The anesthetic management of a child with ohtahara syndrome and severe stridor: a case report.

BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor. CASE PRESENTATION: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient's breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient's respiratory and cardiovascular function were stable. CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.

Update on aortopexy and posterior tracheopexy for tracheomalacia in patients with esophageal atresia.

Despite improving the survival after repair of esophageal atresia (EA), the morbidity of EA repair remains high. Specifically, tracheomalacia (TM) is one of the most frequent complications of EA repair. Continuous positive airway pressure is generally applied for the treatment of TM. However, surgical intervention is required against an apparent life-threatening event or inability to perform extubation for a long period. According to our review, most cases of TM showed symptom improvement after aortopexy. The ratio of the trachea's lateral and anterior-posterior diameter at the brachiocephalic artery crossing the trachea, which reflects the compression of the trachea by the brachiocephalic artery, is a good indicator of aortopexy. Our finding suggests that most TM cases associated with EA may not be caused by tracheal fragility alone, but may involve blood vessel compression. Posterior tracheopexy (PT) is also an effective treatment for TM. Recently, open or thoracoscopic PT was able to be performed simultaneously with EA repair. In many cases, aortopexy or PT is a safe and effective surgical treatment for TM with EA. Other surgical procedures, such as external stenting, should be considered for patients with diffuse-type TM for whom aortopexy and PT appear relatively ineffective.

Predictors of response to endoscopic management of subglottic/tracheal stenosis in patients without tracheostomy.

INTRODUCTION: Subglottic and tracheal stenosis (SGTS) in adults is an acquired or idiopathic condition that can lead to dyspnea, and even life-threatening airway obstruction. Endoscopic techniques have advanced and largely eclipsed open surgery, with open surgery now reserved for refractory cases (Hseu et al., 2013; Feinstein et al., 2017). Currently, there is no accepted guideline for the endoscopic treatment of SGTS. Thus, the aim of the present study is to examine the impact of various clinical and pathological characteristics on outcomes to endoscopic treatment in a cohort of SGTS patients. DISCLOSURE: None of the authors have any financial or personal relationship that could cause a conflict of interest regarding this article. METHODS: Retrospective chart review was performed for 41 patients presenting with SGS without a tracheostomy over a 4-year-period (2018-2022), within a single tertiary care center. Quantitative outcomes including number of dilation procedures undergone and need for open procedures were examined. The qualitative variables included a history of pulmonary disease, prior tracheostomy/tracheal resection, presence of tracheomalacia, granulation tissue, excessive dynamic airway collapse (EDAC), and etiology of idiopathic subglottic stenosis. RESULTS: The presence of granulation tissue seen on tracheoscopy was associated with a higher number (4+) of dilation procedures (p = 0.01). A history of pulmonary disease (p = 0.037), the presence of tracheomalacia (p = 0.039), and the presence of granulation tissue (0.003) were all associated with a need for open procedures. CONCLUSION: Patients with the presence of granulation tissue, tracheomalacia, and a history of pulmonary disease were more associated with more severe disease requiring either a higher number of endoscopic procedures or need for open procedures.

Successful management of a tracheomalacia patient with active endotracheal bleeding due to intraoperative innominate artery injury: A case report.

RATIONALE: Intraoperative innominate artery injury is life-threatening in tracheomalacia patients with prolonged tracheostomy. Anesthetic management is challenging in cases with massive hemorrhage into the endotracheal tube. We report a case in which we successfully managed a tracheomalacia patient with acute endotracheal bleeding due to innominate artery injury. PATIENT CONCERNS: A 24-year-old patient with tracheomalacia was scheduled to undergo exploratory thoracotomy for the treatment of intermittent bleeding at the tracheostomy site. During exploration, sudden active bleeding due to innominate artery injury was observed in the endotracheal lumen. DIAGNOSES: The patient was diagnosed with tracheomalacia. INTERVENTIONS: We immediately used the bronchoscope to place the tip of the endotracheal tube at the bleeding site and hyperinflated the cuff. OUTCOMES: The ballooned cuff compressed the active bleeding site, so no additional bleeding was detected by bronchoscopy, and no additional massive bleeding was observed in the operative field. LESSONS: Immediate and appropriate overinflation of the endotracheal tube cuff by an anesthesiologist may provide improved surgical field visibility and time for critical surgical procedures in cases of massive hemorrhaging.

Pulmonary function of children with tracheomalacia and associated clinical factors.

OBJECTIVES: Spirometry is easily accessible yet there is limited data in children with tracheomalacia. Availability of such data may inform clinical practice. We aimed to describe spirometry indices of children with tracheomalacia, including Empey index and flow-volume curve pattern, and determine whether these indices relate with bronchoscopic features. METHODS: From the database of children with tracheomalacia diagnosed during 2016-2019, we reviewed their flexible bronchoscopy and spirometry data in a blinded manner. We specially evaluated several spirometry indices and tracheomalacia features (cross-sectional lumen reduction, malacic length, and presence of bronchomalacia) and determined their association using multivariable regression. RESULTS: Of 53 children with tracheomalacia, the mean (SD) peak expiratory flow (PEF) was below the normal range [68.9 percent of predicted value (23.08)]. However, all other spirometry parameters were within normal range [Z-score forced expired volume in 1 s (FEV1 ) = -1.18 (1.39), forced vital capacity (FVC) = -0.61 (1.46), forced expiratory flow between 25% and 75% of vital capacity​​​​​​ (FEF25%-75% ) = -1.43 (1.10), FEV1 /FVC = -1.04 (1.08)], Empey Index = 8.21 (1.59). The most common flow-volume curve pattern was the "knee" pattern (n = 39, 73.6%). Multivariable linear regression identified the presence of bronchomalacia was significantly associated with lower flows: FEV1 [coefficient (95% CI) -0.78 (-1.54, -0.02)], FEF25%-75% [-0.61 (-1.22, 0)], and PEF [-12.69 (-21.13, -4.25)], all p ≤ 0.05. Other bronchoscopic-defined tracheomalacia features examined (cross-sectional lumen reduction, malacic length) were not significantly associated with spirometry indices. CONCLUSION: The "knee" pattern in spirometry flow-volume curve is common in children with tracheomalacia but other indices, including Empey index, cannot be used to characterize tracheomalacia. Spirometry indices were not significantly associated with bronchoscopic tracheomalacia features but children with tracheobronchomalacia have significantly lower flow than those with tracheomalacia alone.

Thoracoscopic posterior tracheopexy during primary esophageal atresia repair ameliorate tracheomalacia in neonates: a single-center retrospective comparative cohort study.

BACKGROUND: Esophageal atresia (EA) is often associated with tracheomalacia (TM). The severity of TM symptoms varies widely, with serious cases requiring prolonged respiratory support and surgical treatment. Although we performed thoracoscopic posterior tracheopexy (TPT) during primary EA repair to prevent or reduce the symptoms of TM, few studies have investigated the safety and effectiveness of TPT during primary EA repair. Therefore, this study aimed to evaluate the safety and efficacy of TPT in neonates. METHODS: We retrospectively reviewed the records of all patients diagnosed with TM who underwent primary thoracoscopic EA repair between 2013 and 2020 at the Nagoya University Hospital. Patients were divided into two groups: TPT (TPT group) and without TPT (control group). TPT has been performed in all patients with EA complicated by TM since 2020. We compared patient backgrounds, surgical outcomes, postoperative complications, and treatment efficacy. RESULTS: Of the 22 patients reviewed, eight were in the TPT group and 14 were in the control group. There were no statistically significant differences in the surgical outcomes between the groups (operation time: p = 0.31; blood loss: p = 0.83; time to extubation: p = 0.30; time to start enteral feeding: p = 0.19; time to start oral feeding: p = 0.43). Conversion to open thoracotomy was not performed in any case. The median operative time required for posterior tracheopexy was 10 (8-15) min. There were no statistically significant differences in postoperative complications between the groups (chylothorax: p = 0.36; leakage: p = 1.00; stricture: p = 0.53). The respiratory dependence rate 30 days postoperative (2 [25%] vs. 11 [79%], p = 0.03) and the ratio of the lateral and anterior-posterior diameter of the trachea (LAR) were significantly lower in the TPT group (1.83 [1.66-2.78] vs. 3.59 [1.80-7.70], p = 0.01). CONCLUSIONS: TPT during primary EA repair for treatment of TM significantly lowered respiratory dependence rate at 30 days postoperative without increasing the risk of postoperative complications. This study suggested that TPT could improve TM associated with EA.

Posterior tracheopexy for preterm infants with severe bronchopulmonary dysplasia and severe tracheobronchomalacia: A case series.

We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.

Poor correlation between airway fluoroscopy and rigid bronchoscopic evaluation in paediatric tracheomalacia.

INTRODUCTION: Tracheomalacia is a common congenital condition causing stridor in young children. However, the evaluation of these children remains poorly standardised across institutions. METHODS: We conducted a retrospective review of all children undergoing an elective laryngotracheobronchoscopy at a single tertiary paediatric institution between March 2010 and December 2018. Emergency bronchoscopies and children with tracheostomies were excluded. 1163 children undergoing an elective bronchoscopy were included in this study, and 545 children also had an airway fluoroscopy. RESULTS: The median age at bronchoscopy was 17 months, and the majority of children were male. Tracheomalacia was diagnosed in 21.6% of children at bronchoscopy, of these 48.5% had tracheomalacia diagnosed on a previous airway fluoroscopy. Overall, airway fluoroscopy had a low sensitivity (62.3%) and a low specificity (67.5%) for diagnosis of tracheomalacia when compared with bronchoscopy. Increasing severity of tracheomalacia on airway screen significantly predicted a diagnosis of tracheomalacia on bronchoscopy. CONCLUSIONS: Airway fluoroscopy has a low sensitivity and specificity in diagnosis of tracheomalacia and should be used judiciously rather than as a screening tool for children with stridor. However, this imaging technique may be beneficial in investigating children with severe symptoms who have had other conditions such as laryngomalacia excluded.

Myocardial strain in newborn infants with tracheomalacia due to vascular rings, a pilot study.

BACKGROUND: Vascular rings (VR) may cause severe tracheomalacia and upper airway obstruction (UAO). Increased pulmonary artery pressure and cardiac dysfunction have been described in patients with chronic UAO, but has not been investigated in infants with obstruction associated with VR. The aim of this study is to evaluate myocardial strain in infants with UAO due to VR. METHOD: Demographic characteristics, respiratory symptoms, percentage of tracheal obstruction measured and classified using Computer Tomography, and lung function testing (LFT) were collected. Left (LV) and right ventricle (RV) systolic functions were measured using speckle tracking echocardiography longitudinal strain analysis (LS). Pulmonary artery pressure was evaluated using maximal tricuspid regurgitation jet velocity (TR) and LV end-systolic eccentricity index (EI). RESULTS: Fifteen cases were included in the study, six had mild tracheal obstruction (<50%), nine moderate-severe obstruction (≥50%). LV LS and RV LS were significantly reduced in cases with moderate to severe airway obstruction cases compared to those with mild airway obstruction (LV LS -15.9 versus -19.9%; RV LS -15.7 versus -20.5%, p = .04 and p = .02, respectively). Respiratory symptoms were more pronounced in moderate-severe cases. No significant differences in TR, EI, and LFT were observed. CONCLUSIONS: In cases of VR with severe tracheomalacia RV and LV myocardial strain is reduced, suggesting secondary cardiac dysfunction.

Is secondary tracheomalacia associated with airway inflammation and infection?

BACKGROUND: Recurrent lower respiratory tract infections are among the most prevalent symptoms in secondary tracheomalacia due to mediastinal vascular anomalies (MVAs). It is not known whether this condition could result in persistent lower respiratory tract inflammation and subclinical infection. METHODS: A retrospective study was performed on records of children with tracheomalacia due to MVAs and recurrent respiratory infections who underwent computed tomography scan, bronchoscopy, and bronchoalveolar lavage (BAL) as part of their clinical evaluation. RESULTS: Thirty-one children were included in the study: 21 with aberrant innominate artery, four with right aortic arch, one with double aortic arch, and five with aberrant innominate artery associated with right aortic arch. Cytological evaluation of bronchoalveolar lavage fluid showed increased neutrophil percentages and normal lymphocyte and eosinophil proportions. Microorganism growth was detected in 13 BAL samples, with a bacterial load ≥104 colony-forming units/mL in eight (25.8%) of them. Most isolates were positive for Haemophilus influenzae. Bronchiectasis was detected in four children, all with BAL culture positive for H. influenzae. Four patients underwent MVA surgical correction and 27 conservative management, i.e., respiratory physiotherapy in all and high-dose amoxicillin/clavulanic acid (40 mg/kg/day) for 2-4 weeks in those with significant bacterial growth. There was an excellent outcome in most of them. CONCLUSIONS: Neutrophilic alveolitis is detectable in secondary tracheomalacia but is associated with a clinically significant bacterial load only in a quarter of the patients. Caution should be used regarding inappropriate antibiotic prescriptions to avoid the emergence of resistance, whilst airway clearance maneuvers and infection preventive measures should be promoted.

Association of childhood tracheomalacia with bronchiectasis: a case-control study.

OBJECTIVE: Children with tracheomalacia can develop chronic lower airway infection and neutrophilic inflammation. It is plausible children with tracheomalacia are at increased risk of developing bronchiectasis. We hypothesised that compared with controls, tracheomalacia in children is associated with bronchiectasis. DESIGN: Single-centre, case-control study. SETTING AND PATIENTS: 45 children with chest high-resolution CT (c-HRCT) confirmed bronchiectasis (cases) and enrolled in the Australian Bronchiectasis Registry were selected randomly from Queensland, and 90 unmatched children without chronic respiratory symptoms or radiographic evidence of bronchiectasis (disease controls). Cases and controls had flexible bronchoscopy performed for clinical reasons within 4 weeks of their c-HRCT. INTERVENTIONS: The bronchoscopy videos were reviewed in a blinded manner for: (a) any tracheomalacia (any shape deformity of the trachea at end-expiration) and (b) tracheomalacia defined by the European Respiratory Society (ERS) statement (>50% expiratory reduction in the cross-sectional luminal area). MAIN OUTCOME MEASURES AND RESULTS: Cases were younger (median age=2.6 years, IQR 1.5-4.1) than controls (7.8 years, IQR 3.4-12.8), but well-balanced for sex (56% and 52% male, respectively). Using multivariable analysis (adjusted for age), the presence of any tracheomalacia was significantly associated with bronchiectasis (adjusted OR (ORadj)=13.2, 95% CI 3.2 to 55), while that for ERS-defined tracheomalacia further increased this risk (ORadj=24.4, 95% CI 3.4 to infinity). CONCLUSION: Bronchoscopic-defined tracheomalacia is associated with childhood bronchiectasis. While causality cannot be inferred, children with tracheomalacia should be monitored for chronic (>4 weeks) wet cough, the most common symptom of bronchiectasis, which if present should be treated and then investigated if the cough persists or is recurrent.

Case report: a step-wise management of concurrent presentation of congenital single lung and aberrant right subclavian artery in an infant girl.

INTRODUCTION: Congenital single lung (CSL) is a rare condition, and symptomatic patients often present with respiratory distress or recurrent respiratory infection due to mediastinal shift causing vascular or airway compression. Aberrant right subclavian artery (ARSA) is another rare congenital anomality that can lead to tracheal or esophageal compressions. There is only one other case of concurrent presentation of CSL and ARSA reported, which presented unique challenge in surgical management of our patient. Here we present a step-wise, multidisciplinary approach to manage symptomatic CSL and ARSA. CASE PRESENTATION: An infant girl with a prenatal diagnosis of CSL developed worsening stridor and several episodes of respiratory illnesses at 11 months old. Cross-sectional imaging and bronchoscopic evaluation showed moderate to severe distal tracheomalacia with anterior and posterior tracheal compression resulting from severe mediastinal rotation secondary to right-sided CSL. It was determined that her tracheal compression was mainly caused by her aortic arch wrapping around the trachea, with possible additional posterior compression of the esophagus by the ARSA. She first underwent intrathoracic tissue expander placement, which resulted in immediate improvement of tracheal compression. Two days later, she developed symptoms of dysphagia lusoria due to increased posterior compression of her esophagus by the ARSA. She underwent transposition of ARSA to the right common carotid with immediate resolution of dysphagia lusoria. As the patient grew, additional saline was added to the tissue expander due to recurrence in compressive symptoms. CONCLUSIONS: Concurrent presentation of CSL and ARSA is extremely rare. Asymptomatic CSL and ARSA do not require surgical interventions. However, if symptomatic, it is crucial to involve a multidisciplinary team for surgical planning and to take a step-wise approach as we were able to recognize and address both tracheomalacia and dysphagia lusoria in our patient promptly.

Adult Aortotracheal Fistula as Sequela of Double Aortic Arch Repair in Infancy: A Case Report.

INTRODUCTION: Double aortic arch is a rare congenital malformation of the aortic arch that most frequently presents in childhood. Early surgical intervention typically yields excellent outcomes. OBJECTIVES: To describe aortotracheal fistula as a rare, yet serious complication of vascular ring and subsequent aortic aneurysm in an adult patient. METHODS: Clinical history, as well as radiographic and endoscopic imaging were obtained to describe the development, diagnosis, and clinical course of this patient's aortotracheal fistula. Additionally, follow up data was obtained to document the healing of this fistula after surgical repair. RESULTS: We describe a case of a 46-year-old male with DiGeorge Syndrome and a double aortic arch, repaired in childhood, which developed into an aortotracheal fistula after tracheostomy placement as an adult. CONCLUSIONS: This case demonstrates that dangerous complications of a double aortic arch can persist into adulthood, even after surgical repair in infancy. Each patient's unique anatomy must be considered when thinking about airway management and prevention of complications of this rare congenital anomaly.

Tracheomalacia in bronchopulmonary dysplasia: Trachealis hyper-relaxant responses to S-nitrosoglutathione in a hyperoxic murine model.

BACKGROUND: Bronchopulmonary dysplasia (BPD) with airway hyperreactivity is a long-term pulmonary complication of prematurity. The endogenous nonadrenergic, noncholinergic signaling molecule, S-nitrosoglutathione (GSNO) and its catabolism by GSNO reductase (GSNOR) modulate airway reactivity. Tracheomalacia is a major, underinvestigated complication of BPD. We studied trachealis, left main bronchus (LB), and intrapulmonary bronchiolar (IPB) relaxant responses to GSNO in a murine hyperoxic BPD model. METHODS: Wild-type (WT) or GSNOR knockout (KO) newborn mice were raised in 60% (BPD) or 21% (control) oxygen during the first 3 weeks of life. After room air recovery, adult trachealis, LB, and IPB smooth muscle relaxant responses to GSNO (after methacholine preconstriction) were studied using wire myographs. Studies were repeated after GSNOR inhibitor (GSNORi) pretreatment and in KO mice. RESULTS: GSNO relaxed all airway preparations. GSNO relaxed WT BPD trachealis substantially more than WT controls (P < .05). Pharmacologic or genetic ablation of GSNOR abolished the exaggerated BPD tracheal relaxation to GSNO and also augmented BPD IPB relaxation to GSNO. LB ring contractility was not significantly different between groups or conditions. Additionally, GSNORi treatment induced relaxation of WT IPBs but not trachealis or LB. CONCLUSION: GSNO dramatically relaxed the trachealis in our BPD model, an effect paradoxically reversed by loss of GSNOR. Conversely, GSNOR inhibition augmented IBP relaxation. These data suggest that GSNOR inhibition could benefit both the BPD trachealis and distal airways, restoring relaxant responses to those of room air controls. Because therapeutic options are limited in this high-risk population, future studies of GSNOR inhibition are needed.

Traqueomalacia, complicación de la exéresis de un bocio difuso endotorácico bilateral / Tracheomalacia: a complication after removal of bilateral endothoracic diffuse goiter

Introducción: En cirugía de la glándula tiroides la mortalidad es nula en algunas series por lo que la morbilidad es la mayor preocupación del cirujano. Objetivo: Presentar la evolución clínica de una paciente con tiroidectomía total por bocio endotorácico bilateral. Caso clínico: Paciente que se le realizó tiroidectomía total por bocio endotorácico bilateral, técnicamente difícil, biopsia por congelación negativa de malignidad, bocio coloide. Nervios recurrentes visualizados. Debido al tamaño de la glándula y lo complejo que resultó la extracción se decidió trasladar a la paciente a la sala de recuperación intubada y ventilada para proceder a la recuperación de la ventilación espontánea y la extubación en un período más prolongado. Fue extubada una primera vez que fue fallida. Fue reintubada y ocurrió en una segunda ocasión después de ser reintervenida por un posible hematoma de la herida. En la TAC preoperatoria se observó una glándula tiroides grande con prolongación endotorácia bilateral y estenosis alta de la tráquea. Conclusión: Las complicaciones de obstrucción de la vía aérea después de una tiroidectomía no son frecuentes. Generalmente, después de una tiroidectomía total o parcial se trata de recuperar al paciente en el quirófano para después de la extubación realizar una laringoscopia que descarte la parálisis de las cuerdas vocales. La traqueomalacia como complicación después de una tiroidectomía no es frecuente, por lo que se alerta que de no haber diagnosticado y tratado rápidamente la paciente podría tener un desenlace fatal(AU)

Introduction: In surgery of the thyroid gland, mortality is null in some series, so morbidity is the surgeon's greatest concern. Objective: To present the clinical evolution of a patient with total thyroidectomy due to bilateral endothoracic goiter. Clinical case: This patient underwent total thyroidectomy due to bilateral endothoracic goiter, technically difficult, negative freeze biopsy of malignancy, colloid goiter. Visualized recurrent nerves. Due to the size of the gland and how complex the extraction was, it was decided to transfer the patient to the intubated and ventilated recovery room to proceed with the recovery of spontaneous ventilation and extubation in a longer period. She was extubated the first time it failed. She was re-intubated and it occurred on a second occasion after being re-operated due to a possible hematoma of the wound. In the preoperative CT scan, a large thyroid gland with bilateral endothoracic prolongation and high stenosis of the trachea was observed. Conclusion: Complications of airway obstruction after thyroidectomy are not frequent. Generally, after a total or partial thyroidectomy, it is a matter of recovering the patient in the operating room, in order to perform, after extubation, a laryngoscopy to rule out paralysis of the vocal cords. Tracheomalacia as a complication after thyroidectomy is not frequent, so it is warned that failure to diagnose and treat the patient quickly could have a fatal outcome(AU)

Should Proton Pump Inhibitors be Systematically Prescribed in Patients With Esophageal Atresia After Surgical Repair?

OBJECTIVE: To evaluate outcomes of patients with esophageal atresia (EA) on systematic treatment with proton pump inhibitors (PPI) since the neonatal period and to determine factors associated with successful discontinuation of PPI. STUDY DESIGN: Longitudinal cohort study with prospective data collection of 73 EA patients, over 11 years systematically treated with PPI. Outcome and predictive factors for discontinuation of PPI treatment were evaluated at study end in February 2017. The incidence of anastomotic strictures was compared with a historical cohort of 134 EA patients followed in the same institution between 1990 and 2005 before the era of systematic PPI treatment. RESULTS: PPI treatment was discontinued definitively in 48% of patients during follow-up. Prematurity, longer initial hospitalization, moderate-to-severe tracheomalacia, anastomotic leak and anastomotic stricture had a significant negative association with PPI discontinuation on univariate analysis (P < 0.05). On adjusted multivariable Cox regression analysis, moderate-to-severe tracheomalacia and anastomotic leak were negatively associated with discontinuation of PPI treatment (hazard ratio 0.26 [95% CI 0.12-0.59]; P = 0.001 and hazard ratio 0.38 [95% CI 0.16-0.93]; P = 0.03, respectively). There was no significant difference in the incidence of anastomotic strictures in the present cohort compared with the historical cohort (44% vs 39%); (P > 0.05). CONCLUSIONS: PPI treatment does not prevent the formation of anastomotic strictures and appears to be over-prescribed in children with airway symptoms because of tracheomalacia. This suggests that PPI treatment could be prescribed more selectively. Close monitoring and long-term follow-up, however, of these vulnerable patients in specialized multidisciplinary clinics is imperative.