It is all about form and function: a new approach to giant neonatal sacrococcygeal teratomas.

INTRODUCTION: Giant sacrococcygeal teratomas (GSCTs) involve severe deformation of the buttock region in addition to potential functional impacts. Little interest has been given to improving the aesthetic post-operative appearance in children with these tumours. METHODS: We describe a new technique for immediate reconstruction of GSCTs using buried dermal-fat flaps and a low transverse scar in the infragluteal fold. RESULTS: Our technique allows wide exposure for tumour resection and functional restoration of the pelvic floor while placing the scars in anatomical locations and restoring buttock aesthetics including gluteal projection and infragluteal fold definition. CONCLUSION: Reestablishment of function and form should be kept in mind at initial surgery in GSCT surgery to maximize results and enhance post-operative outcomes. LEVEL OF EVIDENCE: IV.

Characterization of the Transition Zone in Short Segment Hirschsprung Disease Using Calretinin Immunostaining.

Introduction: The detailed expression pattern of calretinin immunohistochemistry in the transition zone (TZ) of Hirschsprung disease (HSCR) has not yet been reported. This study aims to examine the value of calretinin immunohistochemistry for more accurately determining the distal and proximal border of the TZ in short segment HSCR. Methods: Specimens of pull-through surgery from 51 patients with short form of HSCR were analyzed on two longitudinal strips using hematoxylin and eosin (H&E) staining and calretinin immunohistochemistry. Results: In all but two patients, the first appearance of calretinin expression was seen on mucosal nerve fibers before the appearance of any ganglion cells, indicating the distal border of the TZ. The maximum distance between the distal border of the TZ and the proximal border of the TZ, defined by ganglion cells in a normal density on H&E stained sections, a strong calretinin expression on mucosal nerve fibers and in >80% of submucosal and myenteric ganglion cells, with no nerve hypertrophy and absence of ganglionitis was 60 mm. Conclusion: The distal border of the TZ is characterized by calretinin positive intramucosal neurites in nearly all of short form of HSCR and not by calretinin expression on ganglion cells.

Glial Cell-Derived Neurotrophic Factor Induces Enteric Neurogenesis and Improves Colon Structure and Function in Mouse Models of Hirschsprung Disease.

BACKGROUND & AIMS: Hirschsprung disease (HSCR) is a life-threatening birth defect in which the distal colon is devoid of enteric neural ganglia. HSCR is treated by surgical removal of aganglionic bowel, but many children continue to have severe problems after surgery. We studied whether administration of glial cell derived neurotrophic factor (GDNF) induces enteric nervous system regeneration in mouse models of HSCR. METHODS: We performed studies with four mouse models of HSCR: Holstein (HolTg/Tg, a model for trisomy 21-associated HSCR), TashT (TashTTg/Tg, a model for male-biased HSCR), Piebald-lethal (Ednrbs-l//s-l, a model for EDNRB mutation-associated HSCR), and Ret9/- (with aganglionosis induced by mycophenolate). Mice were given rectal enemas containing GDNF or saline (control) from postnatal days 4 through 8. We measured survival times of mice, and colon tissues were analyzed by histology, immunofluorescence, and immunoblots. Neural ganglia regeneration and structure, bowel motility, epithelial permeability, muscle thickness, and neutrophil infiltration were studied in colon tissues and in mice. Stool samples were collected, and microbiomes were analyzed by 16S rRNA gene sequencing. Time-lapse imaging and genetic cell-lineage tracing were used to identify a source of GDNF-targeted neural progenitors. Human aganglionic colon explants from children with HSCR were cultured with GDNF and evaluated for neurogenesis. RESULTS: GDNF significantly prolonged mean survival times of HolTg/Tg mice, Ednrbs-l//s-l mice, and male TashTTg/Tg mice, compared with control mice, but not Ret9/- mice (which had mycophenolate toxicity). Mice given GDNF developed neurons and glia in distal bowel tissues that were aganglionic in control mice, had a significant increase in colon motility, and had significant decreases in epithelial permeability, muscle thickness, and neutrophil density. We observed dysbiosis in fecal samples from HolTg/Tg mice compared with feces from wild-type mice; fecal microbiomes of mice given GDNF were similar to those of wild-type mice except for Bacteroides. Exogenous luminal GDNF penetrated aganglionic colon epithelium of HolTg/Tg mice, inducing production of endogenous GDNF, and new enteric neurons and glia appeared to arise from Schwann cells within extrinsic nerves. GDNF application to cultured explants of human aganglionic bowel induced proliferation of Schwann cells and formation of new neurons. CONCLUSIONS: GDNF prolonged survival, induced enteric neurogenesis, and improved colon structure and function in 3 mouse models of HSCR. Application of GDNF to cultured explants of aganglionic bowel from children with HSCR induced proliferation of Schwann cells and formation of new neurons. GDNF might be developed for treatment of HSCR.

Characterization of Esophageal Motility in Children With Operated Esophageal Atresia Using High-resolution Impedance Manometry and Pressure Flow Analysis.

OBJECTIVES: Esophageal dysmotility is common in patients with esophageal atresia (EA). High-resolution impedance manometry and pressure flow analysis (PFA) allow characterization of biomechanical events that drive bolus flow. The aims were to assess esophageal motility in children with EA, using PFA, and to test whether there is a correlation between PFA parameters and symptoms or endoscopic/histologic findings. METHODS: High-resolution impedance manometry was performed in 16 children with EA (median age 11 years), compared with 13 patient controls (median age 14 years; P = NS vs patients). Wet swallows were analyzed using PFA. Medical charts were reviewed for symptoms and pathology results of the attendant esophagoscopy. Patients with EA were arbitrarily subgrouped according to their motility pattern: group A with presence of distal contraction in ≥50% of the swallows and group B with presence of distal contractions in <50% of the swallows. RESULTS: Esophageal peristaltic motor patterns were abnormal in all patients with EA. Bolus transport was impaired as shown by the higher impedance ratio in EA than in controls (0.47 vs 0.22; P < 0.001). Impedance ratio was also higher in group B (n = 8) versus group A (n = 8) (P < 0.001). Symptoms of dysphagia were not correlated with the PFA measures. Contractile segment impedance, a marker of mucosal integrity, was significantly lower in the EA group. CONCLUSIONS: Bolus transport was severely altered in patients with EA but was not predictive of symptoms. The presence of residual distal contractions is associated with a more efficient bolus propulsion.

Should Proton Pump Inhibitors be Systematically Prescribed in Patients With Esophageal Atresia After Surgical Repair?

OBJECTIVE: To evaluate outcomes of patients with esophageal atresia (EA) on systematic treatment with proton pump inhibitors (PPI) since the neonatal period and to determine factors associated with successful discontinuation of PPI. STUDY DESIGN: Longitudinal cohort study with prospective data collection of 73 EA patients, over 11 years systematically treated with PPI. Outcome and predictive factors for discontinuation of PPI treatment were evaluated at study end in February 2017. The incidence of anastomotic strictures was compared with a historical cohort of 134 EA patients followed in the same institution between 1990 and 2005 before the era of systematic PPI treatment. RESULTS: PPI treatment was discontinued definitively in 48% of patients during follow-up. Prematurity, longer initial hospitalization, moderate-to-severe tracheomalacia, anastomotic leak and anastomotic stricture had a significant negative association with PPI discontinuation on univariate analysis (P < 0.05). On adjusted multivariable Cox regression analysis, moderate-to-severe tracheomalacia and anastomotic leak were negatively associated with discontinuation of PPI treatment (hazard ratio 0.26 [95% CI 0.12-0.59]; P = 0.001 and hazard ratio 0.38 [95% CI 0.16-0.93]; P = 0.03, respectively). There was no significant difference in the incidence of anastomotic strictures in the present cohort compared with the historical cohort (44% vs 39%); (P > 0.05). CONCLUSIONS: PPI treatment does not prevent the formation of anastomotic strictures and appears to be over-prescribed in children with airway symptoms because of tracheomalacia. This suggests that PPI treatment could be prescribed more selectively. Close monitoring and long-term follow-up, however, of these vulnerable patients in specialized multidisciplinary clinics is imperative.

High-resolution Esophageal Manometry Patterns in Children and Adolescents With Rumination Syndrome.

BACKGROUND: Rumination is defined by effortless regurgitation within seconds or minutes of ingested food. The aim of this study was to determine the high-resolution esophageal manometry (HREM) pattern in children with rumination syndrome. METHODS: HREM was evaluated in 15 pediatric patients with rumination syndrome according to the Rome criteria and compared with 15 controls. Primary rumination was defined as a clinical rumination episode associated with a rise of gastric pressure above 30 mmHg. Secondary rumination was defined as a clinical rumination episode associated with a rise of gastric pressure above 30 mmHg during a transient lower esophageal sphincter relaxation (TLESR). RESULTS: Ninety-two episodes of rumination were demonstrated during HREM study in 12 of the 15 patients (80%; 1-29 episodes per patient; median intragastric pressure 49.6 mmHg). Primary rumination occurred in 3 patients and secondary rumination in 5 patients. One patient had primary and secondary rumination episodes. In 3 patients, classification of rumination episodes was not possible due to repetitive swallowing leading to lower esophageal sphincter relaxation. In the control group, no episodes of rumination occurred. The sensitivity and the specificity of the HREM study (association of a clinical rumination episode with a rise in gastric pressure >30 mmHg) to confirm the diagnosis of rumination were 80% and 100%, respectively. CONCLUSIONS: HREM allows confirming diagnosis of rumination syndrome and to differentiate between primary and secondary rumination in the presence of objective rumination episodes. Further research is needed to study whether HREM results may influence treatment and outcome of children with rumination syndrome.

Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula.

OBJECTIVE: To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA). METHODS: All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively. RESULTS: Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%. CONCLUSION: ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.

Esophageal dysmotility is present before surgery in isolated tracheoesophageal fistula.

After surgical correction of esophageal atresia with or without tracheoesophageal fistula, esophageal body motility dysfunction has been reported in nearly all patients. Using high-resolution esophageal manometry before surgical repair in 2 children with isolated tracheoesophageal fistula, we sought to determine whether dysmotility was present before any surgical insult to test the hypothesis that dysmotility associated with esophageal atresia with or without tracheoesophageal fistula is related to intrinsic primary factors linked to abnormal development of the esophagus. Both had an abnormal esophageal motility: one exhibited hypomotility with distal contraction, whereas the other showed a complete aperistalsis pattern. This suggests that esophageal dysmotility is congenital in nature rather than secondary to surgery.

Outcomes of Premature Infants With Type C Esophageal Atresia.

BACKGROUND: To review the outcomes of premature patients with type C esophageal atresia (EA). METHODS: In this retrospective cohort study, charts of patients of type C EA patients were reviewed from 1992 to 2022. Outcomes of premature patients were compared to term patients. Preterm patients were analyzed to compare outcomes of infants with very low birth weights (VLBW) to patients >1,500 g as well as primary versus delayed anastomosis. RESULTS: Among 192 type C EA, 67 were premature. Median and interquartile range (IQR) gestational age and birth weight of preterm patients were 34 [33-36] weeks and 1965 [1740-2290] g. Delayed anastomosis was performed in 12 (18%) preterm vs. 3 (2%) term patients (p = 0.0003). Short-term postoperative outcomes were similar between preterm and term patients, except for recurrent fistula (16% vs. 6%, p = 0.01). Prematurity was associated with an increased need for long-term enteral tube feeding (56% vs. 10%, p = 0.0001) and parenteral nutrition (10 days vs. 0 days, p = 0.0004). The length of stay was 3 times longer when patients were premature (50 days vs. 17 days, p = 0.002). Delayed surgery in preterm patients was associated with post-operative leaks, strictures, recurrent fistula, prolonged enteral tube feeding, and gastrostomy insertion. Patients with very low birth weight (VLBW) were compared to other preterm patients and showed no difference in terms of rate of delayed surgery, and post-operative outcomes. CONCLUSION: Despite increased prematurity-related comorbidities and low birth weight, premature infants with type C EA/TEF have similar post-operative outcomes to term patients though recurrent fistula was more frequent with prematurity. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: III.

The Canadian Pediatric Surgery Workforce: A 10-year Prospective Assessment.

BACKGROUND: The Canadian Association of Paediatric Surgeons launched a 10-year prospective assessment of the Canadian pediatric surgery workforce and training environment, beginning in 2013. The results of the first 5 years (2013-2017) were previously published. Here, we present the results of the last 5 years (2018-2022), and the cumulative results of the past decade. METHODS: With IRB approval, a web-based survey was sent to all pediatric surgery division chiefs in Canada each year (2013-2022). The survey gathered workforce data on pediatric surgery practices, as well as data regarding fellowship graduates from Canadian training programs. RESULTS: Complete responses were received from all 18 divisions (100% response rate). Over the decade studied, the number of pediatric surgeons and full-time equivalent positions increased from 73 to 81, and 65 to 82, respectively. Thirty positions were vacated (15 retirement, 6 new Canadian practice, 8 leaving Canada, 1 other), and 38 were filled (20 new Canadian fellowship graduates, 8 Canadian surgeons moving from other sites in Canada, 10 surgeons coming from outside Canada). Seventy-five fellows completed training eligible for North American certification, including 34 Canadians, 31 Americans, and 10 non-North American foreign nationals (9 of whom left North America after training). The proportion of Canadian graduates who desired, but could not find, a Canadian position improved from 44% in the first 5 years to 20% in the second 5 years. CONCLUSIONS: The Canadian pediatric surgery workforce has experienced a modest increase over a decade. A mismatch still exists between Canadian pediatric surgery graduates and attending staff positions, but the situation has improved during the last 5 years. TYPE OF STUDY: Survey.

Characterization of esophageal motility following esophageal atresia repair using high-resolution esophageal manometry.

BACKGROUND: Esophageal dysmotility, a considerable issue following esophageal atresia (EA) repair, has been reported but has not been precisely described and characterized. Using high-resolution esophageal manometry (HREM), we characterized the esophageal motility patterns in children with repaired EA and compared these patterns of dysmotility with symptomatology. METHODS: HREM was performed as an outpatient procedure in patients with repaired EA. The tracings were analyzed using the software provided by the company and were then reviewed visually. Charts were reviewed for medical/surgical histories and symptoms were assessed by a standardized questionnaire. RESULTS: Forty patients (25 boys, 15 girls) with a median age of 8 years (11 months-18 years) underwent an HREM. Thirty-five patients had type C EA and 5 had type A EA. Only 7 patients were asymptomatic at the time of the examination. HREM results were abnormal in all of the patients. Three different esophageal motility patterns were derived from HREM tracing analysis: aperistalsis (15 patients, 38%), pressurization (6 patients, 15%), and distal contractions (19 patients, 47%). Distal contractions pattern was found exclusively in type C EA. Dysphagia was encountered in the 3 groups. Gastroesophageal reflux disease-related symptoms predominated in the aperistalsis group. CONCLUSIONS: HREM improves our understanding and allows precise characterization of esophageal dysmotility in patients who have undergone EA repair.

Effect of amoxicillin/clavulanate on gastrointestinal motility in children.

AIM: The aim of the present study was to evaluate the effect of amoxicillin/clavulanate (A/C) on gastrointestinal motility. METHODS: Twenty consecutive pediatric patients referred for antroduodenal manometry received 20 mg/kg of A/C into the small bowel lumen. In 10 patients (group A), A/C was given 1 hour after and in 10 (group B), 1 hour before ingestion of a meal. Characteristics of the migrating motor complex, including presence, frequency, amplitude, and propagation of duodenal phase III and phase I duration and phase II motility index (MI), were evaluated 30 minutes before and after A/C administration. RESULTS: There were no statistically significant differences in age and sex between the 2 groups. Manometry studies were considered normal in 8 patients in each group. In group A, 2 patients developed duodenal phase III after receiving A/C, and no significant difference was found in the MI before and after the drug administration. In group B, 9 patients developed duodenal phase III (P <0.05 vs group A). All phase III occurred within a few minutes from the medication administration. Most duodenal phase III contractions were preceded by an antral component during fasting but never after the medication was administered in either of the 2 groups (P<0.001 vs fasting). In group B, the duration of duodenal phase I was shorter after drug administration (P<0.05). There was no significant difference in duodenal phase II MI before and after A/C administration for the 2 study groups. CONCLUSIONS: In children, administration of A/C directly into the small bowel before a meal induces phase III-type contractions in the duodenum, with characteristics similar to those present in the fasting state. These data suggest the possible use of A/C as a prokinetic agent. Further studies are needed to clarify its specific mechanism of action and the group of patients most likely to benefit from its use.

Effect of transanastomotic feeding tubes on anastomotic strictures in patients with esophageal atresia and tracheoesophageal fistula: The Quebec experience.

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.

Intestinal venous congestion as a complication of elective silo placement for gastroschisis.

Use of a spring-loaded silastic silo has been advocated as a means of gentle reduction of the herniated bowel, while avoiding the possible complications of primary closure of gastroschisis. We recently encountered intestinal venous congestion during elective silo reduction of gastroschisis. Two babies with gastroschisis were treated postdelivery with a spring-loaded silo placed under the fascial defect and the eviscerated bowel suspended within the silo. Patient #1 had no bowel matting. On day of life 2, the bowel within the silo was noted to be dusky. The silo was removed, and the bowel was indeed congested, but viable. Complete reduction with a modified Bianchi closure was performed at the bedside. Patient #2 had severe matting of the bowel and did not require intubation for silo placement. As daily reductions progressed, the bowel was noted to be congested on day 2. On day 3, removal of the silo revealed frank bowel necrosis with impending perforation. Two-thirds of the small bowel required resection, leaving the child with short bowel. Venous congestion within a silo should be given prompt attention, including removal of the silo, as bowel infarction may result.

Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC).

PURPOSE: This study aims to characterize risk factors for Hirschsprung-associated enterocolitis (HAEC). We hypothesize that earlier pull-through surgery is associated with lower risks of developing postoperative HAEC. METHODS: A comparative study of 171 Hirschsprung patients treated from 1990 to 2017 was performed. Patients without HAEC were compared to patients with preoperative and/or postoperative HAEC. Results are presented as median [IQR] or frequency (%). Pearson's χ2 test and Wilcoxon rank sum test were performed with a significance level at p < 0.05. Multivariable logistic regression analysis was used to adjust for potential confounders. A subanalysis was done to evaluate laparoscopic, laparotomy, and transanal surgeries. RESULTS: Risk of developing preoperative HAEC was significantly associated with congenital malformations (OR 2.63 [1.11, 6.24]; p = 0.02). Birth weight was lower in patients with preoperative HAEC (OR 0.48 [95% CI 0.25, 0.93]; p = 0.03). On regression analysis, intestinal obstruction after surgery was significantly associated with postoperative HAEC (OR 8.2 [3.18, 21.13]; p < 0.0001). Patients with earlier pull-through surgery did not have a lower risk of developing postoperative HAEC. CONCLUSIONS: Timing of surgery does not seem to be associated with a higher risk of developing pre- and postoperative HAEC. Predisposing factors for preoperative HAEC included associated malformations and lower birth weight, whereas intestinal obstruction was found to be associated with postoperative HAEC. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Postoperative noninvasive ventilation and complications in esophageal atresia-tracheoesophageal fistula.

PURPOSE: This study examines the impact of postoperative noninvasive ventilation strategies on outcomes in esophageal atresia-tracheoesophageal fistula (EA-TEF) patients. METHODS: A single center retrospective chart review was conducted on all neonates followed at the EA-TEF Clinic from 2005 to 2017. Primary outcomes were: survival, anastomotic leak, stricture, pneumothorax, and mediastinitis. Statistical significance was determined using Chi-square and logistic regression (p ≤ .05). RESULTS: We reviewed 91 charts. Twenty-five infants (27.5%) were bridged with postextubation noninvasive ventilation (15 on Continuous Positive Airway Pressure (CPAP), 5 on Noninvasive Positive Pressure Ventilation (NIPPV), and 14 on High-Flow Nasal Cannula (HFNC)). Overall, 88 (96.7%) patients survived, 25 (35.7%) had a stricture, 14 (20%) had anastomotic leak, 9 (12.9%) had a pneumothorax, and 4 (5.7%) had mediastinitis. Use of NIPPV was associated with increased risk of mediastinitis (P = .005). Use of HFNC was associated with anastomotic leak (P = .009) and mediastinitis (P = .036). CONCLUSIONS: These data suggest that postoperative noninvasive ventilation techniques are associated with a significantly higher risk of anastomotic leak and mediastinitis. Further prospective research is needed to guide postoperative ventilation strategies in this population. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: IV.

Prevalence and Predictive Factors of Histopathological Complications in Children with Esophageal Atresia.

OBJECTIVES: Endoscopic follow-up after esophageal atresia (EA) tracheoesophageal fistula (TEF) repair is recommended to detect esophageal histopathological complications. We investigated the prevalence of histopathologically proven esophageal complications (peptic esophagitis, gastric metaplasia, and eosinophilic esophagitis) and assessed the predictors of these complications in children with EA-TEF. MATERIALS AND METHODS: This is a prospective longitudinal cohort study performed between September 2005 and December 2014 comprising 77 children with EA-TEF followed-up until February 2017. Univariate analysis was performed using the Wilcoxon's rank-sum test for continuous variables and the Pearson's chi-square test for categorical variables. Multivariable analysis was performed using a Cox regression hazard model. The association between clinical factors and histopathologically proven complications was estimated using a Cox regression hazard model with time until the appearance of complications as the time scale. RESULTS: All 77 children received proton pump inhibitors (PPIs) (n = 73) or H2 receptor antagonists (H2RA). A total of 252 endoscopies were performed in 73 children (median 2.6/child, range: 1-29). Median age at study completion was 4.9 years (range: 2.3-11.5 years). Histopathologically proven complications occurred in 38 children (52%): peptic esophagitis (n = 32, 44%), eosinophilic esophagitis (n = 15, 21%), and gastric metaplasia (n = 9, 12%). A total of 82% patients were on PPI or H2RA at the time of diagnosis of histological complication. Multivariable Cox regression analysis showed that patients with recurrent anastomotic strictures (>3 dilations) had a higher risk of occurrence of histopathologically proven complications over time (hazard ratio: 3.11, 95% confidence interval [CI]: 1.53-6.34). On univariate analysis, the result of the first endoscopy was not associated with the occurrence of histopathologically proven complications (odds ratio: 0.8, 95% CI: 0.16-3.95). CONCLUSION: Histopathologically proven complications with potential long-term consequences occurred in approximately 50% of children after EA-TEF repair. A history of recurrent anastomotic strictures is associated with the occurrence of these complications. The result of the first endoscopy does not predict the histopathological outcome. Children with EA-TEF warrant close and systematic long-term follow-up at specialized multidisciplinary clinics with endoscopic evaluation.

The Canadian pediatric surgery workforce: A 5-year prospective study.

BACKGROUND: In 2014, a survey study of the Canadian pediatric surgery workforce predicted a need for 2 new pediatric surgeons/yr. in Canada. We sought to assess these predictions and evaluate the status of the workforce. METHODS: With IRB approval, a web-based survey was sent to pediatric surgery division chiefs in Canada each year (2013-2017). The survey data included: number of practicing pediatric surgeons, full time equivalent (FTE) positions, and fellowship graduates. RESULTS: There was a 100% response rate (18 divisions). From 2013 to 2017, the number of practicing pediatric surgeons and FTE positions increased (73 to 78, and 64.6 to 67.5, respectively). Eleven positions were vacated (4 retirement, 7 new practice), and 18 were filled. Eight were filled by new Canadian graduates, 7 by Canadians previously working in Canada or abroad, and 3 by European surgeons. Thirty-eight fellows completed training in Canada, including 24 non-Canadians who all left Canada. Nine Canadians who started practicing immediately after fellowship took positions in Canada (5) and the US (4). CONCLUSIONS: Predictions made in 2014 were largely accurate. There has been modest growth in the Canadian pediatric surgery workforce over the last 5 years. A significant mismatch continues to exist between Canadian pediatric surgery graduates and attending staff positions. TYPE OF STUDY: Survey. LEVEL OF EVIDENCE: V.

Diagnosis and Management of Necrotizing Enterocolitis: An International Survey of Neonatologists and Pediatric Surgeons.

BACKGROUND: Necrotizing enterocolitis (NEC) is a serious complication of prematurity. Currently, there is limited evidence to guide investigation and treatment strategies. OBJECTIVES: To evaluate the parameters used to diagnose or exclude NEC, and to identify differences between neonatologists and pediatric surgeons. METHODS: A scenario-based survey was sent to neonatologists and pediatric surgeons. RESULTS: 173 physicians from 26 countries completed the survey (55% neonatologists and 45% pediatric surgeons). Bloody stools, abdominal tenderness, low platelet counts, and increased lactate levels increased the likelihood of NEC for 82, 72, 56, and 45% of respondents, respectively. Intestinal pneumatosis, portal venous gas, and pneumoperitoneum on X-ray increased the likelihood of NEC for 99, 98, and 92% of respondents, respectively. Clinical examination and laboratory tests were insufficient to exclude NEC, but normal intestinal movements and normal gut wall thickness on ultrasonography decreased the likelihood of NEC for 38 and 33% of respondents, respectively. Neonatologists more frequently relied on increased gastric residuals and abdominal distension to diagnose NEC (p = 0.04 and p = 0.03, respectively), whereas pediatric surgeons more frequently reported that absence of bloody stools helped to exclude NEC (p = 0.04). In a deteriorating patient with suspected NEC, 39% of respondents would broaden the antibiotic spectrum, and 42% would recommend a laparotomy. CONCLUSION: Our results indicate a wide variation in the management of NEC, with significant differences between neonatologists and pediatric surgeons. A better appreciation of the relative significance and weighting that should be applied to the clinical features and investigations should reduce the variation in interpretation that appears to exist.