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.

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.

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.

Qualitative features of esophageal fluorescence angiography and anastomotic outcomes in children.

BACKGROUND: Indocyanine green (ICG) is commonly used to assess perfusion, but quality defining features are lacking. We sought to establish qualitative features of esophageal ICG perfusion assessments, and develop an esophageal anastomotic scorecard to risk-stratify anastomotic outcomes. METHODS: Single institution, retrospective analysis of children with an intraoperative ICG perfusion assessment of an esophageal anastomosis. Qualitative perfusion features were defined and a perfusion score developed. Associations between perfusion and clinical features with poor anastomotic outcomes (PAO, leak or refractory stricture) were evaluated with logistic and time-to-event analyses. Combining significant features, we developed and tested an esophageal anastomotic scorecard to stratify PAO risk. RESULTS: From 2019 to 2021, 53 children (median age 7.4 months) underwent 55 esophageal anastomoses. Median (IQR) follow-up was 14 (10-19.9) months; mean (SD) perfusion score was 13.2 (3.4). Fifteen (27.3%) anastomoses experienced a PAO and had significantly lower mean perfusion scores (11.3 (3.3) vs 14.0 (3.2), p = 0.007). Unique ICG perfusion features, severe tension, and primary or rescue traction-induced esophageal lengthening [Foker] procedures were significantly associated with PAO on both logistic and Cox regression. The scorecard (range 0-7) included any Foker (+2), severe tension (+1), no arborization on either segment (+1), suture line hypoperfusion >twice expected width (+2), and segmental or global areas of hypoperfusion (+1). A scorecard cut-off >3 yielded a sensitivity of 73% and specificity of 93% (AUC 0.878 [95%CI 0.777 to 0.978]) in identifying a PAO. CONCLUSIONS: A scoring system comprised of qualitative ICG perfusion features, tissue quality, and anastomotic tension can help risk-stratify esophageal anastomotic outcomes accurately. LEVELS OF EVIDENCE: Diagnostic - II.

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.

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.

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.

Predictors and Outcomes of Fully Covered Stent Treatment for Anastomotic Esophageal Strictures in Esophageal Atresia.

BACKGROUND AND AIMS: Anastomotic strictures following surgical repair is one of the most common complications in esophageal atresia (EA). The utility of esophageal stenting to treat anastomotic esophageal strictures in pediatrics is unclear. Our primary aim was to evaluate whether esophageal stenting, in conjunction with dilation and other endoscopic therapies, prevented surgical stricture resection (SR). Our secondary aims were to evaluate predictors of successful esophageal stenting and evaluate adverse events from stent placement. METHODS: A retrospective review of pediatric patients with EA complicated by esophageal strictures was performed. The change in stricture diameter in millimeters from the time of stent removal to subsequent endoscopy was defined as delta diameter (ΔD). A receiver operating characteristic (ROC) curve analysis was performed to determine the discriminatory ability of ΔD. Youden J index was used to identify optimal cutoff-point in predicting stent success. A univariate and multivariate analysis were done to assess predictors of success. RESULT: Forty-nine esophageal anastomoses were stented to treat esophageal strictures. Stents prevented SR in 41% of patients. ROC curve analysis utilizing Youden J index identified ΔD of ≤4 mm (area under the curve = 0.790; 95% confidence interval: 0.655-0.924; P < 0.001) as the optimal cutoff point in differentiating stent success. The most common adverse events were erosions/ulcerations, granulation tissue formation, and vomiting/retching. CONCLUSION: Stent therapy in preventing SR at the site of EA repair was successful in 41% in our population with good long term follow-up. The most significant predictor of success in this study was the change in luminal diameter (≤4 mm) at initial poststent follow-up.

Predictors of anti-reflux procedure failure in complex esophageal atresia patients.

BACKGROUND: Anti-reflux procedures (ARP) in esophageal atresia (EA) patients can be challenging and prone to failure. These challenges become more evident with increasing complexity of EA. We sought to determine predictors of ARP failure in complex EA patients. METHODS: Single-institution retrospective review of complex EA patients (e.g. long-gap EA, esophageal strictures, hiatal hernia, and reoperative ARP) who underwent an ARP from 2002 to 2019. ARP failure was defined as hiatal hernia recurrence, wrap migration/loosening, or need for reoperation. Predictors of failure were evaluated using univariate and multivariable time-to-event analysis. RESULTS: 121 patients underwent 140 ARP at a median age of 13.5 months (IQR 7, 26.5). Nissen fundoplication (89%) was the most common ARP. Mesh (bovine pericardium) reinforcement was used in 41% of the patients. Median follow-up was 3.2 years (IQR 0.9, 5.8); 44 instances of ARP failure occurred (31%), though only 20 (14%) required reoperation. Median time to failure was 8.7 months (IQR 3.2, 25). Though fewer mesh-reinforced ARP failed (21% with vs 39% without, p = 0.02), on multivariable analysis only partial fundoplication (aHR 2.22 [95% CI 1.01-4.78]) and minimally invasive repair (aHR 2.57 [95% CI 1.12-6.01]) were significant predictors of ARP failure. CONCLUSION: In our practice of complex EA patients, where ARP fail in nearly one third of cases, a Nissen fundoplication performed via laparotomy provided the lowest risk of ARP failure.

Initial Esophageal Anastomosis Diameter Predicts Treatment Outcomes in Esophageal Atresia Patients With a High Risk for Stricture Development.

Background and Aims: Children with esophageal atresia (EA) who undergo surgical repair are at risk for anastomotic stricture, which may need multiple dilations or surgical resection if the stricture proves refractory to endoscopic therapy. To date, no studies have assessed the predictive value of anastomotic diameter on long-term treatment outcomes. Our aim was to evaluate the relationship between anastomotic diameter in the early postoperative period and need for frequent dilations and stricture resection within 1 year of surgical repair. Methods: A retrospective chart review was performed of patients who had EA repair or stricture resection (SR). Medical records were reviewed to evaluate the diameter of the anastomosis at the first endoscopy after surgery, number and timing of dilations needed to treat the anastomotic stricture, and need for stricture resection. A generalized estimating equations (GEE) modeling with a logit link and binomial family was done to analyze the relationship between initial endoscopic anastomosis diameter and the outcome of needing a stricture resection. Median regression was implemented to estimate the association between number of dilations needed based on initial diameter. Results: A total of 121 patients (56 females) with a history of EA (64% long-gap EA) were identified who either underwent Foker repair at 46% or stricture resection with end-to-end esophageal anastomosis at 54%. The first endoscopy occurred a median of 22 days after surgery. Among all cases, a narrower anastomoses were more likely to need stricture resection with an OR of 12.9 (95% CI, 3.52, 47; p < 0.001) in patients with an initial diameter of <3 mm. The number of dilations that patients underwent also decreased as anastomotic diameter increased. This observation showed a significant difference when comparing all diameter categories when looking at all surgeries taken as a whole (p < 0.008). Conclusion: Initial anastomotic diameter as assessed via endoscopy performed after high-risk EA repair predicts which patients will require more esophageal dilations as well as the likelihood for stricture resection. This data may serve to stratify patients into different endoscopic treatment plans.

Effect of Posterior Tracheopexy on Risk of Recurrence in Children after Recurrent Tracheo-Esophageal Fistula Repair.

BACKGROUND: A recurrent tracheo-esophageal fistula can complicate esophageal atresia and tracheo-esophageal fistula (TEF) repair in children. Therapeutic approaches and the rate of recurrence vary widely. Most reports are limited by small cohorts and short-term follow-up, and rates of re-recurrence are substantial, making it difficult to select the treatment of choice. We aimed to review our experience with the treatment of recurrent TEF using posterior tracheopexy, focusing on operative risks and long-term outcomes. STUDY DESIGN: We conducted a retrospective review of patients with esophageal atresia TEF with recurrent TEF treated at 2 institutions from 2011 to 2020. We approach recurrent TEFs surgically. Once the TEF is divided and repaired, the membranous trachea is sutured to the anterior longitudinal ligament of the spine (posterior tracheopexy) and the esophagus is rotated into the right chest (rotational esophagoplasty), separating the suture lines widely. To detect re-recurrence, patients undergo endoscopic surveillance during follow-up. RESULTS: Sixty-two patients with a recurrent TEF were surgically treated (posterior tracheopexy/rotational esophagoplasty) at a median age of 14 months. All had significant respiratory symptoms. On referral, 24 had earlier failed endoscopic and/or surgical attempts at repair. Twenty-nine required a concomitant esophageal anastomotic stricturoplasty or stricture resection. Postoperative morbidity included 3 esophageal leaks, and 1 transient vocal cord dysfunction. We have identified no recurrences, with a median follow-up of 2.5 years, and all symptoms have resolved. CONCLUSIONS: The surgical treatment of recurrent TEFs that incorporates a posterior tracheopexy and rotational esophagoplasty is highly effective for preventing re-recurrence with low perioperative morbidity.

Prophylactic negative vacuum therapy of high-risk esophageal anastomoses in pediatric patients.

BACKGROUND: Esophageal anastomoses are at risk for leak or stricture. Negative pressure vacuum-assisted closure (VAC) therapy is used to treat leak. We hypothesized that a prophylactic VAC (pEVAC) at the time of new anastomosis may lead to fewer leaks and strictures. METHODS: Single center retrospective case-control study of patients undergoing high-risk esophageal anastomoses between July 2015 and January 2019. Outcomes of leak and long-term anastomotic failure (refractory stricture requiring surgery) were compared between groups. RESULTS: Sixteen patients had a pEVAC placed during LGEA repair (N = 10) or stricture resection (N = 6). Of pEVAC cases, 3 (N = 1 Foker, N = 2 stricture resections) experienced leak (18.8%). In comparison, leak occurred in 9/41 (22%) Foker patients and in 1/20 (5%) stricture resections without pEVAC, all p > 0.05. Long-term anastomotic failure was more common in the pEVAC cohort versus controls (56.3% versus 11.5%, p < 0.001). CONCLUSIONS: Prophylactic EVAC placement does not appear to reduce leak and is associated with significantly greater odds of long-term anastomotic failure. Further device refinement could improve its potential role in prophylaxis of high-risk anastomoses, but future research is needed to better understand optimal patient selection, device design, and duration of pEVAC therapy.

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.

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.

Endoscopic incisional therapy and other novel strategies for effective treatment of congenital esophageal stenosis.

BACKGROUND & AIMS: Congenital esophageal stenosis (CES) is an inborn condition of the esophagus that can be refractory to endoscopic dilation. Surgical intervention is not curative, with patients experiencing frequent ongoing need for therapy for anastomotic stricture postoperatively. We hypothesized that novel methods of endoscopic CES management including endoscopic incisional therapy (EIT) would lead to less surgical intervention. METHODS: We retrospectively reviewed the medical records of all patients with CES treated by our tertiary care center who had at least one endoscopy between July 2007 and July 2019. Statistical comparison of cohorts who underwent advanced endoscopic therapy involving EIT versus traditional endoscopic therapy with balloon dilation was performed. Primary outcome measure was need for surgical intervention. RESULTS: Thirty-six patients with CES met inclusion criteria. Thirty-four ever had at least one endoscopic intervention such as balloon dilation, steroid injection, stenting, and/or endoscopic incisional therapy (EIT) at their CES. Esophageal vacuum assisted closure (EVAC) was used for treatment or prevention of esophageal leak. Odds of surgical intervention were significantly lower in the group who received therapeutic endoscopy with EIT (odds ratio (OR) 0.1; p = 0.007). Clinical feeding outcomes were similar in the endoscopic and surgical management groups. Odds of complications after therapeutic endoscopies involving EIT were significantly greater than those without EIT (odds ratio 6.39; 95% confidence interval (2.34, 17.44); p < 0.001), though our rates of esophageal leak significantly decreased over time as our use of EVAC increased (Spearman's ρ = -0.884; p = 0.004). CONCLUSION: Complementary endoscopic techniques such as EIT broaden the toolbox of the treating physician and may allow for avoidance of surgery in CES. LEVEL OF EVIDENCE: Level III.

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.

Protein-Losing Enteropathy in the Setting of Iron Deficiency Anemia: A Case Series.

Protein-losing enteropathy (PLE) in the setting of severe iron deficiency anemia (IDA) and excessive cow milk intake is an uncommonly recognized phenomenon. Here, we describe a series of 7 toddlers who presented for evaluation of edema in the setting of excessive cow milk intake between November 2016 and January 2019. Laboratory studies in each patient were consistent with IDA and hypoalbuminemia with evidence of PLE. Diagnostic evaluation and treatment of each patient differed, although all were instructed to restrict cow milk and provided with oral iron supplementation. The edema had resolved, and the IDA had improved in all 7 patients by the time of their follow-up outpatient appointments. Iron deficiency and PLE should be considered in patients who present with anasarca.

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.