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This interactive session was held at the 51st Annual Meeting of the Canadian Association of Pediatric Surgeons (CAPS) in preparation for the transition of Pediatric Surgery training in Canada to Competency by Design (a CBME-based model of residency training developed by the Royal College of Physicians and Surgeons of Canada).

Boot camps seek to impart knowledge and skills for individuals entering new roles. We sought to evaluate knowledge, skills, and confidence of in-coming pediatric surgery trainees with a 2.5-day pediatric surgery boot camp.A curriculum included key aspects of pediatric surgery delivered during interactive lectures, small group discussions, and simulation. With REB approval, participant demographics were collected. Pre- and posttests assessed knowledge and trainee confidence. Comparative statistics and multivariate analysis of variance (MANOVA) were performed.Between 2017 and 2018, 16 individuals from North American pediatric surgery training programs participated in two boot camps. Ten had North American general surgery training, and eleven had no pediatric surgery exposure ≥1 year prior. All participants expressed increased confidence with course material after boot camp [F(18,11) = 3.137;p 0.05]. Performance improved significantly (pre- vs. posttests, 47.0% vs. 62.4%; p 0.05). MANOVA between faculty and trainees demonstrated agreement on the value of individual sessions [F(15,3) = 0.642;p = 0.76]. Neonatal bowel obstruction, gastrostomy tube complications, esophageal atresia, pain management, and informed consent were rated most useful.Trainees and teaching faculty considered the boot camp valuable. Trainees demonstrated significant improvements in core knowledge and confidence. The initial pediatric surgery boot camp experience shows promise in facilitating the transition to discipline for new trainees.Prospective treatment study.IV.

Preoperative assessment of surgical neonates often relates to issues of prematurity, low birth weight, or associated malformations. This review explores the preoperative cardiopulmonary evaluation in specific newborn surgical populations, the role of echocardiography in congenital diaphragmatic hernia perioperative management, the impact of bronchopulmonary dysplasia in the ex-preterm surgical neonate and a brief discussion on the risk of general anesthesia and specific anesthetic considerations for any surgical neonate. Newborns with congenital anomalies requiring early general surgical intervention should have an assessment for congenital heart disease. In the asymptomatic neonate, a thorough physical exam may be sufficient preoperatively. Neonates born with esophageal atresia or anorectal malformations should have a full evaluation for VACTERL associations. Initial echocardiography in congenital diaphragmatic hernia is used to evaluate anatomy, but there is emerging evidence to suggest the use of echocardiography in the ongoing surveillance of CDH to influence the timing of surgical intervention. Bronchopulmonary dysplasia is present in up to 40% of ex-premature neonates and increases the risk of postoperative apneas and need for ventilatory support. However, all surgical neonates have an increased risk of post-operative apneas, and the need for surgical intervention should be balanced with the risk of general anesthesia.

Objective: In infants with gastroschisis, outcomes were compared between those where operative reduction and fascial closure were attempted ≤24 hours of age (PC), and those who underwent planned closure of their defect >24 hours of age following reduction with a pre-formed silo (SR). Summary of Background Data: Inadequate evidence exists to determine how best to treat infants with gastroschisis. Methods: A secondary analysis was conducted of data collected 2006–2008 using the British Association of Pediatric Surgeons Congenital Anomalies Surveillance System, and 2005–2016 using the Canadian Pediatric Surgery Network. 28-day outcomes were compared between infants undergoing PC and SR. Primary outcome was number of gastrointestinal complications. Interactions were investigated between infant characteristics and treatment to determine whether intervention effect varied in sub-groups of infants. Results: Data from 341 British and Irish infants (27%) and 927 Canadian infants (73%) were used. 671 infants (42%) underwent PC and 597 (37%) underwent SR. The effect of SR on outcome varied according to the presence/absence of intestinal perforation, intestinal matting and intestinal necrosis. In infants without these features, SR was associated with fewer gastrointestinal complications [aIRR 0.25 (95% CI 0.09–0.67, P = 0.006)], more operations [aIRR 1.40 (95% CI 1.22–1.60, P < 0.001)], more days PN [aIRR 1.08 (95% CI 1.03–1.13, P < 0.001)], and a higher infection risk [aOR 2.06 (95% CI 1.10–3.87, P = 0.025)]. In infants with these features, SR was associated with a greater number of operations [aIRR 1.30 (95% CI 1.17–1.45, P < 0.001)], and more days PN [aIRR 1.06 (95% CI 1.02–1.10, P = 0.003)]. Conclusions: In infants without intestinal perforation, matting, or necrosis, the benefits of SR outweigh its drawbacks. In infants with these features, the opposite is true. Treatment choice should be based upon these features.

KEY POINTS Congenital diaphragmatic hernia (CDH), which occurs in about 1 in 3300 live births, is a congenital defect in the diaphragm that allows herniation of abdominal viscera into the thorax.[1][1] The resulting abnormal lung development leads to pulmonary hypoplasia and pulmonary hypertension

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.

The Canadian Pediatric Surgery Network (CAPSNet) has been collecting population-based data regarding congenital diaphragmatic hernia (CDH) across its 17 perinatal sites since 2005. With500 infants registered to date, CAPSNet has addressed many critical knowledge gaps pertaining to CDH care. Most importantly, it has identified variability in both CDH practice and outcome across Canada. Using the successful Evidence-based Practice for Improving Quality (EPIQ) method, CAPSNet is undertaking a national, multidisciplinary effort to standardize best practices for CDH, from prenatal diagnosis to hospital discharge, based on the best available evidence. The present article outlines the value of clinical research networks and the process CAPSNet will undertake to produce national consensus guidelines for CDH care.Le Réseau canadien de chirurgie pédiatrique recueille des données en population sur la hernie diaphragmatique congénitale (HDC) dans 17 sites périnatals depuis 2005. Puisque plus de 500 nourrissons y sont inscrits jusqu’à présent, le Réseau a corrigé de nombreuses lacunes liées aux soins de la HDC. Qui plus est, il a décelé la variabilité dans la pratique et les résultats de la HDC au Canada. Selon la méthode EPIC (un acronyme anglais qui signifie pratique fondée sur des données probantes pour améliorer la qualité), le Réseau déploie des efforts nationaux et multidisciplinaires pour normaliser les pratiques exemplaires en matière de soins de la HDC, du diagnostic prénatal au congé de l’hôpital, d’après les meilleures données probantes. Le présent article souligne la valeur des réseaux de recherche clinique et le processus que le Réseau entreprendra pour produire des lignes directrices consensuelles nationales sur les soins de la HDC.

Evaluate trends in method of access (percutaneous cannulation vs open cannulation) for pediatric extracorporeal membrane oxygenation and determine the effects of cannulation method on morbidity and mortality.Retrospective cohort study.The Extracorporeal Life Support Organization's registry was queried for pediatric patients on extracorporeal membrane oxygenation for respiratory failure from 2007 to 2015.None.Of 3,501 patients identified, 77.2% underwent open cannulation, with the frequency of open cannulation decreasing over the study period from approximately 80% to 70% (p0.001). Percutaneous cannulation patients were more commonly male (24.2% vs 21.5%; p = 0.01), older (average 7.6 vs 4.5 yr; p0.001), and heavier (average 33.0 vs 20.2 kg; p0.001). Subset analysis of patients on venovenous extracorporeal membrane oxygenation revealed higher rates of mechanical complications due to blood clots (28.9% vs 22.6%; p = 0.003) or cannula problems (18.9% vs 12.7%; p0.001), cannula site bleeding (25.3% vs 20.2%; p = 0.01) and increased rates of cannula site repair in the open cannulation cohort. Limb related complications were not significantly different on subset analysis for venovenous extracorporeal membrane oxygenation patients stratified by access site. Logistic regression analysis revealed that method of access was not associated with a difference in mortality.The proportion of pediatric patients undergoing percutaneous extracorporeal membrane oxygenation cannulation is increasing. Mechanical and physiologic complications occur with both methods of cannulation, but percutaneous cannulation appears safe in this cohort. Further analysis is needed to evaluate long-term outcomes with this technique.

A previously healthy 2-year-old boy presented to the emergency department with a decreased level of consciousness. A physical examination was unremarkable except for miosis and atypical limb movements. The patient underwent an extensive workup, including the search for metabolic, infectious, neurologic, and toxicologic etiologies. An abdominal ultrasound was performed because the child continued to remain neurologically impaired with no cause identified on other investigations. The ultrasound revealed a persistent uncomplicated ileoileal intussusception. The patient was taken to the operating room for surgical reduction. The child recovered fully postoperatively. This case illustrates the rare presentation of intussusception encephalopathy, which can be a diagnostic dilemma, especially when none of the symptoms of intussusception are present. Endogenous opioid poisoning is hypothesized to be the cause of the miosis and may hint at the diagnosis and aid in early management.

Objective This goal of this review was to examine the clinical evidence in support of commonly utilized measures intended to reduce complications following elective colorectal surgery. Data source Literature searches were performed to identify relevant studies from Medline, PubMed, and Cochrane databases. Study selection The American Pediatric Surgery Association Outcomes and Clinical Trials Committee selected eight questions to address this topic systematically in the context of three management areas: 1) appropriate utilization of systemic antibiotics for colorectal procedures, 2) reduction of stool burden through mechanical bowel preparation, and 3) intraluminal gut decontamination through use of enteral nonabsorbable antibiotics. Primary outcomes of interest included the occurrence of infectious and mechanical complications related to stool burden and intraluminal bacterial concentration (incisional surgical site infection, anastomotic leakage, and intraabdominal abscess). Results The evidence in support of each management category was systematically reviewed, graded, and summarized in the context of the review's primary outcomes. Practice recommendations were made as deemed appropriate by the committee. Conclusions Clinical evidence in support of interventions to reduce infectious complications following colorectal surgery is derived almost exclusively from the adult literature. High-quality evidence to guide clinical practice in children is sorely needed, as the available data may have only limited relevance to pediatric colorectal diseases.

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.

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 The incidence of sensorineural hearing loss (SNHL;>20dB loss) in CDH survivors is debated. We evaluated long-term audiological outcomes at a single tertiary care center with ECMO capability and an established neonatal follow-up program. Methods With REB approval, records of CDH survivors from 2000 to 2010 were retrospectively analyzed. Demographic, postnatal, and audiometric information was gathered. All underwent auditory brainstem response (ABR) or otoacoustic emissions screening before discharge and complete audiological surveillance. Thirty-three patients were evaluated to age 4+ years with others continuing follow-up. Results Forty-three patient records were reviewed with 1 excluded (transferred to another institution). Median GA and BW were 39weeks (35–41) and 3.1kg (2–4), respectively. Median ventilation days were 10 (2–189) with 34 infants ventilated 5+ days. Sixteen (36%) received HFOV, 21 (49%) iNO, and 5 (12%) ECMO. The median time to CDH repair was 3days (1–23), and 11 (26%) required patch repair. Nine infants (21%) received diuretics and oxygen after discharge. Audiological surveillance identified only one patient with SNHL (received HFO, iNO, and patch repair). Conclusion Neonatal screening identifies CDH survivors at risk for hearing difficulties but must be followed with comprehensive testing until school age. The incidence of SNHL may be less than previously reported in this population.

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.

The Canadian Pediatric Surgery Network (CAPSNet) was created in 2005 by a geographically representative, multidisciplinary group of clinicians and researchers with the intent of establishing a national research registry for gastroschisis (GS) and congenital diaphragmatic hernia (CDH). Since then, CAPSNet has used this registry and its 16-center network to make contributions to the knowledge base informing best practices for GS and CDH care. More recently, CAPSNet has expanded its focus to include quality assurance and improvement at each of its sites, by issuing a benchmarked outcomes "report card" with its annual report. Finally, a major objective of CAPSNet has been to establish and adopt standardized, evidence-based practice guidelines for GS and CDH across all Canadian perinatal centers.

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.

Introduction Continuous positive airway pressure (CPAP), used either alone or associated with heliox (CPAP-He), has become a popular therapeutic option for bronchiolitis. This systematic review assesses the impact of CPAP on endotracheal intubation, carbon dioxide pressure (PCO2) and respiratory distress in patients with bronchiolitis. Methods Systematic search including studies that used CPAP or CPAP-He in infants with bronchiolitis admitted to a PICU. Data analysis included descriptive statistics and the GRADE system. Results Five CPAP (one crossover randomized controlled trial [RCT] and four before–after studies) and three CPAP-He (one quasi-RCT and two before–after) studies were included. CPAP was reported to reduce PCO2 (−6.9 to −11.7 mmHg, respectively, P

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.

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.

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.

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.

Purpose Increasing numbers of programs participating in the pediatric surgery match has resulted in economic and logistical issues for candidates, General Surgery residencies, and Pediatric Surgery training programs (PSTP). We sought to determine the ideal number of interviews conducted by programs based on resultant rank order lists (ROL) of matched candidates. Methods PSTPs received 4 online surveys regarding interview practices (2011–2012, 2014), and matched candidate ROL (2008–2010, 2012, 2014). Program directors (PD) also provided estimates regarding minimum candidate interview numbers necessary for an effective match (2011–2012, 2014). Kruskal–Wallis equality-of-populations rank tests compared ROL and interview numbers conducted. Quartile regression predicted ROL based on the interview numbers. Wilcoxon signed rank-sum tests compared the interview numbers to the minimal interview number using a matched pair. p Values Results Survey response rates ranged from 85–100%. Median ROL of matched candidates (2–3.5) did not differ between programs (p=0.09) and the lowest matched ROL for any year was 10–12. Interview numbers did not affect the final candidate ROL (p=0.22). While PDs thought the minimum median interview number should be 20, the number actually conducted was significantly higher (p Conclusion These data suggest that PSTPs interview excessive numbers of candidates. Programs and applicants should evaluate mechanisms to reduce interviews to limit costs and effort associated with the match.

The management of pulmonary hypertension is multi-faceted, with therapies directed at supporting cardiovascular and pulmonary function, treating the underlying cause (if feasible), and preventing irreversible remodeling of the pulmonary vasculature. Recently, manipulation of signaling pathways and mediators contained within the pulmonary vascular endothelial cell has become a new target. This article will review the pathophysiology of pulmonary hypertension and the broad principles involved in its management, with specific emphasis on pharmacological therapies directed at the pulmonary vascular endothelium.

Purpose The purpose of this study was to compare RCDH to LCDH from the perspective of prenatal diagnosis, illness severity, treatment, and outcome. Methods A retrospective study of all cases of CDH registered in the Canadian Pediatric Surgery Network (CAPSNet) database from 2005 to 2013 was conducted. Side of defect comparisons were made by prenatal diagnostic features, birth demographic data, intensity of medical treatment, timing and type of surgery, and outcomes. Outcomes prediction with logistic regression modeling using side of defect as an exploratory covariate was performed. Results The study cohort included 498 patients, of which 84 (17%) cases had RCDH. Prenatal diagnosis was more commonly made for LCDH. No difference existed in perinatal risk factors (GA, illness severity (SNAP-II) score, associated anomalies), preoperative treatment intensity (use of vasodilators, inotropes), timing of surgery, ventilation days, need for ECMO, LOS, and overall survival. Significant differences between RCDH and LCDH were detected for patch repair rate (48.2% vs. 30.6%; p =0.036) and recurrence (4.1% vs. 0.6%; p =0.038). Stepwise regression modeling identified side of hernia as independently predictive of need for patch. Conclusions Overall, little difference exists between RCDH and LCDH in terms of prognostic factors and outcomes.

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 CanMEDS competency "scholar" encompasses the creation, dissemination, application, and translation of medical knowledge. We hypothesize that a structured journal club (JC) for pediatric surgical trainees would meet these objectives in an enjoyable and long-lasting manner.A JC involving two pediatric surgery training programs was created with each session focusing on a specific study design. Pre-tests/post-tests were administered before/after each session with durability of learning assessed during the following session. Metrics analyzed included participant satisfaction and an appraisal of evidence-based medicine (EBM) principals. Test results were analyzed using the paired T-test with p0.05 considered significant.On average, 14 participants attended each session, with all trainees present (4). While participants believed they understood EBM principles, 40% were unfamiliar with question formulation, 48% were unfamiliar with critical appraisal tools, and 60% had not appraised an article within the previous year. Pre-test to post-test comparison yielded an improvement in mean score (20=perfect score): 10.8 to 16.9, p0.01. Measures of participant satisfaction were uniformly positive.A structured Pediatric Surgery Journal Club addresses scholarly training objectives in a highly satisfactory manner and yields durable learning. A web-based curriculum based on this model could serve as an important educational tool for trainees and attending staff alike.

The efficacy of exogenous surfactant administration is influenced by numerous factors, which has resulted in variable outcomes of clinical trials evaluating this treatment for the acute respiratory distress syndrome (ARDS). We investigated several of these factors in an animal model of acid aspiration including different surfactant preparations, and different delivery methods. In addition, high-frequency oscillation (HFO), a mode of mechanical ventilation known to recruit severely damaged lungs, was utilized. Lung injury was induced in adult rabbits via intratracheal instillation of 0.2 N HCl followed by conventional mechanical ventilation (CMV) until Pa(O2)/FI(O2) values ranged from 220 to 270 mm Hg. Subsequently, animals were given one of three surfactants administered via three different methods and physiological responses were assessed over a 1-h period. Regardless of the surfactant treatment strategy utilized, oxygenation responses were not sustained. In contrast, HFO resulted in a superior response compared with all surfactant treatment strategies involving CMV. The deterioration in physiological parameters after surfactant treatment was likely due to overwhelming protein inhibition of the surfactant. In conclusion, various surfactant treatment strategies were not effective in this model of lung injury, although the lungs of these animals were recruitable with HFO, as reflected by the acute and sustained oxygenation improvements.

Spinal cord complications are rarely encountered in elective pediatric surgery. We present a patient who became paraplegic after resection of a chest wall tumor. After neoadjuvant radio- and chemotherapy, a 10-year-old girl with primitive neuroectodermal tumor (PNET) of the right chest underwent a right chest wall resection. Ribs #3, 4, 5, and 6 were resected en masse with a pulmonary wedge resection of right upper and middle lobes. To obtain clear margins, the ribs were disarticulated from the vertebral column. Significant bleeding arose when the fourth rib was detached. Presuming an intercostal vessel bleed, the area was packed with surgicel with resolution of the bleeding. The patient was kept sedated and ventilated in the PICU. The next day, she complained of paresis of her lower extremities. MRI revealed compression of the spinal cord at the T4 level. Emergency decompression and laminectomy was performed. Blood clot and surgicel were found in the area of the spinal canal. Giant epidural veins were encountered as well, again with massive bleeding. The patient never regained neurological function below the insult to the cord. Despite negative margins and ongoing chemo- and radiotherapy, her tumor recurred and she died to her disease less than 1 year later. Chest wall tumors arising near the spinal canal may be associated with enlarged Batson's plexus that may hemorrhage during surgical resection. We recommend immediate imaging or prophylactic laminectomy for any concerns of spinal hemorrhage. Patients should also receive regular assessment of neurovitals, despite issues of pain control and sedation.

Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignant tumor that can be associated with cystic lung lesions in children. This neoplasm is histologically characterized by primitive blastoma and a malignant mesenchymal stroma. The authors describe a 3-year-old boy who presented with a history of fever and cough. Radiological imaging demonstrated a large cystic lesion replacing the left lower lobe. The patient underwent thoracoscopic resection of the lesion. Interestingly, the histopathology demonstrated a type 1 PPB. Type 1 lesions are usually observed in young infants, whereas older infants and children tend to present with type 2 or 3 PPB, which carry a poorer prognosis and higher risk of recurrence. Thus, the presence of large or peripherally based lung cysts should raise the suspicion of PPB. Resection is warranted for all such lesions.

A previously healthy, fully immunized, 14 kg four-year-old girl presented to the local emergency department with a seven-day history of upper respiratory tract symptoms and 24 h of fever, irritability and decreased oral intake. Her temperature on presentation was 38.9°C, heart rate 120 beats/min, respiratory rate 25 breaths/min, and blood pressure 90/60 mmHg. She was alert but irritable on physical examination, mildly volume-depleted and had evidence of meningismus. Her electrolytes were normal, notably a serum sodium concentration of 138 mmol/L, and there was pleocytosis of the cerebrospinal fluid. The patient was admitted with a working diagnosis of bacterial meningitis, given a 20 mL/kg normal saline bolus and commenced on intravenous (IV) vancomycin and ceftriaxone. She was started on maintenance IV fluids, consisting of dextrose 3.3% in NaCl 0.3% (‘2/3 – 1/3’) with KCl 20 mmol/L at 50 mL/h. She was also permitted to drink oral fluids as tolerated. Twenty-four hours later, the child’s irritability improved; however, she was noted to have headache and nausea. She was assessed by the paediatrician who reassured the family that these symptoms were consistent with meningitis. Her urine output was noted to be adequate, though it was not specifically quantified. Electrolytes were not repeated. That evening (36 h following admission), the patient was noted to be increasingly lethargic and subsequently had a generalized seizure. She was managed with IV lorazepam, followed by a phenobarbital load of 20 mg/kg. Stat electrolytes requested at the onset of the seizure revealed hyponatremia ([Na+]: 122 mmol/L). Hypertonic saline (3% NaCl) was administered, and arrangements were made for transfer to a tertiary care centre. En route she suffered a respiratory arrest, requiring intubation and ventilation. The child progressed to cardiac arrest, with unsuccessful resuscitation. In addition to evidence of meningitis (pathology and cultures were positive for Neisseria meningitidis, serotype B), the autopsy revealed severe cerebral edema with secondary brainstem herniation believed to be caused by the acute hyponatremia. Note: For privacy reasons, elements from published cases were used to construct this vignette (1,2). LEARNING POINTS Paediatric hyponatremia cases have been reported in the literature, the majority of which documented the use of hypotonic IV fluids (1).Though severe cases are infrequent, their devastating outcomes have prompted the emergence of recent research and evidence for safe IV fluid practices in children. Acute hyponatremia is defined as a fall in serum sodium concentration from normal to

Purpose Neonates with necrotizing enterocolitis (NEC) receive numerous radiologic investigations that potentially increase their lifetime cancer mortality risk. We evaluated our radiologic practice pattern for patients with NEC and estimated cumulative radiation exposure and lifetime cancer risk. Methods Infants with NEC in a tertiary care NICU had patient demographics, imaging, treatments/interventions, and outcomes analyzed over 3years. The number and type of imaging were recorded, including NEC-related imaging (thoraco-abdominal "babygrams" and abdominal radiographs), and all other imaging modalities. Patients were stratified by birth weight: group 1 ( 1501g). Pre-existing normative data were used to calculate radiation exposure, absorption, and attributable cancer risk from NEC-related imaging. Results Sixty-four neonates with 72 episodes of NEC were identified. Overall survival was 75.0%. When stratified by birth weight, mean abdominal radiographs and babygrams comprised 51%, 60%, and 74% of total imaging, giving median mGy doses of 2.1, 0.4, and 0.2, respectively. Compared to normative data, radiation dosing, and median cumulative cancer lifetime mortality risk increased by an average of 4.3× from baseline, with two cases documenting a 20-fold increase. Conclusion Neonates with NEC are exposed to significant amounts of radiation directly attributable to disease surveillance. Non-radiologic surveillance methods could significantly reduce radiation exposure and cancer risk in these infants.

Confusion, controversy, and uncertainty are all terms applicable to the diagnosis and management of congenital lung lesions both prenatally and postnatally. This review examines the current status of fetal diagnosis and treatment of these lesions; reviews the various classifications, including congenital cystic adenomatoid malformation/congenital pulmonary airway malformation, sequestrations, variants and hybrid lesions; discusses the risk of malignant transformation or misdiagnosis with pleuropulmonary blastoma; presents the arguments in favor and against resection of asymptomatic lesions, the timing of such resection, and the long-term pulmonary function after resection; and reviews the experience with thoracoscopic resection of congenital lung lesions.

Background/Purpose Disease-specific outcome predictors are required for gastroschisis. We derived and validated a gastroschisis prognostic score (GPS) based on bowel appearance after birth. Methods Visual scoring of bowel matting, necrosis, atresia, and perforation generated a novel gastroschisis bowel injury score recorded in a national database. Reweighting of score components by regression analysis led to assessments of model calibration and goodness of fit. The GPS was validated in subsequent cases. Results Records from 225 infants were used for model derivation. Only intestinal necrosis independently predicted mortality by regression analysis (odds ratio, 11.5; 95% confidence interval, 4.2-31.4). Model recalibration identified that a GPS of 4 or more predicted mortality in 75% of nonsurvivors and 99% of survivors ( P = .0001). A GPS of 2 or more demonstrated significantly worse survival outcomes compared with scores of 0 or 1 (length of stay: P = .011, days to first enteral feed: P = .013, days on total parenteral nutrition: P = .006). Model validation with 184 new patients yielded continued high-quality discrimination of outcomes. The GPS demonstrated "near-perfect" interobserver reliability between 2 surgeons ( κ ≥ 0.86). Conclusions The GPS allows the accurate and reliable identification of high-risk groups for mortality and morbidity based on bowel appearance at birth. This information can drive discussions regarding family counseling, resource allocation, and new therapies for these patients.

Some centers advocate using antibiotics before enema reduction to prevent septic complications. Our objective was to determine whether using antibiotics before reduction provided any improvement in outcomes.With institutional review board approval, patients from 2 centers were compared: 1 where antibiotics were administered, and one where they were not. This retrospective cohort study from January 2005 to December 2010 evaluated demographic data, episodes of postreduction fever, hospital stay, and analgesia requirements.One hundred eighteen patients were identified: 83 males (70.3%) and 35 females (29.7%). The median age was 24 months (range, 1-180). Fifty-six patients (57.7%) received antibiotics, whereas 41 (42.7%) did not. Twenty-one patients (17.8%) had missing data and were excluded. The incidence of fever postreduction was not statistically different between groups: 12.8% for those who received antibiotics vs 17.9% for those who did not (P = .7367). No adverse antibiotic reactions were reported. Average time to oral feeds was 7.3 vs 10.6 hours (P = .06), and the length of stay was 1.7 vs 1.4 days (P = .07).Although antibiotics are administered routinely in some centers, they appear of little value. Financial costs and potential adverse reactions must be considered. Further prospective evaluation is being conducted using a larger sample size to confirm these results.

Background/Purpose Traditionally, basic science research and publication record have led to a successful Pediatric Surgery Match. With changing applicant research backgrounds, we evaluated if these or other factors still apply. Methods A SurveyMonkey questionnaire was distributed to 57 applicants with known contact information. We assessed demographic/financial data, application details and match results, research experience, publications, presence of a pediatric surgery fellowship at their home program, and applicant ranking criteria. Results Forty-three (75%) responses were received. Twenty-five candidates matched, 12 (48%) to 1 of their first 3 choices. The median number of programs applied to was similar for matched and unmatched candidates (30), but matched candidates attended more interviews (21 vs 14.5; P = .03). Matched applicants had more publications (9.5 vs 5.1; P = .03), although research experience was similar to unmatched candidates. Research focus for matched vs total applicants included basic science (5 vs 12), clinical (4 vs 6), and both (11 vs 16). Five candidates matched without research experience. Ten (40%) applicants matched to institutions where they completed residency/research/fellowship training. Twelve (49%) applicants matched from programs without a fellowship program. Conclusion A strong publication record remains important, although clinical research is being valued more. Candidates from nonfellowship programs can be successful. This information may be useful to mentor future applicants and lays the foundation for a critical evaluation of the match process.

Background Perinatal management of congenital diaphragmatic hernia (CDH) and gastroschisis (GS) remains nonstandardized and institution specific. This analysis describes practice and outcome variation across a national network. Methods A national, prospective, disease-specific database for CDH and GS was evaluated over 4 years. Centers were evaluated individually and defined as low (low-volume center [LVC]) or high (high-volume center [HVC]) volume based on case mean. Results Congenital diaphragmatic hernia . Two hundred fifteen liveborn cases were studied (mean, 14.3 cases/center) across 15 centers (8 LVCs and 7 HVCs). Significant interinstitutional practice variation was noted in rates of termination (0%-40%) and cesarean delivery (0%-61%). Centers demonstrated marked variation in ventilation strategies, vasodilator and paralytic use, timing of surgery, and rates of primary closure. Overall survival was 81.4% (LVC, 76.9%; HVC, 82.4%; P = .43). Gastroschisis . Four hundred sixteen cases were investigated (mean, 26 cases/center; range, 6-72) across 16 centers (10 LVCs and 6 HVCs). Cesarean delivery rates varied widely between centers (0%-86%) as did timing of closure (early vs delayed, 1%-100%). There was no difference in length of stay, days on total parenteral nutrition, and overall survival (94.3% vs 97.2%; P = .17) between LVCs and HVCs. Conclusions The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis.

Background/Purpose Fetal tracheal occlusion (TO) has been investigated as a treatment option for lung hypoplasia secondary to congenital diaphragmatic hernia. Tracheal occlusion has been shown to accelerate lung growth, but its effect on bronchial branching is unknown. In this study, we characterize the effects of in vitro TO on bronchial branch development in fetal lung explants derived from the nitrofen rat model of congenital diaphragmatic hernia. Methods Rat dams were gavaged nitrofen on gestational day 9.5, and fetal lungs were harvested for explant culture on gestational day 14 (term, 22 days). Four experimental groups were investigated, with TO performed ex vivo using cautery: control, control + TO, nitrofen, and nitrofen + TO. Explants were incubated for 72 hours. Representative photographs were taken at 0, 24, 48, and 72 hours from the time of culture, and the number of distal branches was counted for each explant. The Student t test was used to compare distal branch measurements. Results A minimum of 12 fetal lung explants were cultured for each group. By 24 hours, all explants undergoing TO had more branch iterations than explants that did not. Moreover, TO in nitrofen-exposed explants increased bronchial branching to control levels by 24 hours in culture. Conclusion Our results suggest that TO at day 14 increases branching in normal and nitrofen-exposed lung explants. In addition, TO increases airway branching in nitrofen-exposed explants to control levels suggesting that early TO reverses the lung hypoplasia seen in this model.

Background The treatment of northern aboriginal children (NAC) is often complicated by distance from a treating facility. We sought to compare outcomes of NAC requiring transfer with appendicitis to those who presented locally. We hypothesized that NAC with appendicitis experienced higher rates of perforation and increased length of stay (LOS). Methods A retrospective chart review of 210 appendectomies was performed. Charts were reviewed for age, sex, weight, days of symptoms before presentation, time of transfer, leukocyte count (white blood cell count), usage of antibiotics prior to transfer, time to operation, type of procedure and findings, pathology, postoperative outcomes, and LOS. Results Sixty-eight children were NAC, whereas 142 were local. The average transfer times for NAC was 10 hours (range, 4-20 hours). The two groups had similar ages (11.1 vs 10.7 years), time to presentation (1.64 vs 1.85 days), and LOS (2.91 vs 2.90 days). Significantly higher perforation rates (44 vs 28%; P = .02), higher white blood cell count (17.9 vs 16.0; P = .02), and longer times to operation after arrival (10.3 vs 7.0 hours; P = .0002) were noted in NAC. Postoperative complications were similar between groups. Forty-seven (69%) NAC received antibiotics prior to transfer, which did not affect rate of rupture. Conclusion NAC with appendicitis experience longer transfer times and higher perforation rates than local children without a difference in length of stay or complications. Pretransfer antibiotics do not reduce perforation rates but may impact complications. We endorse their use if a delay in transfer is anticipated.

Background/Purpose The management of neonates with giant omphalocele remains challenging and multiple strategies have been described. We present the case of a 34-week-old neonate with isolated giant omphalocele managed with an external surgical skin closure system as a component of a staged closure strategy. Case Presentation An Inuit boy of 34 weeks gestation was born by urgent Caesarean delivery at an affiliated obstetrical hospital with a giant ruptured omphalocele and loss of abdominal domain. He was transferred to our institution and a silastic silo was fashioned and placed in the operating room. He returned to the operating room several times and was treated by placement of a combined Gore-Tex (WL Gore and Associates, Flagstaff, Ariz)/silastic inlay mesh. An eschar formed over this temporary closure, and we elected to place a dynamic skin closure device to continue gradual bedside reduction. The initial abdominal wall defect was 8.5 cm in transverse diameter and was reduced to 4.5 cm over 3 weeks. Complete closure was subsequently achieved without the need for skin grafting. Discussion The use of a dynamic reduction skin closure device has not been documented previously in the pediatric population or in the context of a congenital defect. We describe the use of an external surgical skin closure device in the context of the staged closure of a giant neonatal omphalocele and postulate that such a device may prove useful in the treatment of other congenital tissue defects.

Most surgeons agree that complex neonatal ovarian cysts, regardless of size, warrant operative intervention. Management of simple cysts >4 cm is still controversial, although many favor intervention because of the increased risk of torsion. Whereas laparoscopic cyst resection is favored by some, others prefer less invasive percutaneous needle aspiration. We present a newborn infant who was admitted with sepsis and respiratory failure after home delivery. Ultrasound done on day 8 to check for umbilical venous line placement incidentally showed a simple cyst measuring 3.6 × 5.9 × 6.9 cm that was presumed to be of ovarian origin. Percutaneous needle aspiration was atraumatic and revealed serous fluid, with a high estradiol level. Four days later, surgery was indicated for clinical deterioration with suspected hemorrhage into the cyst. We found a midgut volvulus with extensive necrosis secondary to a jejunal duplication cyst. Ovaries were normal and there was no evidence of malrotation. Postoperatively, after discussion with the parents, support was withdrawn and the child died. We should not rely solely on ultrasonographic features and fluid characteristics to diagnose a large neonatal abdominal cyst, but rather confirm the diagnosis with laparoscopy.

Background/Purpose: Spindle epithelial tumor with thymus-like elements (SETTLE) is a rare tumor of the thyroid observed in children and adolescents. We present a case series of 3 patients with SETTLE, focusing on the clinical and pathologic features of this rare tumor. Methods: Three male patients presented at ages 4.5, 6.5, and 7 years with a right thyroid mass. All were treated by standard hemithyroidectomy. None had evidence of distant metastases at presentation. The diagnosis of SETTLE was confirmed at the time of the initial operation in 2 of the 3 patients. Results: All patients had uneventful postoperative courses. Two patients remain disease-free 4 and 7 years postresection, respectively. One patient presented 10 years after resection with shortness of breath and hemoptysis secondary to multiple bilateral parenchymal lung metastases. This patient received chemotherapy against the epithelial components of the tumor with a 25% response based on imaging studies. Conclusion: Spindle epithelial tumor with thymus-like elements is rare tumor that should be suspected if spindle elements are observed in the resected thyroid specimen. Because these patients may present with delayed metastases, follow-up is recommended. However, chemotherapy against specific tumor elements is only marginally effective.

The timing and need of resection of asymptomatic congenital lung lesions are controversial. The morbidity of such surgery needs to be considered in the decision analysis. We analyzed the contemporary outcome of infants and neonates undergoing lung resection.With institutional review board approval, all patients 12 months or younger undergoing lung resection between 1995 and 2004 in 2 hospitals were reviewed. Demographic data, indications for surgery, operative procedure, complications, use of regional anesthesia, length of stay (LOS), and follow-up were assessed.Forty-five patients (28 male, 17 female) with a median age of 4 months (2 days-12 months) were evaluated. Congenital lesions (42) were the most frequent indication for surgery. Twenty-two (48.9%) patients had cardiorespiratory symptoms or infection preoperatively. Lobectomy was the most common operation (40/45). Three patients had intraoperative difficulty (bleeding, hypotension, desaturation). Significant postoperative complications occurred in 7 patients: prolonged air leak or chest tube drainage (4), anemia or bleeding (2), respiratory distress requiring reintubation (1). Fewer complications occurred in asymptomatic vs symptomatic patients (1/23 vs 6/22). Of 12 patients, 7 (58%) requiring 24 hours of ventilation or longer were 3 months or younger. Increasing age did significantly influence the chance of successful extubation (P = .01; odds ratio, 1.5; 95% confidence interval, 1.0-2.0), as did the use of epidural anesthesia (P.001). Median LOS was 6 days (2-89 days). Asymptomatic patients had shorter LOS (median, 4 days; range, 2-20 days; P = .024) vs symptomatic patients (median, 8 days; range, 4-89 days). The only death occurred from underlying heart disease. Mean follow-up at 35 months (12-132 months) revealed no subjective reduction in cardiopulmonary function.Lung resection is safe and well tolerated in infancy. Surgery should be scheduled before the development of symptoms but likely after 3 months of age to improve the chances of postoperative extubation. The use of regional anesthesia may facilitate this.

Purpose Gastroesophageal reflux (GER) is observed in 22% to 81% of neonates with congenital diaphragmatic hernia (CDH). The purpose of this study was to identify factors that may predict GER requiring fundoplication in neonates with CDH. Methods A retrospective chart review was performed on all neonates with CDH treated at our hospital from June 1997 to June 2005. Preoperative respiratory status, side of the CDH, and method of repair were assessed as predictors of GER and the need for fundoplication. Results Of the 42 patients with CDH, 3 died before intervention, leaving 39 patients eligible for study. All but 1 patient survived until discharge. Twenty-one (54%) developed GER of whom 9 (23%) required fundoplication. Although the side of the CDH was not a determinant of GER or the need for fundoplication, patch repair and the need for extracorporeal life support were determinants of both. Conclusions Gastroesophageal reflux is common among babies with CDH, although symptoms often resolve without surgical intervention. Infants with CDH defects requiring a patch repair and those requiring advanced physiologic support, especially extracorporeal life support, are likely to develop severe GER necessitating fundoplication. Early recognition and treatment of GER among high-risk patients may shorten hospital stay and minimize patient morbidity. Early fundoplication should be considered for those patients at the highest risk.

Background Fetal tracheal occlusion (TO) has been investigated as a treatment option for lung hypoplasia secondary to congenital diaphragmatic hernia (CDH). TO increases lung size, but it is unclear whether TO stimulates mature lung growth or simply induces alveolarization without concomitant bronchial development. In this study, we characterize bronchial branch development in fetal rats with CDH with or without TO through conventional histological and morphometric analysis as well as lung casting. Materials and methods Rat dams were gavaged nitrofen at gestational day 9.5, and 3 to 4 fetuses per dam underwent fetal TO on gestational day 19 (term = 22 days). Fetuses were sacrificed on day 21, the presence of CDH was confirmed, and the lung weight to body weight ratio (LW/BW) was calculated. Lung casts of all research groups were created using liquid silicon and bronchial branches were quantified from lung periphery to carina. Results CDH fetuses had smaller LW/BW ratios and a lesser percentage (%) of airspace when compared to controls, and manifested less lung branching than controls. Fetuses treated by TO had a greater LW/BW ratio and % airspace, but did not have a different number of branch iterations. Fetuses with CDH and TO demonstrated a restoration in LW/BW ratio to control levels ( P = 0.42), but the number of bronchial branch iterations remained less than control animals. Conclusion The results of this study suggest that TO in this animal model at gestational day 19 promotes distal airway proliferation but does not reverse the underdevelopment of bronchial branching seen in lung hypoplasia due to CDH.

Objective Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare mullerian anomaly consisting of uterine didelphy, hemivaginal septum, and ipsilateral renal agenesis. The purpose of this study was to evaluate the natural history and outcome of patients with HWWS. Methods With ethics review board approval, all patients with uterine/vaginal anomalies were reviewed between 1982 and 2004. Patients with cloacal and/or anorectal anomalies were excluded. Presenting symptoms, preoperative investigations, operative management, and long-term follow-up were assessed. Results Of 80 patients identified with uterine/vaginal anomalies, 12 had HWWS. Median age at presentation was 13 years. Most patients (11/12) in this series presented with either abdominal pain and/or pelvic masses. Two patients had intra-abdominal abscesses. Seven patients were menstruating at presentation with 4 of these patients having dysmenorrhea. Symptom duration ranged from 0.5 to 12 months. Diagnosis was confirmed by ultrasound (n = 11), computed tomographic scan (n = 3), and/or magnetic resonance imaging (n = 2). Operative management included vaginal septectomy and drainage of the hematocolpos/hematometrocolpos. One patient required salpingectomy for pyosalpinx. Follow-up ultrasounds revealed no recurrent collections. Median follow-up was 3 years (2 months to 16 years). Eleven patients were asymptomatic after treatment. One patient complained of irregular menses. Conclusion This is one of the largest reviews of HWWS in pediatric patients to date. Good long-term outcome occurs after vaginal septectomy. This diagnosis should be suspected in females with a pelvic mass and ipsilateral renal agenesis.

Congenital lung malformations are often discovered incidentally on routine prenatal sonography or postnatal imaging. Lesions such as congenital cystic adenomatoid malformations (CCAM), sequestrations, bronchogenic cysts and congenital lobar emphysema may be asymptomatic at birth or at the time of discovery later in life. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CCAM and intralobar sequestration have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CCAM, intralobar sequestration and intrapulmonary bronchogenic cysts it is best not to wait for complications to occur. For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can occur. At this age the postoperative course is usually smooth and long-term follow-up has shown normal respiratory function. Mediastinal bronchogenic cysts also tend to become symptomatic and elective resection is recommended. On the other hand, asymptomatic congenital lobar emphysema may regress spontaneously and observation is warranted. The management of small noncommunicating extralobar sequestrations is more controversial; it is known that these lesions can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.