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.

A 20-month-old boy presented with a 2-week history of pallor and progressive abdominal distention. Nutritional history revealed long-standing excessive cow milk intake. He was subsequently found to be profoundly iron deficient and hypoproteinemic, with an elevated fecal α-1-antitrypsin level and occult blood positive stool, consistent with protein-losing enteropathy. He was treated with cow milk restriction and oral iron supplements, which resulted in resolution of his edema and laboratory anomalies. While small numbers of previous case reports have described the potential association between excessive cow milk intake and severe iron deficiency and protein-losing enteropathy, this constellation of clinical symptoms is infrequently recognized in clinical practice. As iron deficiency is recognized as the most common nutritional deficiency in the United States, it is important to keep excessive cow milk intake in mind when evaluating young children presenting with severe iron deficiency and protein-losing enteropathy.

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.

Summary 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.

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.