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.

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.

Comparison of Detection Methods for Tracheoesophageal Fistulae With a Novel Method: Capnography With CO2 Insufflation.

BACKGROUND: Tracheoesophageal fistulae (TEF) are difficult to detect and require a high index of suspicion. We hypothesized that capnography to identify a spike in end-tidal carbon dioxide (etCO2) during esophagoscopy with carbon dioxide (CO2) insufflation would facilitate TEF diagnosis because of gas passage from the esophagus to the trachea. METHODS: Medical records of 42 consecutive cases of recurrent, acquired, or missed congenital TEF diagnosed between January 2015 and November 2019 that underwent esophagoscopy with CO2 insufflation were reviewed. A control cohort of 97 similarly endoscopically evaluated patients with surgical confirmation of absence of recurrent TEF (eg, patients undergoing posterior tracheopexy) was also collected. All patients underwent pre-operative esophagoscopy, bronchoscopy, and capnography; diagnostic abilities of various combinations of modalities for TEF identification were calculated. RESULTS: Statistical analysis identified a maximum intra-esophagoscopy end-tidal CO2 level of 68 mmHg as the optimal discriminator between cases and controls, though in practice, we anecdotally find that recurrent TEFs typically permit rapid rise ≥90 mmHg. Increasing numbers of diagnostic modalities increased diagnostic sensitivity to detect recurrent TEF; the highest diagnostic sensitivity for TEF identification was achieved by the combination of intra-esophagoscopy fluoroscopy with bronchoscopy and capnography ≥68 mmHg (sensitivity = 88.1%). There were multiple cases of TEF (N = 7 for etCO2 ≥68 mmHg, N = 3 for etCO2 ≥90 mmHg) identified by capnography that were missed by esophagoscopy. There were 5 (for etCO2 ≥68 mmHg) or 6 (for etCO2 ≥90 mmHg) cases of recurrent TEF that were missed by all nonsurgical methods. CONCLUSION: Attention to etCO2 during esophagoscopy with CO2 insufflation represents a simple, novel way to detect TEF. Identification of TEF remains challenging, though combinations of diagnostic modalities improve diagnostic sensitivity.

Rules Are Meant to Be Broken: Examining the "Rule of 3" for Esophageal Dilations in Pediatric Stricture Patients.

BACKGROUND AND AIMS: The "rule of 3" is a 40-year-old expert opinion that suggests dilating an esophageal stricture more than 3 mm is unsafe. Few studies have evaluated this tenet, and do not specify how much larger than 3 mm is reasonable. Our aim was to determine the optimal point for maximum dilation diameter with acceptable risk in a pediatric population. METHODS: A retrospective review in pediatric patients with esophageal strictures was performed. The number of millimeters the stricture was dilated, defined as delta dilation diameter (ΔDD), was determined by subtracting the initial stricture diameter from the diameter of the largest balloon used. Receiver operating characteristic curve analysis was used to evaluate the discriminatory ability of ΔDD. Youden J index was used to identify optimal cut-point in predicting perforation. RESULTS: Two hundred eighty-four patients underwent 1384 balloon dilations. Overall perforation rate was 1.66%. There were 8 perforations in 1075 dilations with ΔDD ≤5 mm (0.7%) and 15 perforations in 309 dilations with ΔDD >5 mm (4.9%). Youden J index found an optimal cutoff to be at a ΔDD of ≤5 mm. The cumulative rate of perforation for all dilations ≤5 mm was 0.74% whereas the cumulative risk of perforation for all dilations ≥6 mm was 4.85% (P < 0.001). CONCLUSIONS: Balloon dilations that expand the initial esophageal anastomosis ≤5 mm in a pediatric population appear to not unduly increase the risk of perforation. Further prospective studies are needed to further investigate the potential for a new rule of 5 for balloon dilation.

Intralesional Steroid Injection Therapy for Esophageal Anastomotic Stricture Following Esophageal Atresia Repair.

OBJECTIVES: The role of intralesional steroid injection (ISI) in the treatment of anastomotic stricture in patients with esophageal atresia remains unclear. The aim of this study was to evaluate the efficacy and safety of ISI. METHODS: A total of 158 patients with esophageal atresia with at least 1 ISI for the treatment of esophageal anastomotic stricture between 2010 and 2017 were identified. The change in stricture diameter (ΔD) was compared between procedures with dilation alone (ISI-) and dilation with steroid injection (ISI+). RESULTS: A total of 1055 balloon dilations were performed (452 ISI+). The median ΔD was significantly greater in the ISI+ group: 1 mm (interquartile range [IQR] 0, 3) versus 0 mm (IQR -1, 1.5) (P < 0.0001). The ISI+ group had greater percentage of improved diameter (P < 0.0001) and lesser percentages of unchanged and decreased diameters at subsequent endoscopy (P = 0.0009, P = 0.003). Multivariable logistic regression confirmed the significance of ISI on increasing the likelihood of improved stricture diameter with an adjusted odds ratio of 3.24 (95% confidence interval: 2.15-4.88) (P < 0.001). The ΔD for the first 3 ISI+ procedures was greater than the ΔD for subsequent ISI+ procedures: 1 mm (IQR 0, 3) versus 0.5 mm (IQR-1.25, 2) (P = 0.001). There was no difference in perforation incidence between ISI+ and ISI- groups (P = 0.82). CONCLUSIONS: ISI with dilation was well tolerated and improved anastomotic stricture diameter more than dilation alone. The benefit of ISI over dilation alone was limited to the first 3 ISI procedures.

Tracheobronchomalacia, Tracheobronchial Compression, and Tracheobronchial Malformations: Diagnostic and Treatment Strategies.

Tracheobronchomalacia (TBM) is an excessive dynamic narrowing of the airway that is greatest with increased mediastinal pressure such as coughing, Valsalva, and forced expiration. Airway compression and/or cartilage malformation is a fixed or static narrowing of the airway typically caused by great vessel malposition and/or abnormalities and may also contribute to airway narrowing. Although imprecise and misleading, the term TBM is often used to represent both problems, static and dynamic airway narrowing, which only serves to confuse and may mislead the treatment team into ineffective therapies. The consequences of airway narrowing caused by dynamic TBM and/or static compression includes a range of clinical signs and symptoms, depending on the location, extent, and severity of the airway collapse. All patients with mild to severe TBM benefit from medical management to optimize airway clearance of mucus. The milder cases of TBM may become asymptomatic with this therapy, allowing time for the child to grow and the airway to enlarge without the consequences of recurrent infections. In cases of more severe TBM with clinical sequelae, more aggressive management may be warranted. Multiple options for surgical intervention are available. This article discusses the details of clinical presentation, evaluation, diagnosis, and a variety of treatments.

Utility of repeated therapeutic endoscopies for pediatric esophageal anastomotic strictures.

Anastomotic stricture is a common complication of esophageal atresia (EA) repair. Such strictures are managed with dilation or other therapeutic endoscopic techniques such as steroid injections, stenting, or endoscopic incisional therapy (EIT). In situations where endoscopic therapy is unsuccessful, patients with refractory strictures may require surgical stricture resection; however, the point at which endoscopic therapy should be abandoned in favor of repeat thoracotomy is unclear. We hypothesized that increasing numbers of therapeutic endoscopies are associated with increased likelihood of stricture resection. We retrospectively reviewed the records of patients with EA who had an initial surgery at our institution resulting in an esophago-esophageal anastomosis between August 2005 and May 2019. Up to 2 years of post-surgery endoscopy data were collected, including exposure to balloon dilation, intralesional steroid injection, stenting, and EIT. Primary outcome was need for stricture resection. Receiver operating characteristic (ROC) curve analysis and univariate and multivariable Cox proportional hazards regression analyses were performed. There were 171 patients who met inclusion criteria. The number of therapeutic endoscopies was a moderate predictor of stricture resection by ROC curve analysis (AUC = 0.720, 95% CI 0.617-0.823). With increasing number of therapeutic endoscopies, the probability of remaining free from stricture resection decreased. By Youden's J index, a cutoff of ≥7 therapeutic endoscopies was optimal for discriminating between patients who had versus did not have stricture resection, though an absolute majority of patients (≥50%) remained free of stricture resection at each number of therapeutic endoscopies through 12 endoscopies. Significant predictors of needing stricture resection by univariate regression included ≥7 therapeutic endoscopies, Foker surgery for long-gap EA, fundoplication, history of esophageal leak, and length of stricture ≥10 mm. Multivariate analysis identified only history of leak as statistically significant, though this regression was underpowered. The utility of repeated therapeutic endoscopies may diminish with increasing numbers of endoscopic therapeutic attempts, with a cutoff of ≥7 endoscopies identified by our single-center experience as our statistically optimal discriminator between having stricture resection versus not; however, a majority of patients remained free of stricture resection well beyond 7 therapeutic endoscopies. Though retrospective, this study supports that repeated therapeutic endoscopies may have clinical utility in sparing surgical stricture resection. Esophageal leak is identified as a significant predictor of needing subsequent stricture resection. Prospective study is needed.

Endoscopic Esophageal Vacuum Therapy: A Novel Therapy for Esophageal Perforations in Pediatric Patients.

BACKGROUND: Esophageal perforation is a potentially life-threatening problem if not quickly diagnosed and treated appropriately. Negative-pressure wound therapy, commercially known as V.A.C. therapy, was developed in the early 1990s and is now standard of care for chronic surface wounds, ulcers, and burns. Adapting vacuum sponge therapy for use intraluminally for perforations of the esophagus was first reported in 2008. We report the first pediatric experience on a customized esophageal vacuum-assisted closure (EVAC) device for closure of esophageal perforations. AIM: To evaluate the technical feasibility, safety, and efficacy of EVAC in a pediatric population with esophageal perforations and compare efficacy to a cohort of patients who underwent stenting for esophageal perforation. METHODS: We performed an institutional review board-approved retrospective chart review on all patients who underwent EVAC for esophageal perforations (October 2013-September 2017) and who underwent externally removable stent placement for esophageal perforation (January 2010-December 2017) at our institution. Our primary aim was to evaluate technical feasibility, efficacy, and safety in the treatment of pediatric esophageal perforations. A secondary aim was to compare the efficacy of EVAC to esophageal stenting in healing esophageal perforations in our pediatric population. RESULTS: A total of 17 patients with esophageal atresia underwent therapy for esophageal perforation. Eight sponges were placed for surgical perforation and 9 were placed after endoscopic therapy perforation. The median age of patients was 24 months with the youngest patient being 3 months of age. The success rate of EVAC to seal all esophageal perforations was 88% (15/17). The success rate was similar in both subgroups: surgical anastomotic leaks at 88% (7/8) and endoscopic therapy leaks at 89% (8/9). There were no technical failures with placement. The stent group had a total of 24 patients: 19 were placed secondary to perforations from endoscopic therapy and 5 were placed secondary to surgical anastomotic perforations. The success rate of stents to seal all esophageal perforations was 63% (15/24). The success rate in the subgroups was 74% (14/19) for endoscopic therapy leaks and 20% (1/5) for surgical anastomotic leaks. In comparing success of EVAC and stent therapy, we found a statistically significant difference in favor of EVAC in healing surgical anastomotic perforations (P = 0.032). There was, however, no statistical difference in healing endoscopic therapy perforations (P = 0.360). CONCLUSIONS: EVAC is a novel, promising technique for the treatment of esophageal perforations in a pediatric population. This treatment is comparable to esophageal stenting in iatrogenic endoscopic therapy perforations and superior to stenting surgical perforations. Further prospective studies are needed to compare the effectiveness of EVAC to esophageal stenting. Improvement in device design and customization could further improve success and ease of placement.

Endoscopic Electrocautery Incisional Therapy as a Treatment for Refractory Benign Pediatric Esophageal Strictures.

BACKGROUND AND AIM: Refractory esophageal strictures are rare conditions in pediatrics, and are often due to anastomotic, congenital, or caustic strictures. Traditional treatment options include serial dilation and surgical stricture resection; endoscopic intralesional steroid injections, mitomycin C, and externally removable stents combined with dilation have had variable success rates. Although not as widely used, endoscopic electrocautery incisional therapy (EIT) has been reported as an alternative treatment for refractory strictures in a small number of adult series. The aim of the study was to evaluate the safety and efficacy of EIT in a pediatric population with refractory esophageal strictures. METHODS: A retrospective chart review was conducted on all patients who underwent EIT for esophageal strictures (May 2011-September 2017) at our tertiary-care referral center. A total of 57 patients underwent EIT. Procedural success was defined as no stricture resection, appropriate diameter for age, and fewer than 7 dilations within 24 months of first EIT session. This corresponded to the 90th percentile of the observed number of dilations in the data. All patients included in the study had at least 2-year follow-up. RESULTS: A total of 133 EIT sessions on 58 distinct anastomotic strictures were performed on 57 patients (24 girls). The youngest patient to have EIT was 3 months old and 4.8 kg. There were 36 strictures that met the criteria for refractory stricture and 22 non-refractory (NR) strictures. The median number of dilations before EIT therapy was 8 (interquartile range [IQR]: 6-10) in the refractory group and 3 (IQR: 0-3) in the NR group. In the refractory group, 61% of the patients met the criteria for treatment success. The median number of dilations within 2 years of EIT in the refractory group was 2 (IQR: 0-4). In the NR group, 100% of the patients met criteria for success. The median number of dilations within 2 years of EIT in the NR was 1 (IQR: 0-2). The overall adverse event rate was 5.3% (7/133), with 3 major (2.3%) and 4 minor events (3%). CONCLUSIONS: EIT shows promise as an adjunct treatment option for pediatric refractory esophageal strictures and may be considered before surgical resection even in severe cases. The complication rate, albeit low, is significant, and EIT should only be considered by experienced endoscopists in close consultation with surgery. Further prospective longitudinal studies are needed to validate this treatment.

Double Supercharged Jejunal Interposition for Late Salvage of Long-gap Esophageal Atresia.

BACKGROUND: A variety of surgical techniques exist to manage long-gap esophageal atresia (LGEA), including gastric pull-up (GPU), colonic interposition (CI), jejunal interposition (JI), and distraction lengthening. Salvage reconstruction for late failure of any conduit type is a complex surgical problem fraught with technical difficulty and significant risk. Jejunal interposition can be used as a salvage procedure in the management of LGEA. However, the opposing requirements of conduit length and adequate perfusion make the procedure technically challenging. Chronic comorbidities and abdominal and thoracic adhesions may further complicate these cases. METHODS: We report a technique for the management of 3 late treatment failures of LGEA using pedicled JI in conjunction with 2 additional arterial and venous anastomoses, or double supercharging. For 2 patients who presented with failed CI, pedicled JI was performed and supercharged to internal mammary vessels as well as vasculature preserved from the prior colonic flap mesentery. The third patient presented with failed GPU and underwent pedicled JI that was supercharged caudally to the gastroepiploic vessels and cranially to the left common carotid artery. RESULTS: No flaps were lost in any patients. Median operation time was 16.5 hours. Patients were monitored postoperatively in the intensive care unit for a median of 23 days, extubated after 14 days, and discharged at 41 days. Postoperatively, all patients tolerated an oral diet by discharge and continue to enjoy oral intake of all food consistencies without dysphagia or aspiration. Follow-up time spanned 2 to 4 years (average, 3.3 years). One patient required dilatations and temporary stent for stricture, and another required removal of prominent sternal wires; otherwise, no additional procedures were performed. CONCLUSIONS: Although technically difficult, double supercharged JI should be considered as a salvage operation to restore esophageal continuity after CI or GPU failure for LGEA, when there are otherwise limited reconstructive options.

Surgical Treatment of Tracheobronchomalacia: A novel approach.

Tracheobronchomalacia, as a whole, is likely misdiagnosed and underestimated as a cause of respiratory compromise in pediatric patients. Currently, there is no standardized approach for the overall evaluation of pediatric tracheobronchomalacia (TBM) and the concept of excessive dynamic airway collapse (EDAC); no grading score for the evaluation of severity; nor a standardized means to successfully approach TBM and EDAC. This paper describes our experience standardizing the approach to these complex patients whose backgrounds include different disease etiologies, as well as a variety of comorbid conditions. Preoperative and postoperative evaluation of patients with severe TBM and EDAC, as well as concurrent development of a prospective grading scale, has allowed us to ascertain correlation between surgery, symptoms, and effectiveness on particular tracheal-bronchial segments. Long-term, continued collection of patient characteristics, surgical technique, complications, and outcomes must be collected given the overall heterogeneity of this particular population.

Quality improvement program reduces venous thromboembolism in infants and children with long-gap esophageal atresia (LGEA).

PURPOSE: Patients with long-gap esophageal atresia (LGEA) treated with the Foker process are at increased risk of venous thromboembolism (VTE). An institutional quality improvement program to decrease VTE risk factor exposure and utilize prophylactic anticoagulation was implemented. We aim to evaluate the efficacy and safety of a VTE risk-reduction program in patients with LGEA. METHODS: Implementation and evaluation of a VTE risk-reduction program in patients with LGEA from 2012 to 2015 was performed. Symptomatic VTE with radiographic confirmation were defined as events. Post-program characteristics were evaluated and compared to a historical cohort. RESULTS: Sixty-seven patients were identified. Two developed VTE (7 %) post-program implementation; compared to 13/40 (33 %) VTE incidence in the historical cohort (p = 0.018). Baseline demographics were similar, including age, esophageal atresia type and gap length. Post-protocol patients had fewer paralysis episodes (p = 0.004), paralysis days (p = 0.003), central venous catheters (p = 0.003), thoracotomies (p < 0.001), ventilator hours (p = 0.02), and decreased hospital (p < 0.001) and ICU stay (p < 0.001). All patients in the VTE risk-reduction program were exposed to prophylactic anticoagulation. No bleeding complications and/or thrombosis-related mortality occurred. CONCLUSION: VTE risk-reduction program implementation decreased symptomatic VTE incidence with associated decreases in ICU and hospital length of stay. Prophylactic anticoagulation can be utilized safely in a complicated pediatric surgical population.

Paravertebral nerve block catheters using chloroprocaine in infants with prolonged mechanical ventilation for treatment of long-gap esophageal atresia.

BACKGROUND: Infants with long-gap esophageal atresia (LGEA) undergo repeated thoracotomies for staged surgical repair known as the Foker process (FP). Associated prolonged mechanical ventilation results in exposure to high doses of opioids and benzodiazepines, and prolonged weaning times and ICU stays. AIM: The aim of this study was to determine the effectiveness of short-term paravertebral nerve block (PVNB) catheters in reducing opioid/benzodiazepine exposure and effects on clinical variables. METHODS: The medical records of seventeen infants were retrospectively reviewed; 11 with PVNB and six without (CG). PVNB were placed using ultrasound-guidance and chloroprocaine infusions implemented in the ICU. Opioids and benzodiazepines were administered via the protocol for 5 days following thoracotomies for Foker-I and Foker-II. RESULTS: Foker-I: Average reduction in morphine and midazolam consumption was 36% (2.18 vs 3.40 mg·kg(-1) ·day(-1) ; P < 0.001) and 31% (2.25 vs 3.25 mg·kg(-1) ·day(-1) ; P = 0.033), respectively, in the PVNB compared with CG. Foker-II: Average reduction in morphine and midazolam consumption was 39% (3.19 vs 5.27 mg·kg(-1) ·day(-1) ) and 38% (3.46 mg·kg(-1) ·day(-1) vs 5.62; P < 0.001), respectively in the PVNB compared with CG. 24-h prior to extubation: Average reduction in morphine and midazolam consumption was 50% (2.91 vs 5.85 mg·kg(-1) ·24 h(-1) ; p = 0.023) and 61% (2.27 vs 5.83 mg·kg(-1) ·24 h(-1) ; P = 0.004), respectively, in the PVNB compared with CG. Infusion wean time, (independence from opioid/midazolam infusions) following extubation was 5 days in the PVNB group and 15 days in CG (P = 0.005). Median ICU stay (IQR) was 40 days (34-45 days) in PVNB patients and 71 days (42-106 days) in controls (P = 0.02). PVNB catheters were left an average of 7 days and there were no complications associated with the nerve blocks. CONCLUSION: Short-term PVNB placement decreases opioid and benzodiazepine exposure, weaning days and ICU stay in infants undergoing prolonged mechanical ventilation for LGEA repair in this small pilot study. Larger studies are warranted to confirm results.

Externally removable stents in the treatment of benign recalcitrant strictures and esophageal perforations in pediatric patients with esophageal atresia.

BACKGROUND: We investigated whether removable stents, such as self-expandable plastic stents (SEPSs) and fully covered self-expandable metal stents (FCSEMSs) could provide an alternative treatment for recalcitrant strictures and esophageal perforations after esophageal atresia (EA) repair. OBJECTIVE: The primary aim of our study was to evaluate technical feasibility. Secondary aims were to evaluate safety and procedural success. DESIGN: Retrospective study. SETTING: Tertiary-care referral center. PATIENTS: A total of 24 children with EA. INTERVENTIONS: Retrospective review of all children with EA who underwent dilation and esophageal stent placement from January 2010 to February 2013 at our institution. MAIN OUTCOME MEASUREMENTS: Healing of perforation and stricture resolution at 30 and 90 days. RESULTS: A total of 41 stents (SEPSs 14, FCSEMSs 27) were placed in 24 patients with EA during the study period, including 14 who had developed esophageal leaks. Procedural success of esophageal stent placement in the treatment of refractory strictures was 39% at 30 days and 26% at 90 days. The success rate was 80% for closure of esophageal perforations with stent therapy after dilation and 25% for perforations associated with surgical repair. Adverse events of stent placement included migration (21% of SEPSs and 7% of FCSEMSs), granulation tissue (37% of FCSEMSs), and deep ulcerations (22% of FCSEMSs). LIMITATIONS: Retrospective study with small sample size. CONCLUSION: SEPSs and FCSEMSs can be placed successfully in small infants and children with a history of EA repair. The stents appear to be safe and beneficial in closing esophageal perforations, especially post-dilation. However, a high stricture recurrence rate after stent removal may limit their usefulness in treating recalcitrant esophageal anastomotic strictures.

Imaging of long gap esophageal atresia and the Foker process: expected findings and complications.

Long gap esophageal atresia (EA) is characterized by esophageal segments that are too far apart for primary anastomosis. Surgical repair utilizing interposition grafts or gastric transposition are often employed. The Foker staged lengthening procedure is an alternative surgical method that utilizes continuous traction on the esophagus to induce esophageal growth and allow for primary esophageal anastomosis. This pictorial review presents the step-by-step radiographic evaluation of the Foker procedure and illustrates the radiographic findings in the most commonly encountered complications in our cohort of 38 patients managed with this procedure from January 2000 to June 2012.

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.

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.

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.