7%), amniotic band syndrome (one patient), Van der Woode syndrome (one patient), Goltz syndrome (one patient), and holoprosencephaly (two patients) were identified. Medical conditions included anaemia (39.4%), infections (9.2%), malnutrition (7.5%), and haemoglobinopathies (4.3%). Conclusions Congenital malformations and medical co-morbidities were frequent in children with OFCs. Further studies and a National Malformations Registry are needed to improve the comprehension of OFCs in Burkina Faso.

Background The Canadian 4-year native liver survival rate for biliary atresia (BA) after Kasai Portoenterostomy (KP) is 39%. The Canadian Biliary Atresia Registry (CBAR) was used to examine variability of surgical and medical management of BA. Methods Gastroenterologists and surgeons in all 14 Canadian pediatric tertiary centers were invited to complete an online survey of their BA management practices. Results Of gastroenterologists, diagnostic procedures included liver biopsy (92%), HIDA scan (58%), and percutaneous cholangiogram (46%). Surgeons reported Roux-en-Y lengths of 20–50cm with 78% avoiding diathermy at the portal plate; 16% performed laparoscopic exploration, but none laparoscopic KP. Postoperative corticosteroids and antibiotics were used by 24% and 85% of gastroenterologists, respectively, with similar rates for surgeons. At discharge, gastroenterologists prescribed oral antibiotics (80%), and ursodeoxycholic acid (95%), while surgeons reported lower rates (62% and 55%). Considerable variation existed in follow-up monitoring. No center had a standard protocol for evaluating suspected cholangitis. There was a lack of consensus for defining failed KP and referral criteria for transplant evaluation. Conclusion In Canada, treatment of BA is not centralized, and there is variability in diagnostic approaches and management. Collaboration through CBAR will allow for implementation and evaluation of standardized surgical and medical management with a goal to improve outcomes. Level of evidence Survey study. Level IV evidence.

We previously demonstrated feasibility, safety, and a reproducible histologic bulking effect after injection of dextranomer hyaluronic acid copolymer (DxHA) into the gastroesophageal junction of rabbits. In the current study, we investigated the potential for DxHA to augment the lower esophageal sphincter (LES) in a porcine model of gastroesophageal reflux disease (GERD).Twelve Yucatan miniature pigs underwent LES manometry and 24-hour ambulatory pH monitoring at baseline, after cardiomyectomy, and 6weeks after randomization to endoscopic injection of either DxHA or saline at the LES. After necropsy, the foregut, including injection sites, was histologically examined.Pigs in both groups had similar weight progression. Cardiomyectomy induced GERD in all animals, as measured by a rise in the median % of time pH5 from 0.6 to 11.6 (p=0.02). Endoscopic injection of DxHA resulted in a higher median difference in LES length (1.8cm vs. 0.4cm, p=0.03). In comparison with saline injection, DxHA resulted in 120% increase in LES pressure, and 76% decrease in the mean duration of reflux episodes, but these results were not statistically significant. Injection of DxHA induced a foreign body reaction with fibroblasts and giant cells.Porcine cardiomyectomy is a reproducible animal GERD model. Injection of DxHA may augment the LES, offering a potential therapeutic effect in GERD.

Summary Severe tracheomalacia (TM) is a difficult problem in esophageal atresia (EA) patients. We reviewed our experience with aortopexy and other interventions for severe TM in this population. With review ethics board approval, a retrospective review of TM in postoperative EA patients was conducted (1989–2010). Demographics, perinatal, and surgical information regarding EA repair was collected. TM infants were analyzed for symptomatology, clinical severity, investigations, interventions, and outcomes. Data are presented as proportions or median(range). One hundred and thirty-two EA patients were reviewed. Most had type C atresia (87.3%), and 18 patients (13.6%) died. Twenty-five patients (18.9%) had TM of whom five (20%) died. Median symptom onset was 18 days (0–729) after EA repair, with stridor (64%) or retractions/distress (44%) being most frequent. Four and two patients had airway obstruction or cardiorespiratory arrest, respectively. Median time from symptom onset to investigations was 11 days; these were most commonly rigid bronchoscopy (56%) and fluoroscopy (36%). Ten patients (40%) had severe TM on bronchoscopy. Six underwent aortopexy, one fundoplication, and three were treated medically. Length of hospital stay (LOS) post-aortopexy was 13 days (5–60), and ventilation time was 2 days (0–9). LOS was 60.5 (1–69) days postdiagnosis in non-aortopexy patients. Readmission rates for respiratory issues were significantly less in the aortopexy (median 0 vs. 5; P = 0.048) group over 2-year follow up after discharge. Complications of aortopexy included transfusion (1) and temporary diaphragmatic paresis (1), and one mortality secondary to severe congenital cardiac anomalies. Our experience suggests that aortopexy is safe and effective for the treatment of severe TM. It is associated with reduced LOS compared with other treatment strategies and few complications or long-term sequelae.

Purpose We analyzed the determinants of outcomes in simple gastroschisis (GS) not complicated by intestinal atresia, perforation, or necrosis. Methods All simple GS patients enrolled in a national prospective registry from 2005 to 2013 were studied. Patients below the median for total parenteral nutrition (TPN) duration (26 days) and hospital stay (34 days) were compared to those above. Univariate and multivariate logistic and linear regression analyses were employed using maternal, patient, postnatal, and treatment variables. Results Of 700 patients with simple GS, representing 76.8% of all GS patients, 690 (98.6%) survived. TPN was used in 352 (51.6%) and 330 (48.4%) patients for ≤ 26 and > 26 days, respectively. Hospital stay for 356 (51.9%) and 330 (48.1%) infants was ≤ 34 and > 34 days, respectively. Univariate analysis revealed significant differences in several patient, treatment, and postnatal factors. On multivariate analysis, prenatal sonographic bowel dilation, older age at closure, necrotizing enterocolitis, longer mechanical ventilation, and central-line associated blood stream infection (CLABSI) were independently associated with longer TPN duration and hospital stay, with CLABSI being the strongest predictor. Conclusions Prenatal bowel dilation is associated with increased morbidity in simple GS. CLABSI is the strongest predictor of outcomes. Bowel matting is not an independent risk factor. Level of evidence 2c.

INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long gap esophageal atresia set out to develop guidelines regarding the definition of long gap esophageal atresia, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that long gap esophageal atresia should be defined as any esophageal atresia that has no intra-abdominal air, realizing that this defines esophageal atresia with no distal trachea-esophageal fistula. Long gap esophageal atresia is considerably more complex than esophageal atresia with distal tracheo-esophageal fistulas, and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major esophageal atresia centers. In light of the infrequent occurrence of long gap esophageal atresia and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.

Background This study sought to establish factors that can prognosticate outcomes of bracing for pectus carinatum (PC). Methods Prospective data were collected on all patients enrolled in a dynamic bracing protocol from July 2011 to July 2015. Pressure of correction (POC) was measured at initiation of treatment, and pressure of treatment (POT) was measured pre- and post-adjustment at every follow-up visit. Univariate and Cox regression analysis tested the following possible determinants of success and bracing duration: age, sex, symmetry, POC, and POT drop during the first two follow-up visits. Results Of 114 patients, 64 (56%) succeeded, 33 (29%) were still in active bracing, and 17 (15%) failed or were lost to follow-up. In successful patients, active and maintenance bracing was 5.66±3.81 and 8.80±3.94months, respectively. Asymmetry and older age were significantly associated with failure. Multivariable Cox proportional hazard analysis of time-to-maintenance showed that asymmetry (p=0.01) and smaller first drop in POT (p=0.02) were associated with longer time to reach maintenance. Conclusions Pressure of correction does not predict failure of bracing, but older age, asymmetry, and smaller first drop in pressure of treatment are associated with failure and longer bracing duration. Level of evidence Prospective Study/Level of Evidence IV.

We previously reported a validated, objective definition of gangrenous, nonperforated appendicitis. In this study, we compared a cohort of children with gangrenous appendicitis treated with abridged antibiotics (AA) to another treated with prolonged antibiotics (PA).In 2012, our service changed its standard of care for gangrenous appendicitis from PA to AA. In PA, patients received postoperative triple antibiotics until ileus resolved, they were afebrile (37.5°C) for 24hours, and achieved a normal WBC count. In AA, patients received two doses of postoperative triple antibiotics. A PA cohort during a 12-month period (February 2010-January 2011) was compared to an AA cohort during another 12-month period (April 2012-March 2013).Twenty patients were treated with AA and 38 patients with PA. AA patients had a significantly shorter overall length of stay (2.1±1.58 vs. 3.18±1.09days, p=0.003), as well as a significantly shorter postoperative stay (1.85±1.42 vs. 2.95±1.14days, p=0.002). There were no differences between the AA and PA cohorts in wound infections (0%), intraabdominal infections (0%), or appendicitis-related readmissions (0%).Abridged postoperative antibiotics for gangrenous appendicitis significantly shorten hospital stay without increasing complications.

Anastomotic strictures (ASs) complicate the postoperative course of roughly one-third of all patients with esophageal atresia with or without tracheoesophageal fistula. Its development is multifactorial, but is due in part to tension on the anastomosis, gastroesophageal reflux disease, and the presence of a leak in the early postoperative period. Efforts at reducing the rate of AS have been largely unsuccessful, although meticulous technique and aggressive acid suppression remain the cornerstones of perioperative care. Once an AS has been confirmed, the first-line treatment remains a course of esophageal dilatation. Adjuncts to dilatation are frequently required, including steroid injection or the topical application of mitomycin C. Currently, there is insufficient evidence to promote one at the expense of the other. Esophageal stenting has recently been added to the algorithm of treatment, although additional literature is required to confirm its safety and efficacy. Finally, stricture resection followed by primary esophageal anastomosis or, rarely, esophageal replacement with an interposition graft remain options for AS refractory to all other forms of treatment.

Is it outdated now to do a thoracotomy to repair esophageal atresia (EA)? Our practices and the literature on the subject of thoracoscopic and open thoracotomy repair of EA were reviewed, seeking answers to the following questions: Is it correct to compare the new thoracoscopic approach for the repair of EA against the thoracotomy techniques of 15-30 years ago? Should post-thoracotomy scoliosis/thoracic deformity reported in up to 56% of patients be a significant current concern? Are the clips used to close the fistula in thoracoscopic repairs as safe as open suture closures? Is the leak and stricture rate similar with thoracoscopic surgery? Are the anesthesia, period of ventilation, pain, time to first feeding, and the length of hospital stay significantly different with current thoracotomy techniques compared with thoracoscopic methods? Is the cosmetic result of a thoracoscopic repair significantly better? Is the learning curve for EA thoracoscopic repair harming patients for minimal long-term benefit? These questions were scientifically unanswerable at this time. The limited EA thoracotomies currently performed have a track record of proven safety and minimal morbidity. The results published by surgeons who are pioneers in thoracoscopy may not be generalizable, and the complication rate from teams with less experience is likely underreported. In selected patients and with experienced teams, thoracoscopic EA repair is appropriate. However, EA repair via thoracotomy should, for now, remain as the 'gold standard'. Further registry-based, multicenter, comparative studies on EA repair methodologies and outcomes should provide important answers.

Purpose Little is known about liveborn CDH patients who die without surgery. We audited a national CDH cohort to determine whether these patients were different from patients who received CDH repair. Methods A national CDH database was analyzed (2005–2009). After excluding infants with severe physiologic instability and genetic/congenital malformations, a potential surgical candidate (PSC) subgroup was identified. PSCs were compared to the operative group (OG) and the operative non-survivor (ONS) subgroup. Standard statistical analyses were performed. Results Of 275 liveborns, 35 (13%) died without surgery. The PSC subgroup ( n =11) had a median survival of 10days (range: 3–18). Ten of 11 PSC infants were treated in ECMO centers, with 4 receiving ECMO. No differences in BW, GA, and rates of minor malformation were observed between PSC and OG patients. While neonatal illness severity (SNAP-II) predicted overall mortality, SNAP-II scores were similar between PSC and ONS groups (34 vs. 29; p =0.431). Furthermore, greater than 80% of infants with SNAP-II scores between 30 and 39 survived in the OG cohort. Conclusion Our analysis demonstrated that PSCs were similar to infants offered surgery based on illness severity and the presence of congenital malformations. We suggest that criteria for surgical ineligibility be developed to standardize the selection of surgical candidates.

Esophageal strictures remain the most frequent complication after esophageal atresia (EA) repair despite refinements in operative techniques. With an incidence of anastomotic stricture between 8% and 49%, EA is the most frequent cause of benign esophageal stricture in children. The mainstay of treatment for esophageal stricture is dilatation with a 58-96% success rate. In order to relieve dysphagia, between 1 and 15 dilatations will be required in each EA patient with an esophageal stricture. However dilatations may lead to complications including perforation (0.1-0.4% of all esophageal benign strictures) and sociopsychological morbidity. Fifty percent of EA strictures will improve in 6 months. However, 30% will persist and require repeat dilatations. The present article explores the variety of non-surgical alternative treatments for anastomotic strictures after EA repair, focusing on triamcinolone acetonide, mitomycin C and esophageal stents. We propose an algorithm for a more standardized therapeutic approach, with the hope that an international panel of experts could meet and establish a consensus.

Myofibromas are benign congenital tumors of soft tissue that can present at birth or during infancy in solitary or multicentric forms. Visceral myofibromas are rarely reported, but are typically symptomatic due to involvement of vital structures. We present two cases of congenital myofibromas, one obstructing the proximal esophagus and the other obstructing the distal rectum. Lessons learned from the treatment of these two patients are shared and the pertinent literature is reviewed.

Isolated torsion of the Fallopian tube, without ovarian torsion, is a rare cause of lower abdominal pain. We report our experience with 4 recent cases, along with data from a 20-year review of the pediatric literature.The records of 4 cases encountered during a 3-year period were reviewed. A literature review was completed by searching Medline, Medline in Process, Embase, Current Contents, and BIOSIS from 1990 to 2010.All 4 patients were diagnosed on laparoscopy. Laparoscopic salpingectomy was performed in 3 cases and laparoscopic detorsion in 1. All three resected specimens revealed hemorrhage and gangrene of the salpinx, with an associated cyst in one. The pediatric literature review, including our report, revealed 33 case reports and case series with 45 patients. Fifty-six percent of cases represented primary torsion, and 44% were secondary to underlying tubal pathology. Thirty percent of girls were premenarchal. The mean age at presentation and symptom duration were 13.2±2.1 years and 5.8±12.5 days, respectively. Fever and leukocytosis were present in 27% and 63%, respectively. Ultrasound, computed tomography scan, and magnetic resonance imaging showed a sensitivity of 22% (8/36), 14% (1/7), and 40% (2/5), respectively. A correct preoperative diagnosis was considered in only 13%. Eighty-eight percent of cases were treated by salpingectomy, and 12% were treated by tubal detorsion. Long-term outcomes of detorsion were not reported.Isolated salpingeal torsion in girls is rarely diagnosed preoperatively, regardless of imaging technique. Laparoscopy is the intervention of choice for definitive diagnosis and management. Salpingectomy is the most frequent treatment. Detorsion without resection may be considered for selected cases, but the long-term outcomes of this approach are unknown.

Biliary atresia is the most common cause of end-stage liver disease and liver cirrhosis in children, and the leading indication for liver transplantation in the paediatric population. There is no cure for biliary atresia; however, timely diagnosis and early infant age at surgical intervention using the Kasai portoenterostomy optimize the prognosis. Late referral is a significant problem in Canada and elsewhere. There is also a lack of standardized care practices among treating centres in this country. Biliary atresia registries currently exist across Europe, Asia and the United States. They have provided important evidence-based information to initiate changes to biliary atresia care in their countries with improvements in outcome. The Canadian Biliary Atresia Registry was initiated in 2013 for the purpose of identifying best standards of care, enhancing public education, facilitating knowledge translation and advocating for novel national public health policy programs to improve the outcomes of Canadian infants with biliary atresia.

Introduction The definition and treatment of gangrenous appendicitis are not agreed upon. We performed a prospective study in children to evaluate an objective definition of gangrenous appendicitis, as well as associated bacteriology, histopathology, and outcomes. Methods Five staff pediatric surgeons prospectively enrolled patients in the study at the time of appendectomy if the following five criteria were met: gray or black discoloration of the appendiceal wall; absence of fecalith outside the appendix; absence of visible hole in the appendix; absence of gross purulence or fibrinous exudate remote from the appendix; and absence of intraoperative appendiceal leak. Peritoneal fluid was cultured, and a standard histopathologic review was undertaken. Persistence of fever (>37.5°C) and ileus was documented daily. Patients were continued postoperatively on ampicillin, gentamicin, and metronidazole until they tolerated diet, manifested a 24-h afebrile period, and had a normal leukocyte count. Hospital stay, readmissions, and infectious complications were recorded. The study took place over a 12-mo period. Results Thirty-eight patients were enrolled, representing 17% of all patients with appendicitis treated during the year. Average age was 10.8 ± 3.5 y. Peritoneal cultures were positive in 53% of cases. Gangrene was documented histologically in 61% of specimens. Hospital stay was 3.2 ± 1.1 d. There were no postoperative infectious complications or readmissions related to the disease. Neither culture results nor histologic gangrene had a statistically significant effect on hospital stay. Conclusions An objective definition of gangrenous appendicitis is reproducible and has good histopathologic association. Recovery from gangrenous appendicitis is not influenced by culture or pathology results, and postoperative complications are rare. Limiting postoperative antibiotics to 24 h in gangrenous appendicitis may significantly decrease the cost of treatment without increasing morbidity.

Neonates with gastroschisis make up an increasing proportion of prolonged surgical NICU admissions. While infectious complications are known to increase patient morbidity, it is unclear whether they vary according to abdominal closure method, or can be predicted by initial patient assessment. A national, prospective, disease-specific database was evaluated for episodes of wound infection (WI) and catheter-related infection (CRI). Antibiotic use and timing, as well as method and location of abdominal closure were studied. The gastroschisis prognostic score (GPS) was calculated and evaluated as a predictor of infectious complications. Of 395 patients, 48 (12.6%) had a documented abdominal WI, and 59 patients (14.9%) had at least one episode of CRI-most commonly coagulase negative staphylococcus. Most abdominal closures took place within 6 h of admission (194 = 51.3%), while 132 (34.9%) were delayed greater than 24 h. The WI rate was greater in the delayed group (21.2 vs. 8.2%, p = 0.0006). The GPS was found to predict development of an infectious complication (WI + CRI, p = 0.04). Infectious complications remain an important consideration in the management of gastroschisis. GPS correlates with the development of infectious complications. Prophylaxis for skin flora and early closure, when feasible, may reduce WI rates.

Inguinal hernia repair (IHR) remains the most common procedure in pediatric surgery. Although postoperative sequelae are well described, we examined if prematurity and age were important determinants of postoperative complications.A retrospective review of children younger than 2 years undergoing IHR from 2004 to 2007 was performed, with a minimum of 1-year follow-up. Patients were segregated into groups based on age at diagnosis (A, 0-3; B: 4-26; C: 27-52; D: 53-104 weeks), with or without prematurity. Incarceration rates were investigated. Complications were categorized as major (vas injury, recurrence, testicular atrophy) or minor (wound infection, "high" testicle, hydrocele) and compared.Two hundred sixty-eight patients were analyzed (98 premature), with 14 major complications (5.2%) and 26 (9.7%) minor complications overall. Groups A and B accounted for more major (12/14) and minor complications (22/26) when compared with groups C and D (P.005). In patients less than 26 weeks (groups A and B), premature infants had more complications than term infants (27.7% vs 12.1%, P = .01). Of 22 patients with incarcerated hernias, 2 (9.1%) had major complications (P.5 vs nonincarcerated patients).Our study suggests that prematurity, rather than age at operation or incarceration, affects complication rates after IHR. This information should be used to frame the discussion of informed consent for this commonly performed procedure.

We present two patients who developed delayed fistulization following esophageal replacement surgery. The first is a 13-year-old child who, at the age of 3 years, underwent a trans-mediastinal colonic esophageal replacement for a refractory corrosive injury followed by a retrosternal reverse gastric tube after an early catastrophic leak. Ten years later, he presented with a history of intermittent chest pain for 6 months. He developed a tension pneumo-pericardial tamponade caused by a fistula between gastric tube and pericardium. He recovered after sternotomy. The second was born prematurely with type C esophageal atresia and other malformations. After esophageal anastomosis, he developed a refractory stricture that was resected at 10 months. Despite a fundoplication at 4 years, the recurrent esophageal stricture required resection at 14 years, accomplished by mobilizing the stomach into the chest through a left thoracoabdominal incision. The postoperative course was complicated by a gastric leak in the chest with empyema, but the patient recovered and was able to eat. Five years later, he underwent an anterior spinal fusion to correct a worsening kyphoscoliosis. Postoperatively, he developed an ARDS picture, leakage of air through the gastrostomy, and a fatal pulmonary hemorrhage secondary to a gastro-bronchial fistula. Fistulization from esophageal replacement surgery represents a rare long-term complication that pediatric surgeons need to be aware of.

Purpose Despite advances in neonatal care of congenital diaphragmatic hernia (CDH), a significant variation exists in the mortality rates reported by individual centers. Center experience (reflected by case volume) may contribute to this variation in outcome. The aim of the study was to determine whether CDH mortality is affected by hospital case volume. Methods The CDH cases were abstracted from a disease-specific, 16-hospital, national network. Thirteen hospitals participated in this study. Anonymized hospitals were categorized as either high (>6 cases) or low-volume (≤6 cases) centers (HVC, n=6; LVC, n=7) according to the median case number per center. Risk-adjusted (Score for Neonatal Acute Physiology, version II [SNAP-II] score) mortality rates were compared between HVC and LVC. Results One hundred twenty-one CDH cases were identified. Overall in-hospital survival was 81%. No significant difference in SNAP-II score was observed between HVC and LVC. Of 97 (15%) infants treated in 6 HVC, 15 (15%) died compared to 8 (33%) of 24 in 7 LVC ( P Conclusion Hospital case volume may be partially responsible for mortality rate variation in CDH. This result requires careful analysis, as case volume may merely be a surrogate for other predictive variables.

PEComa of the gastrointestinal tract, composed of perivascular epithelioid cells with myomelanocytic differentiation, is rare with previous literature limited to 16 case reports. There is a marked female preponderance and approximately one-third of the cases occur in the pediatric age group. We report PEComa with lymph node involvement occurring in the rectum of a 15-year-old girl, treated by surgical resection and adjuvant chemotherapy. The patient is well at 9 months follow-up with neither radiologic nor endoscopic evidence of recurrence. We review the differential diagnosis of intestinal PEComa, which includes malignant melanoma, epithelioid gastrointestinal stromal tumors, clear cell sarcoma of soft parts, alveolar soft part sarcoma, leiomyosarcoma with HMB45 expression, and paraganglioma. Immunohistochemistry can rule out many of these morphologically similar tumors but differentiation from clear cell sarcoma may require reverse transcription-polymerase chain reaction. We discuss the determination of pathologic features indicative of malignancy in PEComa, which is complicated in the gastrointestinal tract due to the small number of cases, variability of pathologic features reported, and inconsistent reporting of outcome. All 4 tumors reporting early recurrence or progression were greater than 5 cm in size and had areas of coagulative tumor necrosis. In addition, high nuclear grade and lymphovascular invasion were seen in 2 of these 4 cases. We propose that a minimum dataset for gastrointestinal PEComa should include these features along with mitotic count, infiltrative border, and tumor stage analogous to that used in colorectal carcinoma.

Management of gastroschisis varies. This study aims to determine which aspects of practice influence outcomes.All cases of simple gastroschisis (N = 99) in the Canadian Pediatric Surgery Network database were analyzed looking at methods of preoperative bowel protection, timing of closure, and closure techniques; and outcome measures included time to onset of enteral feeds, duration of parenteral nutrition (PN), and length of stay (LOS).One third of infants had initial bowel protection using a spring-loaded silo, which was significantly associated with a delay (beyond 24 hours) in establishing primary closure. Neither preoperative bowel protection methods nor defect closure techniques conferred any significant effects on success at establishing primary closure or functional outcomes. After adjusting for all covariates, only failure to establish primary closure was associated with impaired outcomes with significantly delayed initiation of enteral feeds and prolonged LOS. Low birth weight (2000 g) and younger gestational age (36 weeks) were associated with a 3-fold increased risk of longer PN dependence and 5-fold risk of extended LOS, respectively. Babies undergoing the sutureless spontaneous closure technique had significant delays in initiating enteral feeds but no increased requirements for PN or LOS.Modes of preoperative bowel protection and techniques of abdominal wall closure ultimately have no association with functional outcomes in infants with gastroschisis. Failure to establish primary closure, however, is significantly associated with delays in establishing intestinal function and subsequent time to discharge.

Pneumonia is an important clinical problem that affects children of all ages. Although effectively treated on an outpatient basis in the majority of cases, some children with respiratory infections still require hospitalization. This may be particularly true for patients with immunocompromise, for whom the lung represents the most common site of infection. Furthermore, respiratory infections represent a significant source of morbidity and mortality in this patient population. This article focuses on the clinical presentation, etiology, and treatment of childhood pneumonia, with special consideration given to the immunocompromised child. Two specific complications of pneumonia, lung abscess and empyema, are discussed.

Congenital diaphragmatic hernia (CDH) continues to be associated with significant mortality and morbidity rates despite advances in neonatal care. Fetal intervention for CDH has been studied for 25 years. After initial difficulties encountered with open fetal repair, attention has turned to tracheal occlusion (TO) as a method to correct pulmonary hypoplasia before birth. This article reviews our contribution to this field of research and outlines the current status of this treatment modality. Using the fetal lamb model, we have studied the effects of fetal TO on tracheal fluid pressure, lung growth and type II pneumocyte maturation, and surfactant production. We developed a minimally invasive and reversible technique of TO, using a detachable balloon placed using single-port tracheoscopy. We examined differential lung growth, structural maturation, pulmonary artery remodeling, and lung function during an 8-h resuscitation period in lambs, comparing normal controls, lambs with a surgically created CDH, those with CDH+TO, and those with CDH+TO and release of TO 1 week before delivery. We also studied the potential benefits of maternal betamethasone administration and the administration of surfactant at birth. Using a neonatal piglet model, we examined the effect of postnatal pulmonary distension with perfluorocarbon on lung growth. More recently, we turned to the rat nitrofen-induced CDH model to study the effects of TO on bronchial branching and some molecular markers of lung growth (Shh and LGL1). Fetal TO is being used to treat human CDH, but its application remains limited by the absence of reliable and widely reproducible prenatal prognostic criteria. A better understanding of the molecular events guiding the lung growth seen with TO may help to refine its use in humans.

Background/Purpose Cholestasis occurs frequently in patients with small bowel atresia (SBA) and is often attributed to prolonged parental nutrition. When severe or prolonged, patients may undergo unnecessary intensive or invasive investigation. We characterized cholestasis and analyzed the pertinence of investigating this patient population. Methods With Research Ethics Board approval, patients with SBA between 1996 and 2005 were retrospectively reviewed. Demographics, location of atresia, operative findings, complications, investigations, resumption of feeding, duration of prolonged parental nutrition, and follow-up information were examined. Cholestasis was evaluated for incidence, severity, and evolution. Results Fifty-five patients (29 male, 26 female), with a median gestational age and birth weight of 36 weeks and 2025 g, respectively, were reviewed. Care was withdrawn for 2 patients before repair. For the remaining 53 patients, SBA were duodenal atresia in 18, jejunoileal atresia in 32, and multiple atresia in 3. Of 53, 24 (45%) patients developed cholestasis postoperatively (direct/total bilirubin >20%). All patients with short bowel (4) and 60% (6/10) of patients with a delay of enteral feeding more than 14 days postoperatively had cholestasis. Ten patients (36%) proceeded with in-depth evaluations for cholestasis, with 8 (28%) undergoing liver biopsy. No patient had biliary atresia. No deaths were related to isolated cholestasis/cirrhosis. Cholestasis resolved spontaneously in all the survivors. Conclusions Small bowel atresia is frequently associated with postoperative cholestasis that will resolve with time. We recommend a more selective and expectant approach to SBA-associated cholestasis to minimize unnecessary investigations.

Fetal tracheal occlusion (TO) has been used to reverse the lung hypoplasia associated with congenital diaphragmatic hernia (CDH). However, TO has a detrimental effect on type II pneumocyte function and surfactant production. Previously, we have shown that in surgically created CDH lambs, TO improved markedly the response to resuscitation even though the lungs remain surfactant deficient. The goal of this investigation was to assess the effects of exogenous surfactant administered at birth to CDH lambs with or without fetal TO during 8 h of resuscitation. Lambs were divided into five groups: CDH, CDH+surfactant (SURF), CDH+TO, CDH+TO+SURF, and nonoperated controls. A left-sided CDH was created in fetal lambs at 80 d gestation. TO was performed at 108 d, and the lambs were delivered by hysterotomy at 136 d. Bovine lipid extract surfactant was administered before the first breath and again at 4 h of life. All CDH+SURF lambs, but only three of five CDH lambs, survived up to 8 h. When compared with the corresponding nonsurfactant-treated group, surfactant-treated CDH and CDH+TO lambs did not demonstrate improved alveolar-arterial oxygen gradients, pH, or Pco(2). In fact, in the CDH+TO group, surfactant treatment significantly worsened ventilation efficiency as measured by the ventilation efficiency index. The observed improvement in pulmonary compliance secondary to surfactant treatment was not significant. This investigation demonstrates that prophylactic surfactant treatment at birth does not improve gas exchange or ventilation efficiency in CDH lambs with or without TO.

Background/Purpose Short-duration resuscitation (≤4 hours) of lambs with diaphragmatic hernia treated in utero with tracheal occlusion have shown improved lung function compared with untreated diaphragmatic hernia. This may be a transient phenomenon in the treated diaphragmatic hernia lambs because of surfactant deficiency. Our objective was to analyze the effect of fetal tracheal occlusion with or without release of the occlusion 1 week before delivery on pulmonary function during a longer period of resuscitation (8 hours) in the diaphragmatic hernia lamb model. Methods Four groups were compared: diaphragmatic hernia (n = 5), diaphragmatic hernia and tracheal occlusion until delivery (n = 5), diaphragmatic hernia and tracheal occlusion with release of the occlusion 1 week before delivery (n = 5), and normal controls (n = 4). Results Despite persistently decreased surfactant levels, diaphragmatic hernia lambs treated with tracheal occlusion had normal-sized lungs with marked improvement in lung function and gas exchange over 8 hours when compared with untreated lambs with diaphragmatic hernia. Release of the tracheal occlusion 1 week before delivery added no benefit. Conclusions It appears that surfactant-independent mechanisms such as pulmonary growth and structural changes are of foremost importance in relating to improved compliance, oxygenation, and ventilation of diaphragmatic hernia lambs treated with tracheal occlusion.

Recent reviews of gastroschisis identify prematurity and low birth weight as predictors of morbidity and mortality. The authors compared the outcomes of intrauterine growth-restricted infants (IUGR) with gastroschisis to those without growth restriction because IUGR is different from prematurity.A retrospective analysis was performed for infants born with gastroschisis between 1990 and 2000 at 2 pediatric hospitals. Patients were segregated into 3 groups based on birth weight corrected for gestational age: group 1 (IUGR,fifth percentile), group 2 (fifth to 25th percentile), and group 3 (25th percentile). Patient demographics, method of closure, number of surgeries, presence of atresia, and time to full enteral feedings (FPO days) were assessed. Mortality rate, length of stay (LOS), and readmission rates were also compared. Analysis of variance (ANOVA)/Student's t test and Fisher's. Exact tests were used for statistical analysis (P.05 significant). Regression analysis was also performed.One hundred thirteen patients were included (group 1 = 17; group 2 = 43; group 3 = 53). Overall, infants with IUGR had similar outcomes to non-IUGR infants, including FPO and total parenteral nutrition (TPN) days, LOS, readmission, and mortality rates. The method of closure did not affect outcome. Infants with atresia had significantly increased times to full feeding (95 v 34 days; P =.034), more surgeries (2.7 v 1.4; P =.002), and longer LOS (106 v 48 days; P =.011). Infants born at less than 37 weeks' gestation had significantly increased fasting (NPO) days (28 v 18 days; P =.005) and longer LOS (65 v 37 days; P =.006) when compared with infants born at greater than 37 weeks. Logistic regression analysis identified the presence of atresia as an independent risk factor for gastrointestinal dysfunction and the need for prolonged TPN. Prematurity also adversely affected these same parameters, although it did not reach statistical significance.Although infants with gastroschisis are generally small for gestational age, the outcomes of growth-restricted infants are similar to those of other infants. The type of closure does not affect outcome, regardless of birth weight. The presence of atresia or prematurity does lead to longer times for full feeding and LOS. Therefore, routine premature delivery of infants with gastroschisis should not be advocated, even in the context of IUGR.

The aim of this study was to review pediatric spontaneous pneumothorax and describe diagnostic and therapeutic approaches and to review our institutional experience with spontaneous pneumothorax. A medline search using keywords pneumothorax and pediatric in both French and English from 1988 through May 2002 was performed, and pertinent papers regarding the natural history, diagnosis, imaging, and therapy were reviewed. A 10-year chart review of spontaneous pneumothorax recording data on demographics, diagnostic imaging, therapeutic approach, and outcomes was performed. Only 4 case series and one review article were produced by the medline search involving exclusively pediatric spontaneous pneumothrax. One hundred ninety-six other articles involved adult series with a minority of patients under 18 years of age. A subset of articles from this adult-based group were examined to assist in formulating recommendations for diagnosis, imaging, treatment, and surgical management of pediatric spontaneous pneumothorax. Few articles on this topic report randomized studies in the adult literature and none in the pediatric literature. The chart review yielded 53 cases of spontaneous pneumothorax in 40 patients with important data presented in the current review. Although few articles are able to provide best-evidence information, the diagnosis, imaging, treatment, and surgical management of patients with primary spontaneous pneumothorax is achieved with good results. Stronger data specific to pediatric patients are needed. Copyright 2003, Elsevier Science (USA). All rights reserved.

Background/Purpose: In 1990, the authors reported excellent outcomes using a standard protocol to treat pediatric appendicitis. This protocol has been simplified further and a large retrospective review was conducted to assess current outcomes. Methods: All patients treated for presumed appendicitis between April 1997 and December 1999 were reviewed. All patients received preoperative gentamicin and clindamycin. Patients with complicated appendicitis received postoperative ampicillin, gentamicin, and clindamycin or metronidazole. All wounds were closed primarily without drains. Patients with complicated appendicitis were discharged when their ileus resolved, they remained afebrile for 24 hours, and had a normal leukocyte count. Results: A total of 648 patients were reviewed. A total of 9.4% of appendices were pathologically normal, 55.6% were simple acute, 15.7% were gangrenous, and 19.3% were perforated. Hospital stay was 2.21 ± 2.04 days for normal, 1.39 ±.89 for simple acute, 2.97 ± 1.25 for gangrenous, and 6.31 ± 3.51 days for perforated appendices. There were no wound infections in patients with normal or simple acute appendices. Two minor intraabdominal infections (0.56%) occurred in patients with simple appendicitis. Patients with complicated appendicitis (gangrenous or perforated) had wound infection and intraabdominal infection rates of 2.6% and 4.4%, respectively. Conclusions: The authors' current protocol results in reasonable hospital stays and good outcomes. It serves as an evidence-based standard of care for the treatment of pediatric appendicitis. J Pediatr Surg 38:236-242. Copyright 2003, Elsevier Science (USA). All rights reserved.

Resume Le syndrome de Goltz est une dysplasie congenitale des tissus derives du mesoectoderme transmis sur le mode de l’heredite dominante liee a l’X par mutations du gene PORCN situe en Xp11.23. Les lesions cutanees, reparties en plages, peuvent etre associees a diverses malformations congenitales. Nous rapportons un cas de syndrome de Goltz chez 1 nouveau-ne de sexe feminin de race noire. Les lesions cutaneophaneriennes habituellement observees etaient presentes, caracterisees par leur preponderance a gauche avec des anomalies squelettiques ipsilaterales exclusives (fente labiopalatine, agenesie des metatarsiens, des orteils, syndactylie, absence d’une cote). Les conditions limitees de la prise en charge et la perception socioculturelle negative des malformations dans un pays en developpement n’ont pas favorise la survie de l’enfant.

Background: Third and fourth branchial remnants may result in cysts and abscesses that are in close contact with the thyroid gland. These anomalies are rare and often present diagnostic and therapeutic challenges. Methods: The charts of patients diagnosed with a branchial anomaly between July 1991 and July 2001 at the Montreal Children's Hospital were reviewed. All cases of third and fourth branchial remnants or pyriform sinus fistulae were identified. Clinical presentation, imaging, treatment, and outcome were recorded. Results: Eight patients with a third or fourth branchial anomaly were identified and ranged in age from birth to 13 years. All anomalies were left sided. Presenting symptoms consisted of an asymptomatic cervical mass (n = 1), an infected mass (n = 5), neonatal respiratory distress (n = 1), and 1 incidental cyst found on magnetic resonance imaging. Ultrasonography was useful in suggesting the diagnosis in 7 cases. Barium swallow was performed in 3 patients with 2 positive results. Pharyngoscopy results showed the internal opening in 2 of 7 patients. A portion of the thyroid gland was resected in 6 patients. One patient has not yet undergone a definitive procedure. There was 1 recurrence in a patient whose pathology did not confirm a branchial remnant. Conclusions: The diagnosis and management of pyriform sinus anomalies are challenging. Ultrasound scan, computed tomography scan, barium swallow, and pharyngoscopy are all useful. The portion of thyroid involved in the fistula must be excised en bloc with the inflammatory mass, and the tract should be ligated at the level of the pharynx to minimize recurrence. J Pediatr Surg 37:685-690. Copyright 2002, Elsevier Science (USA). All rights reserved.

Purpose: The aim of this study was to compare the proportion of operations for acute necrotizing enterocolitis (NEC) and post NEC strictures. Methods: The authors reviewed 195 charts of children referred to our institution for NEC or post-NEC strictures between 1990 and 1999. Seventy-one children were classified as Bell stage I and were excluded. The remaining 124 patients were classified as either Bell stage II or III and formed the basis of our study. These patients were subdivided into 2 groups: (1) group I (n = 69) comprised patients treated from 1990 until 1994 and (2) group II (n = 55) from 1995 until 1999. Statistical analysis consisted of [Chi ] 2 and Student's t tests. Significance occurred when P [le ] 0.05. Results: Both groups were similar with regard to sex, obstetrical history, indomethacin use, umbilical artery catheter use, and enteral feeding. The total operative rate for all patients with either acute NEC or post NEC strictures increased over time from 46% (32 of 69) in group I to 69% (38 of 55) in group II ( P [lt ] .01). Specifically, post-NEC stricture was the initial operation in 16% (5 of 32) of group I patients versus 37% (14 of 38) of group II patients ( P [lt ] .05). Subdividing each group by method of treatment of their NEC showed that medically treated patients had an increased incidence of stricture over time (group I, 15% v . group II, 48%; P [lt ] .01). Surgically treated children maintained a similar rate of stricture (group I, 36% v . group II, 33%). The mortality rate was comparable in both groups. Conclusions: At our institution, the total operative rate for necrotizing enterocolitis has increased over the last 10 years. This is because of 2 factors: (1) an increase in the percentage of stage III patients and (2) an increase in referrals for post[ndash ]necrotizing enterocolitis strictures. No specific criteria could be identified to predict which patients were at risk for post[ndash ]necrotizing enterocolitis strictures after medical treatment.

Background While fascial closure is traditionally used in gastroschisis (GS), flap closure (skin or umbilical cord) has gained popularity. We evaluated early outcomes and complications of the two techniques. Methods A national, population-based gastroschisis data registry was analyzed from 2005 to 2011. We compared fascial to flap closures and stratified patients into low or high-risk groups using the Gastroschisis Prognostic Score (GPS), a validated marker of post-natal bowel injury. Demographic and outcome data, including length of stay, complications, and markers of resource utilization were analyzed using Fisher’s exact and Student’s t-tests for categorical and continuous variables, respectively (p Results The analyzed dataset included 436 fascial closures (344 [78.8%] low-risk, 92 high-risk) and 129 flap closures (112 [86.7%] low-risk, 17 high-risk; p=0.06). Demographics and birth weight did not differ between groups. In patients with low GPS, flap closure demonstrated significant decreases in resource utilization and failure of closure, without differences in complication rates. Analysis of high-risk patients revealed no statistically significant differences in outcome. Conclusion Flap closure was not associated with an increase in patient morbidity and seemed suitable as a definitive closure method for gastroschisis patients irrespective of disease severity. Furthermore, flap closure reduced several markers of resource utilization in patients with low-risk disease.

Background Anastomotic stricture is the most common complication following repair of esophageal atresia. An Esophageal Anastomotic Stricture Index (EASI) based on the postoperative esophagram may identify patients at high risk of stricture formation. Methods Digital images of early postoperative esophagrams of patients undergoing EA repair from 2005 to 2013 were assessed. Demographics and outcomes including dilations were prospectively collected. Upper (U-EASI) and lower (L-EASI) pouch ratios were generated using stricture diameter divided by maximal respective pouch diameter. Score performances were evaluated with area under the receiver operator curves (AUC) and the Fisher's exact test for single and multiple (>3) dilatations. Interrater agreement was evaluated using the intraclass correlation coefficient (ICC). Results Forty-five patients had esophagrams analyzed; 28 (62%) required dilatation and 19 received >3 (42%). U-EASI and L-EASI ratios ranged from 0.17 to 0.70, with L-EASI outperforming the U-EASI as follows: L-EASI AUC: 0.66 for a single dilatation, 0.65 for >3 dilatations; U-EASI AUC: 0.56 for a single dilatation, 0.67 for >3 dilatations. All patients with an L-EASI ratio of ≤0.30 ( n =8) required multiple esophageal dilatations, p =0.0006. The interrater ICC was 0.87. Conclusion The EASI is a simple, reproducible tool to predict the development and severity of anastomotic stricture after esophageal atresia repair and can direct postoperative surveillance.