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.

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.

Negative pressure suction test: An intraoperative airway maneuver to assess effectiveness of surgical correction of tracheobronchomalacia.

BACKGROUND: Surgical correction of tracheobronchomalacia (TBM) has evolved greatly over the past decade, with select pediatric institutions establishing dedicated surgery and anesthesia teams to navigate the complexities and challenges of surgical airway repairs. Although anesthetic techniques have evolved internally over many years to improve patient safety and outcomes, many of these methods remain undescribed in literature. TECHNIQUE: In this article, we describe the intraoperative negative pressure suction test. This simulates the negative pressure seen in awake and spontaneously breathing patients, including the higher pressures seen during coughing which induce airway collapse in patients with TBM. Also known as the Munoz maneuver in surgical literature, this test has been performed on over 300 patients since 2015. DISCUSSION: The negative pressure suction test allows for controlled intraoperative assessment of surgical airway repairs, replaces the need for risky intraoperative wake-up tests, increases the chances of a successful surgical repair, and improves anesthetic management for emergence and extubation. We provide a guide on how to perform the test and videos demonstrating its efficacy in intraoperative airway evaluation. CONCLUSIONS: As surgeries to repair TBM become more prevalent in other pediatric institutions, we believe that pediatric patients and anesthesia providers will benefit from the insights and methods described here.

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.

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.

Comparative Effectiveness of Recurrent Laryngeal Nerve Monitoring Techniques in Pediatric Surgery.

OBJECTIVES/HYPOTHESIS: The recurrent laryngeal nerve (RLN) is at risk during pediatric cervical, thoracic, and cardiac surgery. We aim to determine the feasibility and effectiveness of RLN monitoring techniques in all pediatric patients. STUDY DESIGN: Retrospective case series. METHODS: Retrospective review of patients/procedures with RLN(s) at risk and RLN monitoring at Boston Children's Hospital July 2019-October 2020. PRIMARY OUTCOMES: pre/postoperative vocal fold mobility by awake flexible fiberoptic laryngoscopy (FFL). RESULTS: One hundred one patients (median [interquartile range, IQR] age 14.6 months [4.6-49.7 months], weight 10 kg [5.2-16.2 kg]) underwent 122 procedures with RLN(s) at risk. RLN monitoring attempted 111 cases, successful 96 (84%). Surgical indications: esophageal atresia/tracheoesophageal fistula, and tracheobronchomalacia. Sixty-two (56%) procedures in reoperative field. Median follow-up 112 days (IQR 41-230). Pre/postoperative FFL performed 84 procedures (69%), 19 new postoperative RLN injuries (16%), median age 12 months, reoperative fields 11 (18%). Prass probes: 34 cases (28 successful, 82%), 6 injuries (18%), age 12.2 (5.8-23.6) months. Dragonfly electrodes: 45 cases (37 successful, 82%), 8 injuries (18%), age 7.5 (3.8-19) months. Nerve integrity monitoring (NIM) integrated electrode endotracheal tube: 33 cases (33 successful, 100%), 5 injuries (15%), age 90 (58.8-136.7) months. Automatic periodic stimulation (APS): 16 cases, 13 successful (81%), four injuries (25%), age 7.2 (5.3-20.6) months. NIM RLN monitoring is significantly more successful than Prass, Dragonfly (95%CI -0.3 to 0.02, P = .02; and 95%CI 0.05-0.31, P = .008). Rates of injury are not different between types of RLN monitoring (P = .94), with APS use (P = .47), or with monitoring success (95%CI -0.36 to 0.09, P = .28). CONCLUSIONS: RLN monitoring is feasible in pediatric patients of all ages. Although NIM type RLN monitoring success is superior, all forms offer similar rates of nerve protection. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:889-894, 2022.

Bronchomalacia in Right Aortic Arch Treated With Descending Aortic Translocation and Airway Splint.

Management of recurrent symptomatic tracheobronchomalacia and tracheobronchial compression after prior aortopexy and tracheobronchopexy is a surgical challenge. In patients with right aortic arch, the course of the aortic arch over the right mainstem bronchus can result in superior and posterior compression of the airway. This report presents 2 cases of recurrent bronchomalacia and bronchial compression treated with descending aortic translocation. The addition of bronchial splinting to aortic translocation effectively relieved airway symptoms.

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.

Radiographic assessment of traction-induced esophageal growth and traction-related complications of the Foker process for treatment of long-gap esophageal atresia.

BACKGROUND: Radiographic assessment of esophageal growth in long-gap esophageal atresia while on traction and associated traction-related complications have not been described. OBJECTIVE: To demonstrate how chest radiography can estimate esophageal position while on traction and to evaluate radiography's utility in diagnosing certain traction system complications. MATERIALS AND METHODS: In this retrospective evaluation of portable chest radiographs obtained in infants with long-gap esophageal atresia who underwent the Foker process between 2014 and 2020, we assessed distances between the opposing trailing clips (esophageal gap) and the leading and trailing clips for each esophageal segment on serial radiographs. Growth during traction was estimated using longitudinal random-effects regression analysis to account for multiple chest radiograph measurements from the same child. RESULTS: Forty-three infants (25 male) had a median esophageal gap of 4.5 cm. Median traction time was 14 days. Median daily radiographic esophageal growth rate for both segments was 2.2 mm and median cumulative growth was 23.6 mm. Traction-related complications occurred in 13 (30%) children with median time of 8 days from traction initiation. Daily change >12% in leading-to trailing clip distance demonstrated 86% sensitivity and 92% specificity for indicating traction-related complications (area under the curve [AUC] 0.853). Cumulative change >30% in leading- to trailing-clip distance during traction was 85% sensitive and 85% specific for indicating traction complications (AUC 0.874). CONCLUSION: Portable chest radiograph measurements can serve as a quantitative surrogate for esophageal segment position in long-gap esophageal atresia. An increase of >12% between two sequential chest radiographs or >30% increase over the traction period in leading- to trailing-clip distance is highly associated with traction system complications.

Reoperation to correct unsuccessful vascular ring and vascular decompression surgery.

OBJECTIVE: Although most children do well after operations to relieve vascular compression of the esophagus and airway, many will have persistent/recurrent symptoms. We review our surgical experience using a customized approach to correct various etiologies of failure after vascular ring/decompression surgery. METHODS: Our institutional database identified children who underwent reoperation for persistent/recurrent symptoms after vascular ring or aberrant arterial decompression surgery between January 2014 and December 2019. Charts were reviewed for operative approaches and clinical data. Findings were analyzed by Fisher exact test for comparison between groups. RESULTS: Twenty-seven children required reoperative surgery. Detailed preoperative workup identified 5 etiologies of failure for a customized approach. Residual scarring was corrected by lysis and rotational esophagoplasty (n = 23/27); fibrotic bands re-creating a ring were divided (n = 11); ongoing vascular compression was addressed by descending aortopexy (n = 19), aberrant subclavian division (n = 7), aortic uncrossing procedure (n = 4), and Kommerell resection (n = 8); anterior aortopexy (n = 6) and anterior tracheopexy (n = 9) corrected cartilage malformation; and tracheobronchomalacia was addressed with posterior airway pexy (n = 26). At available short-term follow-up (median 1 year), 21 of 22 patients (95%) had symptom improvement, and on bronchoscopy, the average number of airway sections with severe tracheobronchomalacia decreased from 2.8 ± 1.7 to 0.5 ± 0.9 (P < .001). CONCLUSIONS: Persistent/recurrent symptoms after release of vascular compression are frequently caused by 5 different etiologies. A multidisciplinary strategy for workup and a customized operative approach can effectively treat these cases and may suggest opportunity at the index surgery to prevent reoperation and achieve optimal outcomes.

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.

Infant study of hemispheric asymmetry after long-gap esophageal atresia repair.

OBJECTIVES: Previous studies have demonstrated that infants are typically born with a left-greater-than-right forebrain asymmetry that reverses throughout the first year of life. We hypothesized that critically ill term-born and premature patients following surgical and critical care for long-gap esophageal atresia (LGEA) would exhibit alteration in expected forebrain asymmetry. METHODS: Term-born (n = 13) and premature (n = 13) patients, and term-born controls (n = 23) <1 year corrected age underwent non-sedated research MRI following completion of LGEA treatment via Foker process. Structural T1- and T2-weighted images were collected, and ITK-SNAP was used for forebrain tissue segmentation and volume acquisition. Data were presented as absolute (cm3 ) and normalized (% total forebrain) volumes of the hemispheres. All measures were checked for normality, and group status was assessed using a general linear model with age at scan as a covariate. RESULTS: Absolute volumes of both forebrain hemispheres were smaller in term-born and premature patients in comparison to controls (p < 0.001). Normalized hemispheric volume group differences were detected by T1-weighted analysis, with premature patients demonstrating right-greater-than-left hemisphere volumes in comparison to term-born patients and controls (p < 0.01). While normalized group differences were very subtle (a right hemispheric predominance of roughly 2% of forebrain volume), they represent a deviation from the expected pattern of hemispheric brain asymmetry. INTERPRETATION: Our pilot quantitative MRI study of hemispheric volumes suggests that premature patients might be at risk of altered expected left-greater-than-right forebrain asymmetry following repair of LGEA. Future neurobehavioral studies in infants born with LGEA are needed to elucidate the functional significance of presented anatomical findings.

Experience with bioresorbable splints for treatment of airway collapse in a pediatric population.

OBJECTIVE: To report our experience with novel external tracheal and bronchial placed bioresorbable splints in children with severe symptomatic airway collapse. METHODS: Retrospective review of patients undergoing bioresorbable splint placement. RESULTS: Between July 2018 and February 2020, 14 patients received 16 external splints (trachea, n = 8; left bronchus, n = 7; and right bronchus, n = 1). Preoperatively, 7 patients had a tracheostomy; 6 of them were receiving mechanical ventilation with ventilator settings so high that they required an inpatient setting, often in an intensive care unit. Median age at implant was 14.5 months (range, 2 months-14 years). Splints were formed from moldable bioresorbable plates (RapidSorb; Synthes, Oberdorf, Switzerland) and were customized intraoperatively around a Hegar dilator. A series of Prolene sutures were placed through into the airway cartilage under simultaneous bronchoscopic and direct visualization and then tied securing the airway within the splint. Concomitant procedures were also performed in the region of the airway splints, consisting of airway reconstruction, cardiovascular procedures, and/or esophageal rotation (related to posterior tracheopexy). Median follow-up was 20 months (interquartile range, 12-21 months). Four patients required no further intervention. Although not necessarily in the splinted region, 7 patients required additional procedures, including posterior tracheobronchopexy (n = 2), temporary tracheal stent placement (n = 1), tracheal resection with end-to-end anastomosis (n = 1), closure tracheostomy (n = 1), and tracheostomy placement (n = 2). One patient required splint replacement and in 1 patient, the splint was removed later. All patients (except 2 deaths from unrelated causes) were discharged home. Three patients required mechanical ventilation at lower settings that allowed home ventilation (1 of those only at night), and 4 patients required tracheostomy collar. Indications for tracheostomy included subglottic stenosis, vocal cord paralysis, pulmonary insufficiency, small airway malacia, and laryngomalacia. CONCLUSIONS: An external bioresorbable splint can provide temporary external support while allowing the age-proportional growth of the airway. We applied readily available bioresorbable plates that were custom-molded based on the location, shape, and length of the collapsing airway in selected patients presenting with severe tracheobronchomalacia from loss of structural support and/or cartilage deformation. Further study that includes long-term outcomes are necessary to define the best role for these external splints as part of comprehensive airway management.

Current concepts in tracheobronchomalacia: diagnosis and treatment.

Airway collapse from dynamic tracheobronchomalacia (TBM), static compression from vascular compression, and/or tracheobronchial deformation are challenging conditions. Patients are best assessed and managed by a multidisciplinary team in centers specializing in complex pediatric airway disorders. Suspicion is made through clinical history and physical examination, diagnosis of location and severity by dynamic 3-phase bronchoscopy, and surgical treatment planning by MDCT and other studies as necessary to completely understand the problems. The treatment plan should be patient-based with a thorough approach to the underlying pathology, clinical concerns, and combined abnormalities. Patients should undergo maximum medical therapy prior to committing to other interventions. For those children considered candidates for surgical intervention, all other associated conditions, including vascular anomalies, chest wall deformities, mediastinal lesions, or other airway pathologies, should also be considered. Our preference is to correct the airway lesions at the same operation as other comorbidities, if possible, to prevent multiple reoperations with their attendant increased risks. We also strongly advocate for the use of recurrent laryngeal nerve monitoring in all cases of cervical or thoracic surgery to minimize the risks to vocal cord function and laryngeal sensation. Studies that evaluate the effect of these interventions on the patient and caregiver's quality of life are needed to fully grasp the impact of TBM on this challenging patient population.

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.

Head circumference in infants undergoing Foker process for long-gap esophageal atresia repair: Call for attention.

INTRODUCTION: We extended our pilot study in infants following long-gap esophageal atresia (LGEA) repair to report head circumference, an easily obtainable indirect measure of brain size. Data are presented in the context of previously reported body weight and T2-weighted MRI measures of intracranial and brain volumes. METHODS: Clinical information and head circumference were obtained for term-born (n = 13) and premature (n = 13) infants following LGEA repair with Foker process, as well as healthy term-born controls (n = 20) <1-year corrected age who underwent non-sedated research MRI. General Linear Model univariate analysis with corrected age at scan as a covariate and Bonferroni adjusted p values assessed group differences. RESULTS: We report no difference in head circumference between the three groups. Such findings paralleled trends in body weight and total intracranial volume but not in brain volume as previously reported for the same pilot cohort. DISCUSSION: Results suggest uncompromised somatic and head growth after repair of LGEA. In contrast, a novel finding of discrepancy between head circumference (novel data) and brain size (previously published data) in the same cohort suggests that head circumference might not be the best indirect measure of brain size in selected group of patients.

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.