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.

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.

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.

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.

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.

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 utility 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. METHODS: The medical records of all EA patients who underwent at least one surveillance endoscopy between March 2004 and March 2023 at an international EA referral center were retrospectively reviewed. The primary outcome was 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 1473 surveillance endoscopies spanning 3687 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 (N = 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 (N=30 refractory reflux, N=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.

Screening for Vocal Fold Movement Impairment in Children Undergoing Esophageal and Airway Surgery.

INTRODUCTION: Children undergoing cervical and/or thoracic operations are at risk for recurrent laryngeal nerve injury, resulting in vocal fold movement impairment (VFMI). Screening for VFMI is often reserved for symptomatic patients. OBJECTIVE: Identify the prevalence of VFMI in screened preoperative patients prior to an at-risk operation to evaluate the value of screening all patients at-risk for VFMI, regardless of symptoms. METHODS: A single center, retrospective review of all patients undergoing a preoperative flexible nasolaryngoscopy between 2017 and 2021, examining the presence of VFMI and associated symptoms. RESULTS: We evaluated 297 patients with a median (IQR) age of 18 (7.8, 56.3) months and a weight of 11.3 (7.8, 17.7) kilograms. Most had a history of esophageal atresia (EA, 60%), and a prior at-risk cervical or thoracic operation (73%). Overall, 72 (24%) patients presented with VFMI (51% left, 26% right, and 22% bilateral). Of patients with VFMI, 47% did not exhibit the classic symptoms (stridor, dysphonia, and aspiration) of VFMI. Dysphonia was the most prevalent classic VFMI symptom, yet only present in 18 (25%) patients. Patients presenting with a history of at-risk surgery (OR 2.3, 95%CI 1.1, 4.8, p = 0.03), presence of a tracheostomy (OR 3.1, 95%CI 1.0, 10.0, p = 0.04), or presence of a surgical feeding tube (OR 3.1, 95%CI 1.6, 6.2, p = 0.001) were more likely to present with VFMI. CONCLUSION: Routine screening for VFMI should be considered in all at-risk patients, regardless of symptoms or prior operations, particularly in those with a history of an at-risk surgery, presence of tracheostomy, or a surgical feeding tube. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3564-3570, 2023.

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.

Pediatric button battery ingestion: A single center experience and risk score to predict severe outcomes.

PURPOSE: The purpose of this study was to analyze the management and outcomes of primary button battery ingestions and their sequelae at a single high-volume center, and to propose a risk score to predict the likelihood of a severe outcome. METHODS: The medical record was queried for all patients under 21 years old evaluated at our institution for button battery ingestion from 2008 to 2021. A severe outcome was defined as having at least one of the following: deep/circumferential mucosal erosion, perforation, mediastinitis, vascular or airway injury/fistula, or development of esophageal stricture. From a selection of clinically relevant factors, logistic regression determined predictors of a severe outcome, which were incorporated into a risk model. RESULTS: 143 patients evaluated for button battery ingestion were analyzed. 24 (17%) had a severe outcome. The independent predictors of a severe outcome in multivariate analysis were location of battery in the esophagus on imaging (96%), battery size >/ = 2 cm (95%), and presence of any symptoms on presentation (96%), with P < 0.001 in all cases. Predicted probability of a severe outcome ranged from 88% when all three risk factors were observed, to 0.3% when none were present. CONCLUSION: We report the presentation, management, and complication profiles of a large cohort of BB ingestions treated at a single institution. A risk score to predict severe outcomes may be used by providers initially evaluating patients with button battery ingestion in order to allocate resources and expedite transfer to a center with pediatric endoscopic and surgical capabilities. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Clinical Research Paper.

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.

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.

Autoimmune Thyroiditis and Risk of Malignancy in Children with Thyroid Nodules.

Background: It is uncertain whether the presence of autoimmune thyroiditis (AIT) increases the risk of thyroid cancer in children with thyroid nodules. This study evaluated the association between AIT and thyroid cancer in pediatric patients with thyroid nodules. Methods: A cross-sectional study was performed of pediatric patients (<19 years old) with a thyroid nodule (≥1 cm) who underwent fine-needle aspiration in an academic pediatric thyroid center. AIT was defined by the presence of thyroid autoantibodies or diffusely heterogeneous sonographic echotexture. The primary outcome was diagnosis of thyroid cancer. The association of AIT with thyroid cancer was evaluated with univariable and multivariable logistic regression. Associations of AIT with subject and nodule characteristics were also assessed. Results: Four hundred fifty-eight thyroid nodules in 385 patients (81% female) were evaluated at a median age of 15.5 years (interquartile range 13.5-17.0). Thyroid cancer was present in 108 nodules (24%). AIT was present in 95 subjects (25%) and was independently associated with an increased risk of thyroid cancer (multivariable odds ratio [OR] 2.19, 95% confidence interval [CI] 1.32-3.62). Thyroid cancer was also independently associated with younger age, nodule size, and solitary nodules, but was not associated with serum thyrotropin concentration. AIT was not associated with the likelihood of subjects undergoing thyroid surgery (p = 0.17). AIT was less commonly associated with follicular thyroid carcinoma than with papillary thyroid carcinoma (OR 0.22, CI 0.05-1.06). Among papillary thyroid carcinomas, AIT was strongly associated with the diffuse sclerosing variant (OR 4.74, CI 1.33-16.9). AIT was not associated with the extent of local, regional, or distant disease at thyroid cancer diagnosis. Conclusions: AIT is independently associated with an increased risk of thyroid cancer in children with thyroid nodules. These findings suggest that the evaluation of thyroid autoantibodies and thyroid echotexture may inform thyroid cancer risk assessment and surgical decision-making in children with thyroid nodules.