Esophageal Dilation in Children: A State of the Art Review.

Esophageal dilations in children are performed by several pediatric and adult professionals. We aim to summarize improvements in safety and new technology used for the treatment of complex and refractory strictures, including triamcinolone injection, endoscopic electro-incisional therapy, topical mitomycin-C application, stent placement, functional lumen imaging probe assisted dilation, and endoscopic vacuum-assisted closure in the pediatric population.

Measurement of Stricture Dimensions Using a Visual Comparative Estimation Method With Biopsy Forceps During Endoscopy.

BACKGROUND: Estimation of the dimensions of endoscopic findings such as stricture diameter is largely subjective. Accurate assessment of stricture dimensions has multiple benefits including facilitating the choice of appropriately sized endoscopic therapies for treating stricture, properly tracking response to endoscopic therapies between procedures, and potentially even predicting outcomes of endoscopic therapy. METHODS: Endoscopies performed in children with repaired esophageal atresia between August 2019 and August 2021 for which both (1) an endoscopic estimate of esophageal stricture diameter obtained by visual comparison with the known dimensions of the biopsy forceps and (2) an intraoperative esophageal fluoroscopy study were performed were included for analysis. Fluoroscopic stricture diameter measurements were manually obtained using a software ruler tool calibrated to the known dimensions of the intraluminal endoscope. Statistical concordance was calculated between the visual diameter estimates and the standard fluoroscopic stricture measurements. RESULTS: One hundred ninety-one endoscopies were included for analysis. Lin's concordance correlation coefficient was 0.92 (95% confidence interval: 0.89-0.94) between the visual diameter estimates and the fluoroscopic stricture measurements. Correlation was strongest for smaller to mid-sized stricture diameters. CONCLUSIONS: Use of the biopsy forceps as a visual reference of known dimensions enables accurate visual estimation of esophageal stricture diameter during endoscopy using commonly available tools, with high concordance with standard fluoroscopic measurement techniques.

Predictors of enteral tube dependence in pediatric esophageal atresia.

Children with esophageal atresia (EA) may require enteral tube feedings in infancy and a subset experience ongoing feeding difficulties and enteral tube dependence. Predictors of enteral tube dependence have never been systematically explored in this population. We hypothesized that enteral tube dependence is multifactorial in nature, with likely important contributions from anastomotic stricture. Cross-sectional clinical, feeding, and endoscopic data were extracted from a prospectively collected database of endoscopies performed in EA patients between August 2019 and August 2021 at an international referral center for EA management. Clinical factors known or hypothesized to contribute to esophageal dysphagia, oropharyngeal dysphagia, or other difficulties in meeting caloric needs were incorporated into regression models for statistical analysis. Significant predictors of enteral tube dependence were statistically identified. Three-hundred thirty children with EA were eligible for analysis. Ninety-seven were dependent on enteral tube feeds. Younger age, lower weight Z scores, long gap atresia, neurodevelopmental risk factor(s), significant cardiac disease, vocal fold movement impairment, and smaller esophageal anastomotic diameter were significantly associated with enteral tube dependence in univariate analyses; only weight Z scores, vocal fold movement impairment, and anastomotic diameter retained significance in a multivariable logistic regression model. In the current study, anastomotic stricture is the only potentially modifiable significant predictor of enteral tube dependence that is identified.

Comparison of Detection Methods for Tracheoesophageal Fistulae With a Novel Method: Capnography With CO2 Insufflation.

BACKGROUND: Tracheoesophageal fistulae (TEF) are difficult to detect and require a high index of suspicion. We hypothesized that capnography to identify a spike in end-tidal carbon dioxide (etCO2) during esophagoscopy with carbon dioxide (CO2) insufflation would facilitate TEF diagnosis because of gas passage from the esophagus to the trachea. METHODS: Medical records of 42 consecutive cases of recurrent, acquired, or missed congenital TEF diagnosed between January 2015 and November 2019 that underwent esophagoscopy with CO2 insufflation were reviewed. A control cohort of 97 similarly endoscopically evaluated patients with surgical confirmation of absence of recurrent TEF (eg, patients undergoing posterior tracheopexy) was also collected. All patients underwent pre-operative esophagoscopy, bronchoscopy, and capnography; diagnostic abilities of various combinations of modalities for TEF identification were calculated. RESULTS: Statistical analysis identified a maximum intra-esophagoscopy end-tidal CO2 level of 68 mmHg as the optimal discriminator between cases and controls, though in practice, we anecdotally find that recurrent TEFs typically permit rapid rise ≥90 mmHg. Increasing numbers of diagnostic modalities increased diagnostic sensitivity to detect recurrent TEF; the highest diagnostic sensitivity for TEF identification was achieved by the combination of intra-esophagoscopy fluoroscopy with bronchoscopy and capnography ≥68 mmHg (sensitivity = 88.1%). There were multiple cases of TEF (N = 7 for etCO2 ≥68 mmHg, N = 3 for etCO2 ≥90 mmHg) identified by capnography that were missed by esophagoscopy. There were 5 (for etCO2 ≥68 mmHg) or 6 (for etCO2 ≥90 mmHg) cases of recurrent TEF that were missed by all nonsurgical methods. CONCLUSION: Attention to etCO2 during esophagoscopy with CO2 insufflation represents a simple, novel way to detect TEF. Identification of TEF remains challenging, though combinations of diagnostic modalities improve diagnostic sensitivity.

Rules Are Meant to Be Broken: Examining the "Rule of 3" for Esophageal Dilations in Pediatric Stricture Patients.

BACKGROUND AND AIMS: The "rule of 3" is a 40-year-old expert opinion that suggests dilating an esophageal stricture more than 3 mm is unsafe. Few studies have evaluated this tenet, and do not specify how much larger than 3 mm is reasonable. Our aim was to determine the optimal point for maximum dilation diameter with acceptable risk in a pediatric population. METHODS: A retrospective review in pediatric patients with esophageal strictures was performed. The number of millimeters the stricture was dilated, defined as delta dilation diameter (ΔDD), was determined by subtracting the initial stricture diameter from the diameter of the largest balloon used. Receiver operating characteristic curve analysis was used to evaluate the discriminatory ability of ΔDD. Youden J index was used to identify optimal cut-point in predicting perforation. RESULTS: Two hundred eighty-four patients underwent 1384 balloon dilations. Overall perforation rate was 1.66%. There were 8 perforations in 1075 dilations with ΔDD ≤5 mm (0.7%) and 15 perforations in 309 dilations with ΔDD >5 mm (4.9%). Youden J index found an optimal cutoff to be at a ΔDD of ≤5 mm. The cumulative rate of perforation for all dilations ≤5 mm was 0.74% whereas the cumulative risk of perforation for all dilations ≥6 mm was 4.85% (P < 0.001). CONCLUSIONS: Balloon dilations that expand the initial esophageal anastomosis ≤5 mm in a pediatric population appear to not unduly increase the risk of perforation. Further prospective studies are needed to further investigate the potential for a new rule of 5 for balloon dilation.

Intralesional Steroid Injection Therapy for Esophageal Anastomotic Stricture Following Esophageal Atresia Repair.

OBJECTIVES: The role of intralesional steroid injection (ISI) in the treatment of anastomotic stricture in patients with esophageal atresia remains unclear. The aim of this study was to evaluate the efficacy and safety of ISI. METHODS: A total of 158 patients with esophageal atresia with at least 1 ISI for the treatment of esophageal anastomotic stricture between 2010 and 2017 were identified. The change in stricture diameter (ΔD) was compared between procedures with dilation alone (ISI-) and dilation with steroid injection (ISI+). RESULTS: A total of 1055 balloon dilations were performed (452 ISI+). The median ΔD was significantly greater in the ISI+ group: 1 mm (interquartile range [IQR] 0, 3) versus 0 mm (IQR -1, 1.5) (P < 0.0001). The ISI+ group had greater percentage of improved diameter (P < 0.0001) and lesser percentages of unchanged and decreased diameters at subsequent endoscopy (P = 0.0009, P = 0.003). Multivariable logistic regression confirmed the significance of ISI on increasing the likelihood of improved stricture diameter with an adjusted odds ratio of 3.24 (95% confidence interval: 2.15-4.88) (P < 0.001). The ΔD for the first 3 ISI+ procedures was greater than the ΔD for subsequent ISI+ procedures: 1 mm (IQR 0, 3) versus 0.5 mm (IQR-1.25, 2) (P = 0.001). There was no difference in perforation incidence between ISI+ and ISI- groups (P = 0.82). CONCLUSIONS: ISI with dilation was well tolerated and improved anastomotic stricture diameter more than dilation alone. The benefit of ISI over dilation alone was limited to the first 3 ISI procedures.

Utility of repeated therapeutic endoscopies for pediatric esophageal anastomotic strictures.

Anastomotic stricture is a common complication of esophageal atresia (EA) repair. Such strictures are managed with dilation or other therapeutic endoscopic techniques such as steroid injections, stenting, or endoscopic incisional therapy (EIT). In situations where endoscopic therapy is unsuccessful, patients with refractory strictures may require surgical stricture resection; however, the point at which endoscopic therapy should be abandoned in favor of repeat thoracotomy is unclear. We hypothesized that increasing numbers of therapeutic endoscopies are associated with increased likelihood of stricture resection. We retrospectively reviewed the records of patients with EA who had an initial surgery at our institution resulting in an esophago-esophageal anastomosis between August 2005 and May 2019. Up to 2 years of post-surgery endoscopy data were collected, including exposure to balloon dilation, intralesional steroid injection, stenting, and EIT. Primary outcome was need for stricture resection. Receiver operating characteristic (ROC) curve analysis and univariate and multivariable Cox proportional hazards regression analyses were performed. There were 171 patients who met inclusion criteria. The number of therapeutic endoscopies was a moderate predictor of stricture resection by ROC curve analysis (AUC = 0.720, 95% CI 0.617-0.823). With increasing number of therapeutic endoscopies, the probability of remaining free from stricture resection decreased. By Youden's J index, a cutoff of ≥7 therapeutic endoscopies was optimal for discriminating between patients who had versus did not have stricture resection, though an absolute majority of patients (≥50%) remained free of stricture resection at each number of therapeutic endoscopies through 12 endoscopies. Significant predictors of needing stricture resection by univariate regression included ≥7 therapeutic endoscopies, Foker surgery for long-gap EA, fundoplication, history of esophageal leak, and length of stricture ≥10 mm. Multivariate analysis identified only history of leak as statistically significant, though this regression was underpowered. The utility of repeated therapeutic endoscopies may diminish with increasing numbers of endoscopic therapeutic attempts, with a cutoff of ≥7 endoscopies identified by our single-center experience as our statistically optimal discriminator between having stricture resection versus not; however, a majority of patients remained free of stricture resection well beyond 7 therapeutic endoscopies. Though retrospective, this study supports that repeated therapeutic endoscopies may have clinical utility in sparing surgical stricture resection. Esophageal leak is identified as a significant predictor of needing subsequent stricture resection. Prospective study is needed.

Esophagitis in Pediatric Esophageal Atresia: Acid May Not Always Be the Issue.

OBJECTIVE: Esophagitis is highly prevalent in patients with esophageal atresia (EA). Peptic esophagitis has long been assumed to be the primary cause of esophagitis in this population, and prolonged acid suppressive medication usage is common; such treatment is of unknown benefit and carries potential risk. METHODS: To better understand the role of commonly used antireflux treatments in EA, we analyzed all patients with repaired EA who underwent endoscopy with biopsies at our institution between January 2016 and August 2018. Macroscopic erosive and histologic esophagitis on biopsy was graded per predefined criteria. Clinical characteristics including acid suppressive medication usage, type of EA and repair, presence of hiatal hernia, and history of fundoplication were reviewed. RESULTS: There were 310 unique patients (33.5% long gap EA) who underwent 576 endoscopies with biopsies during the study period. Median age at endoscopy was 3.7 years (interquartile range 21-78 months). Erosive esophagitis was found in 8.7% of patients (6.1% of endoscopies); any degree of histologic eosinophilia (≥1 eosinophil/high power field [HPF]) was seen in 56.8% of patients (48.8% of endoscopies), with >15 eosinophils/HPF seen in 15.2% of patients (12.3% of endoscopies). Acid suppression was common; 86.9% of endoscopies were preceded by acid suppressive medication use. Fundoplication had been performed in 78 patients (25.2%). Proton pump inhibitor (PPI) and/or H2 receptor antagonist (H2RA) use were the only significant predictors of reduced odds for abnormal esophageal biopsy (P = 0.011 for PPI, P = 0.048 for H2RA, and P = 0.001 for PPI combined with H2RA therapy). However, change in intensity of acid suppressive therapy by either dosage or frequency was not significantly associated with change in macroscopic erosive or histologic esophagitis (P > 0.437 and P > 0.13, respectively). Presence or integrity of a fundoplication was not significantly associated with esophagitis (P = 0.236). CONCLUSIONS: In EA patients, acid suppressive medication therapy is associated with reduced odds of abnormal esophageal biopsy, though histologic esophagitis is highly prevalent even with high rates of acid suppressive medication use. Esophagitis is likely multifactorial in EA patients, with peptic esophagitis as only one of multiple possible etiologies for esophageal inflammation. The clinical significance of histologic eosinophilia in this population warrants further investigation.

Endoscopic Esophageal Vacuum Therapy: A Novel Therapy for Esophageal Perforations in Pediatric Patients.

BACKGROUND: Esophageal perforation is a potentially life-threatening problem if not quickly diagnosed and treated appropriately. Negative-pressure wound therapy, commercially known as V.A.C. therapy, was developed in the early 1990s and is now standard of care for chronic surface wounds, ulcers, and burns. Adapting vacuum sponge therapy for use intraluminally for perforations of the esophagus was first reported in 2008. We report the first pediatric experience on a customized esophageal vacuum-assisted closure (EVAC) device for closure of esophageal perforations. AIM: To evaluate the technical feasibility, safety, and efficacy of EVAC in a pediatric population with esophageal perforations and compare efficacy to a cohort of patients who underwent stenting for esophageal perforation. METHODS: We performed an institutional review board-approved retrospective chart review on all patients who underwent EVAC for esophageal perforations (October 2013-September 2017) and who underwent externally removable stent placement for esophageal perforation (January 2010-December 2017) at our institution. Our primary aim was to evaluate technical feasibility, efficacy, and safety in the treatment of pediatric esophageal perforations. A secondary aim was to compare the efficacy of EVAC to esophageal stenting in healing esophageal perforations in our pediatric population. RESULTS: A total of 17 patients with esophageal atresia underwent therapy for esophageal perforation. Eight sponges were placed for surgical perforation and 9 were placed after endoscopic therapy perforation. The median age of patients was 24 months with the youngest patient being 3 months of age. The success rate of EVAC to seal all esophageal perforations was 88% (15/17). The success rate was similar in both subgroups: surgical anastomotic leaks at 88% (7/8) and endoscopic therapy leaks at 89% (8/9). There were no technical failures with placement. The stent group had a total of 24 patients: 19 were placed secondary to perforations from endoscopic therapy and 5 were placed secondary to surgical anastomotic perforations. The success rate of stents to seal all esophageal perforations was 63% (15/24). The success rate in the subgroups was 74% (14/19) for endoscopic therapy leaks and 20% (1/5) for surgical anastomotic leaks. In comparing success of EVAC and stent therapy, we found a statistically significant difference in favor of EVAC in healing surgical anastomotic perforations (P = 0.032). There was, however, no statistical difference in healing endoscopic therapy perforations (P = 0.360). CONCLUSIONS: EVAC is a novel, promising technique for the treatment of esophageal perforations in a pediatric population. This treatment is comparable to esophageal stenting in iatrogenic endoscopic therapy perforations and superior to stenting surgical perforations. Further prospective studies are needed to compare the effectiveness of EVAC to esophageal stenting. Improvement in device design and customization could further improve success and ease of placement.

Endoscopic Electrocautery Incisional Therapy as a Treatment for Refractory Benign Pediatric Esophageal Strictures.

BACKGROUND AND AIM: Refractory esophageal strictures are rare conditions in pediatrics, and are often due to anastomotic, congenital, or caustic strictures. Traditional treatment options include serial dilation and surgical stricture resection; endoscopic intralesional steroid injections, mitomycin C, and externally removable stents combined with dilation have had variable success rates. Although not as widely used, endoscopic electrocautery incisional therapy (EIT) has been reported as an alternative treatment for refractory strictures in a small number of adult series. The aim of the study was to evaluate the safety and efficacy of EIT in a pediatric population with refractory esophageal strictures. METHODS: A retrospective chart review was conducted on all patients who underwent EIT for esophageal strictures (May 2011-September 2017) at our tertiary-care referral center. A total of 57 patients underwent EIT. Procedural success was defined as no stricture resection, appropriate diameter for age, and fewer than 7 dilations within 24 months of first EIT session. This corresponded to the 90th percentile of the observed number of dilations in the data. All patients included in the study had at least 2-year follow-up. RESULTS: A total of 133 EIT sessions on 58 distinct anastomotic strictures were performed on 57 patients (24 girls). The youngest patient to have EIT was 3 months old and 4.8 kg. There were 36 strictures that met the criteria for refractory stricture and 22 non-refractory (NR) strictures. The median number of dilations before EIT therapy was 8 (interquartile range [IQR]: 6-10) in the refractory group and 3 (IQR: 0-3) in the NR group. In the refractory group, 61% of the patients met the criteria for treatment success. The median number of dilations within 2 years of EIT in the refractory group was 2 (IQR: 0-4). In the NR group, 100% of the patients met criteria for success. The median number of dilations within 2 years of EIT in the NR was 1 (IQR: 0-2). The overall adverse event rate was 5.3% (7/133), with 3 major (2.3%) and 4 minor events (3%). CONCLUSIONS: EIT shows promise as an adjunct treatment option for pediatric refractory esophageal strictures and may be considered before surgical resection even in severe cases. The complication rate, albeit low, is significant, and EIT should only be considered by experienced endoscopists in close consultation with surgery. Further prospective longitudinal studies are needed to validate this treatment.

Enteroscopy.

Noninvasive imaging with CT and magnetic resonance enterography or direct visualization with wireless capsule endoscopy can provide valuable diagnostic information and direct therapy. Enteroscopy technology and techniques have evolved significantly and allow diagnosis and therapy deep within the small bowel, previously attainable only with intraoperative enteroscopy. Push enteroscopy, readily available in most endoscopy units, plays an important role in the evaluation and management of lesions located up to the proximal jejunum. Currently available device-assisted enteroscopy systems, DBE, SBE, and spiral enteroscopy each have their technical nuances, clinical advantages, and limitations. Newer, on-demand enteroscopy systems appear promising, but further studies are needed. Despite slight differences in parameters such as procedural times, depths of insertion, and rates of complete enteroscopy, the overall clinical outcomes with all overtube-assisted systems appear to be similar. Endoscopists should therefore master the enteroscopy technology based on institutional availability and their level of technical expertise.

Electronic chromoendoscopy.

Electronic chromoendoscopy technologies provide image enhancement and may improve the diagnosis of mucosal lesions. Although strides have been made in standardization of image characterization, especially with NBI, further image-to-pathology correlation and validation are required. There is promise for the development of a resect and discard policy for diminutive adenomas by using electronic chromoendoscopy; however, before this can be adopted, further community-based studies are needed. Further validated training tools for NBI, FICE, and i-SCAN will also be required for the use of these techniques to become widespread.

Externally removable stents in the treatment of benign recalcitrant strictures and esophageal perforations in pediatric patients with esophageal atresia.

BACKGROUND: We investigated whether removable stents, such as self-expandable plastic stents (SEPSs) and fully covered self-expandable metal stents (FCSEMSs) could provide an alternative treatment for recalcitrant strictures and esophageal perforations after esophageal atresia (EA) repair. OBJECTIVE: The primary aim of our study was to evaluate technical feasibility. Secondary aims were to evaluate safety and procedural success. DESIGN: Retrospective study. SETTING: Tertiary-care referral center. PATIENTS: A total of 24 children with EA. INTERVENTIONS: Retrospective review of all children with EA who underwent dilation and esophageal stent placement from January 2010 to February 2013 at our institution. MAIN OUTCOME MEASUREMENTS: Healing of perforation and stricture resolution at 30 and 90 days. RESULTS: A total of 41 stents (SEPSs 14, FCSEMSs 27) were placed in 24 patients with EA during the study period, including 14 who had developed esophageal leaks. Procedural success of esophageal stent placement in the treatment of refractory strictures was 39% at 30 days and 26% at 90 days. The success rate was 80% for closure of esophageal perforations with stent therapy after dilation and 25% for perforations associated with surgical repair. Adverse events of stent placement included migration (21% of SEPSs and 7% of FCSEMSs), granulation tissue (37% of FCSEMSs), and deep ulcerations (22% of FCSEMSs). LIMITATIONS: Retrospective study with small sample size. CONCLUSION: SEPSs and FCSEMSs can be placed successfully in small infants and children with a history of EA repair. The stents appear to be safe and beneficial in closing esophageal perforations, especially post-dilation. However, a high stricture recurrence rate after stent removal may limit their usefulness in treating recalcitrant esophageal anastomotic strictures.

Good agreement between endoscopic findings and biopsy reports supports limited tissue sampling during pediatric colonoscopy.

OBJECTIVES: Colonoscopy in children routinely includes the practice of obtaining multiple biopsy samples even in the absence of gross mucosal abnormalities. The aim of our investigation was to examine the level of agreement between endoscopic and histological findings during pediatric colonoscopy. We also investigated the predictors of agreement and abnormal histology. METHODS: We performed an institutionally approved retrospective review of consecutive patients who underwent diagnostic colonoscopy during a 6-month period. Descriptive analyses and regression models were used to determine agreement rates, as well as potential predictors of both agreement and abnormal histology. RESULTS: Of 390 included colonoscopies, endoscopists (n = 26) reported abnormal gross findings in 218 (56%) and pathologists (n = 4) found histopathology in 195 (50%). Considering histology as the criterion standard, endoscopy had a sensitivity of 90% and a specificity of 78%. Reports of grossly normal endoscopic findings were highly associated with agreement (odds ratio [OR] 1.9, P = 0.001). A known diagnosis of inflammatory bowel disease was a strong predictor of abnormal histology (OR 6.4, P < 0.0001). Abdominal pain as a procedural indication was a strong predictor for normal histology (OR 0.4, P < 0.0001). CONCLUSIONS: The results of our study suggest good agreement between endoscopic and histological findings, especially when an endoscopist reports normal-appearing colonic mucosa. We identified predictors of abnormal histology to include known inflammatory bowel disease, whereas abdominal pain was found to be a negative predictor. Future studies are needed to determine evidence-based protocols for obtaining biopsies during colonoscopy in children.

Surveillance Endoscopy in Pediatric Esophageal Atresia: Toward an Evidence-Based Algorithm.

BACKGROUND: Individuals with esophageal atresia (EA) have lifelong increased risk for mucosal and structural pathology of the esophagus. The use of surveillance endoscopy to detect clinically meaningful pathology has been underexplored in pediatric EA. We hypothesized that surveillance endoscopy in pediatric EA has high clinical yield, even in the absence of symptoms. STUDY DESIGN: The medical records of all patients with EA who underwent at least 1 surveillance endoscopy between March 2004 and March 2023 at an international EA referral center were retrospectively reviewed. The primary outcomes were endoscopic identification of pathology leading to an escalation in medical, endoscopic, or surgical management. Logistic regression analysis examined predictors of actionable findings. Nelson-Aalen analysis estimated optimal endoscopic surveillance intervals. RESULTS: Five hundred forty-six children with EA underwent 1,473 surveillance endoscopies spanning 3,687 person-years of follow-up time. A total of 770 endoscopies (52.2%) in 394 unique patients (72.2%) had actionable pathology. Esophagitis leading to escalation of therapy was the most frequently encountered finding (484 endoscopies, 32.9%), with most esophagitis attributed to acid reflux. Barrett's esophagus (intestinal metaplasia) was identified in 7 unique patients (1.3%) at a median age of 11.3 years. No dysplastic lesions were identified. Actionable findings leading to surgical intervention were found in 55 children (30 refractory reflux and 25 tracheoesophageal fistulas). Significant predictors of actionable pathology included increasing age, long gap atresia, and hiatal hernia. Symptoms were not predictive of actionable findings, except dysphagia, which was associated with stricture. Nelson-Aalen analysis predicted occurrence of an actionable finding every 5 years. CONCLUSIONS: Surveillance endoscopy uncovers high rates of actionable pathology even in asymptomatic children with EA. Based on the findings of the current study, a pediatric EA surveillance endoscopy algorithm is proposed.

Endoscopic Management of Congenital Esophageal Defects and Associated Comorbidities.

The endoscopist plays a critical role in the management of patients with congenital esophageal defects. This review focuses on esophageal atresia and congenital esophageal strictures and, in particular, the endoscopic management of comorbidities related to these conditions, including anastomotic strictures, tracheoesophageal fistulas, esophageal perforations, and esophagitis surveillance. Practical aspects of endoscopic techniques for stricture management are reviewed including dilation, intralesional steroid injection, stenting, and endoscopic incisional therapy. Endoscopic surveillance for mucosal pathology is essential in this population, as patients are at high risk of esophagitis and its late complications such as Barrett's esophagus.

Evolution of left-sided thoracoscopic approach for long gap esophageal atresia repair.

BACKGROUND: Left-sided repair for long gap esophageal atresia (LGEA) has been described for patients with a large leftward upper pouch, no thoracic tracheoesophageal fistula (TEF) nor tracheobronchomalacia (TBM), or as salvage plan after prior failed right-sided repair. We describe our experience with left-sided MIS traction induced growth process. METHODS: We retrospectively reviewed patients who underwent Foker process for LGEA at two institutions between December 2016 and November 2021. Patient characteristics, surgical techniques, and outcomes were reviewed. RESULTS: 71 patients underwent Foker process. Of 34 MIS cases, 28 patients (82%) underwent left-sided repair (median gap length 5 cm) at median age 4 months with median 3 (range 2-8) operations and median 13.5 (IQR 11-21) days on traction until esophageal anastomosis. 9 patients (32%) underwent completely MIS approach, whereas 5 patients (18%) converted to open at first operation and 14 patients (50%) converted to open later in the traction process. Traction was internal in 68%, external in 11%, and combination in 21%. Median follow-up was 15.4 (IQR 7.5-31.7) months after anastomosis. 14% had anastomotic leak managed with antibiotics and/or esophageal vacuum therapy. Median number of esophageal dilations was 3.5 (range 0-13). 18% required stricture resection. 39% underwent Nissen fundoplication. None have needed esophageal replacement. CONCLUSIONS: For multiple reasons including the tendency of both esophageal pouches to have a leftward bias, less tracheal compression by upper pouch, and clean field of surgery for reoperative cases, we now more commonly use left-sided approach for MIS LGEA repair compared to right side, regardless of left aortic arch. LEVEL OF EVIDENCE: Level IV Treatment Study.

Surgical Treatment of Esophageal Anastomotic Stricture After Repair of Esophageal Atresia.

BACKGROUND: Anastomotic strictures (AS) after esophageal atresia (EA) repair are common. While most respond to endoscopic therapy, some become refractory and require surgical intervention, for which the outcomes are not well established. METHODS: All EA children with AS who were treated surgically at two institutions (2011-2022) were retrospectively reviewed. Surgical repair was performed for those with AS that were either refractory to endoscopic therapy or clinically symptomatic and undergoing surgery for another indication. Anastomotic leak, need for repeat stricture resection, and esophageal replacement were considered poor outcomes. RESULTS: 139 patients (median age: 12 months, range 1.5 months-20 years; median weight: 8.1 kg) underwent 148 anastomotic stricture repairs (100 refractory, 48 non-refractory) in the form of stricturoplasty (n = 43), segmental stricture resection with primary anastomosis (n = 96), or stricture resection with a delayed anastomosis after traction-induced lengthening (n = 9). With a median follow-up of 38 months, most children (92%) preserved their esophagus, and the majority (83%) of stricture repairs were free of poor outcomes. Only one anastomotic leak occurred in a non-refractory stricture. Of the refractory stricture repairs (n = 100), 10% developed a leak, 9% required repeat stricture resection, and 13% required esophageal replacement. On multivariable analysis, significant risk factors for any type of poor outcome included anastomotic leak, stricture length, hiatal hernia, and patient's weight. CONCLUSIONS: Surgery for refractory AS is associated with inherent yet low morbidity and high rates of esophageal preservation. Surgical repair of non-refractory symptomatic AS at the time of another thoracic operation is associated with excellent outcomes. LEVEL OF EVIDENCE: Level III.

Pharmacogenomics fail to explain proton pump inhibitor refractory esophagitis in pediatric esophageal atresia.

BACKGROUND: Esophagitis is prevalent in patients with esophageal dysmotility despite acid suppression, likely related to poor esophageal clearance. Esophageal atresia (EA) is a classic model of dysmotility where this observation holds true. In adult non-dysmotility populations, failure of esophagitis to respond to proton pump inhibitors (PPI) has been linked to variants in CYP2C19 that influence the activity of the encoded enzyme. It is unknown if CYP2C19 metabolizer phenotype contributes to PPI-refractory, non-allergic esophagitis in EA. METHODS: We performed a cross-sectional study of 314 children with (N = 188) and without (N = 126) EA who were on PPI therapy at the time of endoscopy to evaluate for possible gastroesophageal reflux disease. Patients with eosinophilic esophagitis and/or fundoplication were excluded. Clinical and histology data were collected. Genomic DNA from biopsy samples was genotyped for polymorphisms in CYP2C19. RESULTS: CYP2C19 metabolizer phenotypes were not associated with presence or severity of esophagitis (P = 0.994). In a multivariate logistic regression adjusted for potential confounders, EA was the strongest and only significant predictor of esophagitis (odds ratio 2.72, P = 0.023). Using negative binomial regression, we found that CYP2C19 phenotype was not a significant predictor of eosinophil count in children with PPI-refractory esophagitis. CONCLUSIONS: Patients with EA are significantly more likely to experience PPI-refractory, non-allergic esophagitis than controls regardless of CYP2C19 metabolizer phenotype, suggesting that factors other than CYP2C19 genetics, including dysmotility, are the primary drivers of esophagitis in EA. CYP2C19 genotype failed to predict PPI-refractory, non-allergic esophagitis in both EA and non-EA children.