Effectiveness of intraoperative nerve monitoring in reducing rates of recurrent laryngeal nerve injury in aerodigestive and cardiovascular pediatric surgery.

OBJECTIVE: To investigate the effectiveness of intraoperative nerve monitoring at decreasing vocal fold movement impairment in children undergoing at-risk procedures. BACKGROUND: Children undergoing aerodigestive or cardiovascular procedures are at risk for recurrent laryngeal nerve injury, leading to vocal fold movement impairment. Although intraoperative nerve monitoring has been shown to decrease recurrent laryngeal nerve injury in adults, there is paucity of data in children. METHODS: This was a retrospective, single-center cohort study of children who underwent airway, esophageal, or great vessel surgery between 2018 and 2023. Vocal fold movement impairment was evaluated with pre- and postoperative awake flexible fiberoptic laryngoscopy. Vocal fold movement impairment rates and associated characteristics were compared between those with and without intraoperative nerve monitoring. RESULTS: Among 387 children undergoing 426 at-risk procedures, intraoperative nerve monitoring was used in 72.1% (n = 307) of procedures. Intraoperative nerve monitoring significantly reduced postoperative vocal fold movement impairment compared with those without (11.4% vs 20.2%, P = .019, 43.6% relative risk reduction, number needed to treat: 12). In children with a pre-existing vocal fold movement impairment (n = 79, 18.5%), intraoperative nerve monitoring provided enhanced protection (vocal fold movement impairment 7.8% with intraoperative nerve monitoring compared with 25% without, P = .046, 68.6% relative risk reduction, number needed to treat: 3). Bilateral vocal fold movement impairment was 14 times more likely without intraoperative nerve monitoring (1.8% overall, 0.3% with intraoperative nerve monitoring, 5.6% without; 95% confidence interval 1.6-123.2; P = .006). Increasing intraoperative nerve monitoring use correlated with decreasing vocal fold movement impairment rates year over year (P = .046). Multivariable logistic regression demonstrated intraoperative nerve monitoring to remain significantly associated with reduced risk of vocal fold movement impairment (odds ratio, 0.48; 95% confidence interval, 0.26-0.85; P = .013). CONCLUSION: Intraoperative nerve monitoring in children seems effective at decreasing recurrent laryngeal nerve injury and consequently vocal fold movement impairment. Intraoperative nerve monitoring should be considered in children undergoing cervicothoracic or cardiothoracic procedures, especially in those with preoperative vocal fold movement impairment.

Management Strategies and Outcomes of Distal Congenital Esophageal Strictures in the Setting of Long-gap Esophageal Atresia.

BACKGROUND: The management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies. METHODS: We reviewed children with LGEA + CES surgically treated at two institutions (2018-2024). LGEA repair was performed using the Foker technique (traction-induced esophageal lengthening). A CES strategy was chosen based on preoperative evaluations and intraoperative findings. The configuration and length of the CES were assessed using retrograde flexible esophagoscopy via gastrostomy with contrast fluoroscopy. RESULTS: Eight patients (75% male) with LGEA + CES were treated: Four had type A and four had type B EA. Median gap length was 3.5 cm. Three underwent thoracoscopic esophageal lengthening. After a median follow-up of 18 months (IQR: 9-25), all retained their native esophagus. However, those who had CES resection concurrent with the lengthening process or at the time of EA anastomosis had more challenging perioperative courses: one required additional time on traction and another required esophageal anastomotic stricture resection. CONCLUSIONS: Our experience with LGEA and distal CES emphasizes tailoring surgical approaches to each patient's unique condition, avoiding a one-size-fits-all strategy. However, if the esophageal tissue above the distal CES is in good condition, our preference has shifted towards retaining the CES during traction, performing gentle dilation at anastomosis time, and conducting definitive endoscopic management subsequently. We would caution against making the assumption that salvage of the native esophagus is not possible or that resection of the CES is always needed. LEVEL OF EVIDENCE: Level III.

Botulinum Toxin Enhanced Foker Process for Long Gap Esophageal Atresia.

BACKGROUND: The traction-induced esophageal growth (Foker) process for the treatment of long gap esophageal atresia (LGEA) relies on applying progressive tension to the esophagus to induce growth. Due to its anti-fibrotic and muscle-relaxing properties, we hypothesize that Botulinum Toxin A (BTX) can enhance traction-induced esophageal growth. METHODS: A retrospective two-center cohort study was conducted on children who underwent a BTX-enhanced Foker process for LGEA repair from 2021 to 2023. BTX (10 units/ml, 2 units/kg, per esophageal pouch) was applied at the time of traction initiation. Time on traction, complications, and anastomotic outcomes were compared against historical controls (Foker process without BTX) from 2014 to 2021. RESULTS: Twenty infants (LGEA type A:12, B:4, C:4; 35% reoperative; median [IQR] age 3 [2-5] months), underwent BTX-enhanced Foker process (thoracotomy with external traction: 9; minimally invasive [MIS] multi-staged internal traction: 11). Mean gap lengths were similar between BTX-enhanced external and external traction control patients (mean [SD], 50.6 mm [12.6] vs. 44.5 mm [11.9], p = 0.21). When compared to controls, the BTX-enhanced external traction process was significantly faster (mean [SD], 12.1 [1.6] days vs. 16.6 [13.2] without BTX, p = 0.04) despite similar preoperative gap lengths. There was no difference in time on traction for those undergoing a minimally invasive process. There were no significant differences in complications or anastomotic outcomes in either cohort. CONCLUSION: Botulinum toxin may play a role in accelerating the traction-induced esophageal growth process for LGEA repair. Minimizing time on traction can decrease sedation and paralysis burden while on external traction. Further studies are needed to elucidate the effects of BTX on the esophagus. LEVEL OF EVIDENCE: Level III. TYPE OF STUDY: Retrospective, Two-center, Cohort study.

Clinical Consensus Statement on the Use of Indocyanine Green Fluorescence-guided Surgery in Pediatric Patients.

BACKGROUND AND AIMS: Indocyanine Green Fluorescence (ICG-F)- guided surgery is becoming an increasingly helpful tool in pediatric surgical care. This consensus statement investigates the utility of ICG-F in various pediatric surgical applications, primarily focusing on its evidence base, safety, indications, use across different surgical specialties and dosing strategies. The aim is to establish an international consensus for ICG-F use in pediatric surgery. METHODS: An international panel of 15 pediatric surgeons from 9 countries was assembled. The structured process consisted of a rapid scoping review, iterative discussion sessions, mixed-methods studies with key stakeholders, and voting rounds on individual statements to create draft consensus statements. RESULTS: 100 articles were identified during the review and summarized by application. Based on this condensed evidence, consensus statements were generated after 3 iterative rounds of anonymous voting. Key areas of agreement were quality of evidence, the safety of ICG, pediatric surgical indications, utilization per surgical specialty, and dosing of ICG. CONCLUSION: This consensus statement aims to guide healthcare professionals in managing ICG-F use in pediatric surgical cases based on the best available evidence, key stakeholder consultation, and expert opinions. Despite ICG-F's promising potential, the need for higher-quality evidence, prospective trials, and safety studies is underscored. The consensus also provides a framework for pediatric surgeons to utilize ICG-F effectively. LEVEL OF EVIDENCE: III.

Validation of a PHIS Esophageal Atresia and Tracheoesophageal Fistula Cohort in ICD-10.

OBJECTIVE: Validation of a contemporary International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) congenital esophageal atresia/tracheoesophageal atresia (EA/TEF) cohort in the Pediatric Health Information System (PHIS) database. STUDY DESIGN: Database study, validation. SETTING: Tertiary care center. METHODS: Search methods used to validate an ICD-9-CM EA/TEF cohort in PHIS were modified for ICD-10-CM. A retrospectively and prospectively maintained clinical database at a single high-volume EA/TEF center was used for comparison. Patients treated between October 1, 2015 and July 31, 2022 were included. Searches progressively narrowed the cohort by ICD-10-CM diagnosis codes, expansion to include incorrectly coded as 'iatrogenic, age less than 30 days, and use of at least 1 ICD-10-CM procedure code. Results of PHIS data and institution data were compared for accuracy. RESULTS: The most refined search of PHIS and the EA/TEF clinical database yielded 93 and 84 patients, respectively. The sensitivity was 99% and positive predictive value was 94%. A PHIS search using these methods and encompassing 49 children's hospitals yielded an EA/TEF cohort of 2479 patients. CONCLUSION: We present a validated search method in the PHIS database to identify a high-fidelity cohort of EA/TEF patients for multi-institutional study. We have demonstrated that a carefully maintained clinical database may be used to validate cohorts in PHIS. This cohort allows for improved practice variability and outcomes study of EA/TEF patients. Similar methods may be employed to generate other rare disease cohorts in PHIS. LEVEL OF EVIDENCE: Level 4.

Outcomes of surgical treatment of tracheobronchomalacia in children.

BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. MATERIALS AND METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data. RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. CONCLUSION: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

Lack of standardization and faculty development in pediatric colonoscopy: A qualitative study.

A standard curriculum for pediatric colonoscopy training has neither been required nor universally implemented in North American fellowship programs. This qualitative study assessed the needs of colonoscopy training in pediatric gastroenterology to determine the standardized components of procedural teaching. Focus groups with pediatric gastroenterology attendings, fellows, procedural nurses, and interviews with advanced endoscopists, all practicing at a single institution, were conducted between March and June 2018. Data were analyzed using thematic analysis principles. Four themes emerged: (1) lack of standardization of colonoscopy performance, (2) lack of professional development of procedure teaching skills, (3) need for teaching behaviors that promote learner's performance, and (4) barriers to effective teaching and learning. A conceptual framework was created for developing a standardized "train-the-trainer" curriculum. Our needs assessment supports expansion of efforts to make this comprehensive training available to all pediatric gastroenterologists involved in procedure teaching.

Defining Practice Readiness in Pediatric Surgery Fellowship: A Qualitative Analysis.

INTRODUCTION: While competency-based training is at the forefront of educational innovation in General Surgery, Pediatric Surgery training programs should not wait for downstream changes. There is currently no consensus on what it means for a pediatric surgery fellow to be "practice-ready". In this study, we aimed to provide a framework for better defining competency and practice readiness in a way that can support the Milestones system and allow for improved assessment of pediatric surgery fellows. METHODS: For this exploratory qualitative study, we developed an interview guide with nine questions focused on how faculty recognize competency and advance autonomy among pediatric surgery fellows. Demographic information was collected using an anonymous online survey platform. We iteratively reviewed data from each interview to ensure adequate information power was achieved to answer the research question. We used inductive reasoning and thematic analysis to determine appropriate codes. Additionally, the Dreyfus model was used as a framework to guide interpretation and contextualize the responses. Through this method, we generated common themes. RESULTS: A total of 19 pediatric surgeons were interviewed. We identified four major themes from 127 codes that practicing pediatric surgeons associate with practice-readiness of a fellow: skill-based competency, the recognition and benefits of struggle, developing expertise and facilitating autonomy, and difficulties in variability of evaluation. While variability in evaluation is not typically included in the concept of practice readiness, assessment and evaluation were described by study participants as essential aspects of how practicing pediatric surgeons perceive practice readiness and competency in pediatric surgery fellows. Competency was further divided into interpersonal versus technical skills. Sub-themes within struggle included personal and professional struggle, benefits of struggle and how to identify and assist those who are struggling. Autonomy was commonly stated as variable based on the attending. CONCLUSION: Our analysis yielded several themes associated with practice readiness of pediatric surgery fellows. We aim to further refine our list of themes using the Dreyfus Model as our interpretive framework and establish consensus amongst the community of pediatric surgeons in order to define competency and key elements that make a fellow practice-ready. Further work will then focus on establishing assessment metrics and educational interventions directed at achieving such key elements.

The History and Legacy of the Foker Process for the Treatment of Long Gap Esophageal Atresia.

Historically, children afflicted with long gap esophageal atresia (LGEA) had few options, either esophageal replacement or a life of gastrostomy feeds. In 1997, John Foker from Minnesota revolutionized the treatment of LGEA. His new procedure focused on "traction-induced growth" when the proximal and distal esophageal segments were too far apart for primary repair. Foker's approach involved placement of pledgeted sutures on both esophageal pouches connected to an externalized traction system which could be serially tightened, allowing for tension-induced esophageal growth and a delayed primary repair. Despite its potential, the Foker process was received with criticism and disbelief, and to this day, controversy remains regarding its mechanism of action - esophageal growth versus stretch. Nonetheless, early adopters such as Rusty Jennings of Boston embraced Foker's central principle that "one's own esophagus is best" and was instrumental to the implementation and rise in popularity of the Foker process. The downstream effects of this emphasis on esophageal preservation would uncover the need for a focused yet multidisciplinary approach to the many challenges that EA children face beyond "just the esophagus", leading to the first Esophageal and Airway Treatment Center for children. Consequently, the development of new techniques for the multidimensional care of the LGEA child evolved such as the posterior tracheopexy for associated tracheomalacia, the supercharged jejunal interposition, as well as minimally invasive internalized esophageal traction systems. We recognize the work of Foker and Jennings as key catalysts of an era of esophageal preservation and multidisciplinary care of children with EA.

Diagnostic Accuracy of Laryngeal Ultrasound for Evaluating Vocal Fold Movement Impairment in Children.

PURPOSE: Vocal fold movement impairment (VFMI) secondary to recurrent laryngeal nerve (RLN) injury is a common source of morbidity after pediatric cervical, thoracic, and cardiac procedures. Flexible laryngoscopy (FL) is the gold standard to diagnose VFMI yet can be challenging to perform and/or risks possible clinical decompensation in some children and is an aerosolizing procedure. Laryngeal ultrasound (LUS) is a potential non-invasive alternative, but limited data exists in the pediatric surgical population regarding its efficacy. We aimed to investigate the diagnostic accuracy of LUS compared to FL in evaluating VFMI. METHODS: A prospective, single-center, single-blinded (rater) cohort study was undertaken on perioperative pediatric patients at risk for RLN injury. Patients underwent FL and LUS. Cohen's kappa was used to determine chance-corrected agreement. RESULTS: Between 2021 and 2023, 85 paired evaluations were performed with patients having a median (IQR) age of 10 (4, 42) months and weight of 7.5 (5.4, 13.4) kilograms. The prevalence of VFMI was 27.1%. Absolute agreement between evaluations was 98.8% (kappa 0.97, 95% CI: 0.91-1.00, P < 0.001). The sensitivity and specificity of LUS in detecting VFMI was 95.7% and 100%, yielding a positive predictive value (PPV) of 100% and negative predictive value (NPV) of 98.4% (95% CI: 90-100%). Diagnostic accuracy was 98.8% (95% CI: 93-100%). CONCLUSION: LUS is a highly accurate modality in evaluating VFMI in children. While FL remains the gold standard for diagnosis, LUS offers a low-risk screening modality for children at risk for VFMI such that only those with an abnormal LUS or presence of clinical symptoms discordant with LUS findings should undergo FL. TYPE OF STUDY: Prospective, single-center, single blinded (rater), cohort study. LEVEL OF EVIDENCE: Level II.

How Many Operative Performance Ratings Does a Pediatric Surgery Fellow Need to Be Deemed Practice Ready?

PURPOSE: Identifying the number of cases required for a fellow to achieve competence has been challenging. Workplace-based assessment (WBA) systems make collecting performance data practical and create the opportunity to translate WBA ratings into probabilistic statements about a fellow's likelihood of performing to a given standard on a subsequent assessment opportunity. METHODS: We compared data from two pediatric surgery training programs that used the performance rating scale from the Society for Improving Medical Professional Learning (SIMPL). We used a Bayesian generalized linear mixed effects model to examine the relationship past and future performance for three procedures: Laparoscopic Inguinal Hernia Repair, Laparoscopic Gastrostomy Tube Placement, and Pyloromyotomy. RESULTS: For site one, 26 faculty assessed 9 fellows on 16 procedures yielding 1094 ratings, of which 778 (71%) earned practice-ready ratings. For site two, 25 faculty rated 3 fellows on 4 unique procedures yielding 234 ratings of which 151 (65%) were deemed practice-ready. We identified similar model-based future performance expectations, with prior practice-ready ratings having a similar average effect across both sites (Site one, B = 0.25; Site two, B = 0.25). Similar prior practice-ready ratings were needed for Laparoscopic G-Tube Placement (Site one = 13; Site two = 14), while greater differences were observed for Laparoscopic Inguinal Hernia Repair (Site one = 10; Site two = 15) and Pyloromyotomy (Site one = 10; Site two = 15). CONCLUSION: Our approach to modeling operative performance data is effective at determining future practice readiness of pediatric surgery fellows across multiple faculty and fellow groups. This method could be used to establish minimum case number requirements. TYPE OF STUDY: Original manuscript, Study of Diagnostic Test. LEVEL OF EVIDENCE: II.

Primary Posterior Tracheopexy at Time of Esophageal Atresia Repair Significantly Reduces Respiratory Morbidity.

PURPOSE: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair. METHODS: Review of all newborn primary EA/TEF repairs (2016-2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. RESULTS: Among 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs -1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). CONCLUSION: Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. LEVEL OF EVIDENCE: Level III.

Retrospective analysis of neurological findings in esophageal atresia: Allostatic load of disease complexity, cumulative sedation, and anesthesia exposure.

BACKGROUND: There is limited knowledge regarding the impact of perioperative critical care on frequency of neurological imaging findings following esophageal atresia (EA) repair. METHODS: This is a retrospective study of infants (n = 70) following EA repair at a single institution (2009-2020). Sex, gestational age at birth, type of surgical repair, underlying disease severity, and frequency of neurologic imaging findings were obtained. We quantified the length of postoperative pain/sedation treatment and anesthesia exposure in the first year of life. Data were presented as numerical sums and percentages, while associations were measured using Spearman's Rho. RESULTS: Vertebral/spinal cord imaging was performed in all infants revealing abnormalities in 44% (31/70). Cranial/brain imaging findings were identified in 67% (22/33) of infants in the context of clinically indicated imaging (47%; 33/70). Long-gap EA patients (n = 16) received 10 times longer postoperative pain/sedation treatment and twice the anesthesia exposure compared with short-gap EA patients (n = 54). The frequency of neurologic imaging findings did not correlate with underlying disease severity scores, length of pain/sedation treatment, or cumulative anesthesia exposure. Lack of associations between clinical measures and imaging findings should be interpreted with caution given possible underestimation of cranial/brain findings. CONCLUSIONS: We propose that all infants with EA undergo brain imaging in addition to routine spinal imaging given the high burden of abnormal brain/cranial findings in our cohort. Quantification of pain/sedation and anesthesia exposure in long-gap EA patients could be used as indirect markers in future studies assessing the risk of neurological sequelae as evidenced by early abnormalities on brain imaging.

Bronchoscopic Localization of Tracheoesophageal Fistula in Newborns with Esophageal Atresia: Intubate Above or Below the Fistula?

PURPOSE: In neonates with suspected type C esophageal atresia and tracheoesophageal fistula (EA/TEF) who require preoperative intubation, some texts advocate for attempted "deep" or distal-to-fistula intubation. However, this can lead to gastric distension and ventilatory compromise if a distal fistula is accidently intubated. This study examines the distribution of tracheoesophageal fistula locations in neonates with type C EA/TEF as determined by intraoperative bronchoscopy. METHODS: This was a single-center retrospective review of neonates with suspected type C EA/TEF who underwent primary repair with intraoperative bronchoscopy between 2010 and 2020. Data were collected on demographics and fistula location during bronchoscopic evaluation. Fistula location was categorized as amenable to blind deep intubation (>1.5 cm above carina) or not amenable to blind deep intubation intubation (≤1.5 cm above carina or carinal). RESULTS: Sixty-nine neonates underwent primary repair of Type C EA/TEF with intraoperative bronchoscopy during the study period. Three patients did not have documented fistula locations and were excluded (n = 66). In total, 49 (74 %) of patients were found to have fistulas located ≤1.5 cm from the carina that were not amenable to blind deep intubation. Only 17 patients (26 %) had fistulas >1.5 cm above carina potentially amenable to blind deep intubation. CONCLUSIONS: Most neonates with suspected type C esophageal atresia and tracheoesophageal fistula have distal tracheal and carinal fistulas that are not amenable to blind deep intubation. LEVEL OF EVIDENCE: Level III.

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.

Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review.

Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related aerodigestive compression syndromes that also includes innominate artery compression syndrome, dysphagia lusoria, aortic arch anomalies, and aneurysms of either the aorta or pulmonary artery. Additionally, post-surgical airway compression is a distinct entity in itself. The approach to the diagnosis and management of these varied phenomena has been streamlined by the multidisciplinary team at Boston Children's Hospital. Echocardiography, computed tomographic angiography, esophagram, and three-phase dynamic bronchoscopy are routinely performed in these patients in order to produce a comprehensive understanding of the unique anatomic challenges that each patient presents. Adjunctive diagnostic techniques include modified barium swallow, routine preoperative and postoperative screening of the vocal cords, and radiographic identification of the artery of Adamkiewicz. Beyond the vascular reconstruction, which ranges from subclavian-to-carotid transposition to descending aortic translocation, we liberally apply tracheobronchopexy and rotational esophagoplasty to relieve respiratory and esophageal symptoms. Due to the heightened risk for recurrent laryngeal nerve injury, intraoperative recurrent laryngeal nerve monitoring has become routine in these cases. The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result.

Esophageal Surveillance Practices in Esophageal Atresia Patients: A Survey by the Eastern Pediatric Surgery Network.

INTRODUCTION: Endoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN). METHODS: An anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices. RESULTS: The response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines. CONCLUSIONS: Our survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care. LEVEL OF EVIDENCE: This is a prognosis study with level 4 evidence.