Genomic Contributors to Esophageal Atresia and Tracheoesophageal Fistula: A 12 Year Retrospective Review.

OBJECTIVE: To evaluate genetic testing utilization and diagnostic yield in infants with esophageal atresia (EA)/tracheoesophageal fistula (TEF) over the past 12 years to inform future practices and individualize prognostication and management. STUDY DESIGN: A retrospective cohort study was performed for all infants with EA or EA/TEF hospitalized between January 2011 and January 2023 at a quaternary children's hospital. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed. RESULTS: There were 212 infants who were classified as follows: 1) complex/syndromic with EA/TEF plus an additional major anatomic anomaly (n = 114, of which 74 met VACTERL criteria); 2) isolated/nonsyndromic EA/TEF (n = 88) and 3) isolated/nonsyndromic EA (n = 10). A range of genetic tests were sent with varying diagnostic rates including karyotype analysis in 12 (all with complex/syndromic phenotypes and all positive), chromosomal microarray analysis in 189 (114 of whom were complex/syndromic with an overall diagnostic rate of 3/189), single gene testing for CHD7 in 18 (4 positive), and exome analysis in 37 complex/syndromic patients (8 positive). CONCLUSIONS: EA/TEF with and without additional anomalies is genetically heterogeneous with a broad range of associated phenotypes. While the genetic etiology of EA/TEF with or without VACTERL remains largely unknown, genome wide testing (exome or genome) including copy number analysis is recommended over chromosomal microarray testing. We anticipate that expanded genetic/genomic testing modalities such as RNA sequencing and tissue specific molecular testing are needed in this cohort to improve our understanding of the genomic contributors to EA/TEF.

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 esophageal mucosal bridges in children with esophageal atresia.

BACKGROUND AND STUDY AIMS: Esophageal mucosal bridge (EMB) may be diagnosed at the anastomotic site in children operated on for esophageal atresia (EA) but so far only a few cases (n = 4) have been reported. This study aimed to characterize EMB in children with EA, risk factors, and treatment. PATIENTS AND METHODS: This retrospective multicenter study recorded patient's characteristics, EMB diagnosis circumstances, endoscopic management, follow-up, and EMB recurrence in children with EA aged less than 18 years, compared with paired EA patients without EMB. RESULTS: Thirty patients were included (60% male, 90% EA/tracheoesophageal fistula, 43% associated malformations). Compared to 44 paired controls, EMB was associated with a history of nasogastric tube feeding (31% vs. 9.1%, p = 0.02) and severe gastroesophageal reflux disease (history of fundoplication: 41.4% vs. 13.6%, p < 0.01). 77% had symptoms (food impaction and/or dysphagia). Endoscopic management was performed in 53% of patients (83% electrocoagulation) with no technical difficulties or complications. 80% of the symptomatic patients with EMB improved after endoscopic treatment, independently of anastomotic stricture dilatation or not. CONCLUSION: EMB endoscopic management by electrocoagulation is safe and often leads to symptom improvement.

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.

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.

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.

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 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.

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.

Cautionary tales in the use of magnets for the treatment of long gap esophageal atresia.

BACKGROUND: The use of magnets for the treatment of long gap esophageal atresia or "magnamosis" is associated with increased incidence of anastomotic strictures; however, little has been reported on other complications that may provide insight into refining selection criteria for appropriate use. METHODS: A single institution, retrospective review identified three cases referred for treatment after attempted magnamosis with significant complications. Their presentation, imaging, management, and outcomes were reviewed. RESULTS: All three patients had prior cervical or thoracic surgery to close a tracheoesophageal fistula prior to magnamosis, creating scar tissue that can prevent magnet induced esophageal movement, leading to either magnets not attracting enough or erosion into surrounding structures. Two patients had a reported four centimeter esophageal gap prior to attempted magnamosis, both failing to achieve esophageal anastomosis, suggesting that these gaps were either measured on tension with variability in gap measurement technique, or that the esophageal segments were fixed in position from scar tissue and unable to elongate. One patient had severe tracheobronchomalacia requiring tracheostomy, with improvement in his airway after eventual tracheobronchopexies, highlighting that magnamosis does not address comorbidities often associated with this patient population. CONCLUSIONS: We propose the following inclusion criteria and considerations for magnamosis: an esophageal gap truly less than four centimeters off tension with standardized measurement across centers, cautious use with a history of prior thoracic or cervical esophageal surgery, no associated tracheobronchomalacia or great vessel anomaly that would benefit from concurrent repair, and ideally to be used in centers equipped to manage potential complications. LEVEL OF EVIDENCE: Level IV treatment study.

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.

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.

Evolution, lessons learned, and contemporary outcomes of esophageal replacement with jejunum for children.

BACKGROUND: The jejunal interposition is our preferred esophageal replacement route when the native esophagus cannot be reconstructed. We report the evolution of our approach and outcomes. METHODS: The study was a single-center retrospective review of children undergoing jejunal interposition for esophageal replacement. Outcomes were compared between historical (2010-2015) and contemporary cohorts (2016-2019). RESULTS: Fifty-five patients, 58% male, median age 4 years (interquartile range 2.4-8.3), with history of esophageal atresia (87%), caustic (9%) or peptic (4%) injury, underwent a jejunal interposition (historical cohort n = 14; contemporary cohort n = 41). Duration of intubation (11 vs 6 days; P = .01), intensive care unit (22 vs 13 days; P = .03), and hospital stay (50 vs 27 days; P = .004) were shorter in the contemporary cohort. Anastomotic leaks (7% vs 5%; P = .78), anastomotic stricture resection (7% vs 10%; P = .74), and need for reoperation (57% vs 46%; P = .48) were similar between cohorts. Most reoperations were elective conduit revisions. Microvascular augmentation, used in 70% of cases, was associated with 0% anastomotic leaks vs 18% without augmentation; P = .007. With median follow-up of 1.9 years (interquartile range 1.1, 3.8), 78% of patients are predominantly orally fed. Those with preoperative oral intake were more likely to achieve consistent postoperative oral intake (87.5% vs 64%; P = .04). CONCLUSION: We have made continuous improvements in our management of patients undergoing a jejunal interposition. Of these, microvascular augmentation was associated with no anastomotic leaks. Despite its complexity and potential need for conduit revision, the jejunal interposition remains our preferred esophageal replacement, given its excellent long-term functional outcomes in these complex children who have often undergone multiple procedures before the jejunal interposition.

Contemporary outcomes of the Foker process and evolution of treatment algorithms for long-gap esophageal atresia.

BACKGROUND: Esophageal growth using the Foker process (FP) for long-gap esophageal atresia (LGEA) has evolved over time. METHODS: Contemporary LGEA patients treated from 2014-2020 were compared to historical controls (2005 to <2014). RESULTS: 102 contemporary LGEA patients (type A 50%, B 18%, C 32%; 36% prior anastomotic attempt; 20 with esophagostomy) underwent either primary repair (n=23), jejunal interposition (JI; n = 14), or Foker process (FP; n = 65; 49 primary [p], 16 rescue [r]). The contemporary p-FP cohort experienced significantly fewer leaks on traction (4% vs 22%), bone fractures (2% vs 22%), anastomotic leak (12% vs 37%), and Foker failure (FP→JI; 0% vs 15%), when compared to historical p-FP patients (n = 27), all p ≤ 0.01. Patients who underwent a completely (n = 11) or partially (n = 11) minimally invasive FP experienced fewer median days paralyzed (0 vs 8 vs 17) and intubated (9 vs 15 vs 25) compared to open FP patients, respectively (all p ≤ 0.03), with equivalent leak rates (18% vs 9% vs 26%, p = 0.47). At one-year post-FP, most patients (62%) are predominantly orally fed. CONCLUSION: With continued experience and technical refinements, the Foker process has evolved with improved outcomes, less morbidity and maximal esophageal preservation.

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.