Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-esophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center.

Preoperative and postoperative records of patients with EA, TEF + EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy.

A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025). CONCLUSİON: Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.

Data on long-term outcome of exercise capacity in school-aged children with esophageal atresia (EA) are scarce. We evaluated maximal exercise capacity and its relation to lung function. Moreover, we studied other possible determinants of exercise capacity and lung function.

Exercise capacity of 63 children with EA born 1999-2007 was evaluated at the age of 8 years with the Bruce-protocol. Dynamic and static lung volumes, bronchodilator response and diffusion capacity were measured. Furthermore, perinatal characteristics, hospital admissions for lower respiratory tract infections (RTIs), RTIs treated with antibiotics in the past year, symptoms of gastroesophageal reflux, weight-for-height, and sports participation were evaluated as other potential determinants.

Exercise capacity was significantly below normal: mean (SD) SDS -0.91 (0.97); P < 0.001. All spirometric parameters were significantly below normal with significant reversibility of airflow obstruction in 13.5% of patients. Static lung volumes were significantly decreased (mean (SD) SDS TLC_he -1.06 (1.29); P < 0.001). Diffusion capacity corrected for alveolar volume was normal (mean (SD) SDS K_CO -0.12 (1.04)). Exercise capacity was positively associated with total lung capacity and negatively with SDS weight-for-height. Spirometric parameters were negatively associated with congenital cardiac malformation, duration of ventilation, and persistent respiratory morbidity.

Eight-year-old children with EA had reduced exercise capacity which was only associated with the reduction in TLC_he and higher SDS weight-for-height. We speculate that diminished physical activity with recurrent respiratory tract infections may also play a role in reduced exercise capacity. This should be subject to further research to optimize appropriate intervention.

The aim of this study was to illustrate the pulmonary long term outcome of patients with repaired esophageal atresia and to further examine causes and correlations that might have led to this outcome.

Twenty-seven of 62 possible patients (43%) aged 5-20years, with repaired esophageal atresia were recruited. Body plethysmography and spirometry were performed to evaluate lung function, and the Bruce protocol treadmill exercise test to assess physical fitness. Results were correlated to conditions such as interpouch distance, gastroesophageal reflux or duration of post-operative mechanical ventilation.

Seventeen participants (63%) showed abnormal lung function at rest or after exercise. Restrictive ventilatory defects (solely restrictive or combined) were found in 11 participants (41%), and obstructive ventilatory defects (solely obstructive or combined) in 13 subjects (48%). Twenty-two participants (81%) performed the Bruce protocol treadmill exercise test to standard. The treadmill exercise results were expressed in z-score and revealed to be significantly below the standard population mean (z-score=-1.40). Moreover, significant correlations between restrictive ventilatory defects and the interpouch distance; duration of post-operative ventilation; gastroesophageal reflux disease; plus recurrent aspiration pneumonia during infancy; were described.

It was shown that esophageal atresia and associated early complications have significant impact on pulmonary long term outcomes such as abnormal lung function and, in particular restrictive ventilatory defects. Long-running and regular follow-ups of patients with congenital esophageal atresia are necessary in order to detect and react to the development and progression of associated complications such as ventilation disorders or gastroesophageal reflux disease.

Prognosis study, Level II.

In the past decades improved surgical techniques and better neonatal supportive care have resulted in reduced mortality of patients with esophageal atresia (EA), with or without tracheoesophageal fistula, and in increased prevalence of long-term complications, especially respiratory manifestations. This integrative review describes the techniques currently used in the pediatric clinical practice for assessing EA-related respiratory disease. We also present a novel algorithm for the evaluation and surveillance of lung disease in EA. A total of 2813 articles were identified, of which 1451 duplicates were removed, and 1330 were excluded based on review of titles and abstracts. A total of 32 articles were assessed for eligibility. Six reviews were excluded, and 26 original studies were assessed. Lower respiratory tract infection seems frequent, especially in the first years of life. Chronic asthma, productive cough, and recurrent bronchitis are the most common respiratory complaints. Restrictive lung disease is generally reported to prevail over the obstructive or mixed patterns, and, overall, bronchial hyperresponsiveness can affect up to 78% of patients. At lung imaging, few studies detected bronchiectasis and irregular cross-sectional shape of the trachea, whereas diffuse bronchial thickening, consolidations, and pleural abnormalities were the main chest X-ray findings. Airway endoscopy is seldom included in the available studies, with tracheomalacia and tracheobronchial inflammation being described in a variable proportion of cases. A complete diagnostic approach to long-term respiratory complications after EA is mandatory. In the presence of moderate-to-severe airway disease, patients should undergo regular tertiary care follow-up with functional assessment and advanced chest imaging.

Chronic, long-term respiratory morbidity (CRM) is common in patients with a history of repaired congenital esophageal atresia, typically associated with tracheoesophageal fistula (EA/TEF). EA/TEF patients are at high risk of having aspiration, and retrospective studies have associated CRM with both recurrent aspiration and atopy. However, studies evaluating the association between CRM in this population and either aspiration or atopy have reported conflicting results. Furthermore, CRM in this population may be due to other related conditions as well, such as tracheomalacia and/or recurrent infections. Aspiration is difficult to confirm, short of lung biopsy. Moreover, even within the largest evidence base assessing the association between CRM and aspiration, which has evaluated the potential relationship between gastroesophageal reflux and asthma, findings are contradictory. Studies attempting to relate CRM to prior aspiration events may inadequately estimate the frequency and severity of previous aspiration episodes. There is convincing evidence documenting that chronic, massive aspiration in patients with repaired EA/TEF is associated with the development of bronchiectasis. While chronic aspiration is likely associated with other CRM in patients with repaired EA/TEF, this does not appear to have been confirmed by the data currently available. Prospective studies that systematically evaluate aspiration risk and allergic disease in patients with repaired EA/TEF and document subsequent CRM will be needed to clarify the causes of CRM in this population. Given the prevalence of CRM, patients with repaired EA/TEF should ideally receive regular follow-up by multidisciplinary teams with expertise in this condition, throughout both childhood and adulthood.

The esophagus is derived from the anterior portion of the developmental intermediate foregut, a structure that also gives rise to other organs including the trachea, lung, and stomach. Genetic studies have shown that multiple signaling pathways (e.g. Bmp) and transcription factors (e.g. SOX2) are required for the separation of the esophagus from the neighboring respiratory system. Notably, some of these signaling pathways and transcription factors continue to play essential roles in the subsequent morphogenesis of the esophageal epithelium which undergoes a simple columnar-to-stratified squamous conversion. Reactivation of the relevant signaling pathways has also been associated with pathogenesis of esophageal diseases that affect the epithelium and its stem cells in adults. In this review we will summarize these findings. We will also discuss new data regarding the cell-of-origin for the striated and smooth muscles surrounding the esophagus and how they are differentiated from the mesenchyme during development.

Neonatal surgery is recognized as an independent discipline in general surgery, requiring the expertise of pediatric surgeons to optimize outcomes in infants with surgical conditions. Survival following neonatal surgery has improved dramatically in the past 60 years. Improvements in pediatric surgical outcomes are in part attributable to improved understanding of neonatal physiology, specialized pediatric anesthesia, neonatal critical care including sophisticated cardiopulmonary support, utilization of parenteral nutrition and adjustments in fluid management, refinement of surgical technique, and advances in surgical technology including minimally invasive options. Nevertheless, short and long-term complications following neonatal surgery continue to have profound and sometimes lasting effects on individual patients, families, and society.

Oesophageal atresia and tracheo-oesophageal fistula are congenital anomalies of the oesophagus requiring surgical repair in infancy, either by open or thoracoscopic approach. Although mortality rates associated with this procedure are low, children may go on to have complications throughout childhood and into adulthood, most commonly related to ongoing gastrointestinal and respiratory symptoms. This review outlines the early, mid and long-term outcomes for these children in terms of quality of life and incidence of symptoms.

Children with congenital esophageal atresia with tracheoesophageal fistula (TEF) require complex medical and surgical care, but few guidelines exist to guide the long term care of this population. The purpose of this study is to describe the findings and initial management of a comprehensive aerodigestive team in order to understand the ongoing needs of children with repaired TEF.

A retrospective chart review was performed on children with TEF who were seen in the multidisciplinary Aerodigestive Clinic at Children's Hospital Colorado. Diagnostic studies were ordered based on physician discretion.

Twenty-nine children with TEF were evaluated (mean age 3.8 years) between 2010 and 2014. All children had symptoms attributed to breathing, swallowing, and digestive difficulties. Less than half of the children had seen a pulmonary or gastrointestinal specialist in the past year. Tracheomalacia was diagnosed in all children who had a bronchoscopy (23/23), and the presence of dysphagia was correlated with severe tracheomalacia. 7/25 children who had a swallow study had aspiration. 7/25 children had a diagnosis of active reflux despite current management. Four patients were diagnosed with bronchiectasis as a result of the multidisciplinary evaluation.

Although all children had persistent aerodigestive symptoms, over 50% had not been seen by an appropriate subspecialist in the year prior to the clinic visit. The multidisciplinary evaluation resulted in new diagnoses of bronchiectasis and active reflux, which can both lead to long-term morbidity and mortality. Children with TEF require evaluation by multiple subspecialists to manage not only current symptoms but also long term risks. Ongoing care should be guided by protocols based on known risks. Pediatr Pulmonol. 2016;51:576-581. © 2015 Wiley Periodicals, Inc.

The objective of this study was to describe the incidence of complications in children with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) at a tertiary pediatric hospital and to identify predictive factors for their occurrence. A retrospective chart review of 110 patients born in or transferred to Sydney Children's Hospital with EA/TEF between January 1999 and December 2010 was done. Univariate and multivariate regression analyses were performed to identify predictive factors for the occurrence of complications in these children. From univariate analysis, early esophageal stricture formation was more likely in children with 'long-gap' EA (odds ratio [OR] = 16.32). Patients with early strictures were more likely to develop chest infections (OR = 3.33). Patients with severe tracheomalacia were more likely to experience 'cyanotic/dying' (OR = 180) and undergo aortopexy (OR = 549). Patients who had gastroesophageal reflux disease were significantly more likely to require fundoplication (OR = 10.83) and undergo aortopexy (OR = 6.417). From multivariate analysis, 'long-gap' EA was a significant predictive factor for late esophageal stricture formation (P = 0.007) and for gastrostomy insertion (P = 0.001). Reflux was a significant predictive factor for requiring fundoplication (P = 0.007) and gastrostomy (P = 0.002). Gastrostomy insertion (P = 0.000) was a significant predictive factor for undergoing fundoplication. Having a prior fundoplication (P = 0.001) was a significant predictive factor for undergoing a subsequent aortopexy. Predictive factors for the occurrence of complications post EA/TEF repair were identified in this large single centre pediatric study.

Esophageal atresia (EA) often leads to persistent symptoms and impaired respiratory function in adulthood. The role of peripheral airways in this impairment has not been previously investigated. Furthermore, asthma-like symptoms are common in these patients.

The purpose of this study was to investigate pulmonary outcome, including possible peripheral airway dysfunction, perhaps missed by conventional pulmonary function tests and to see if the diagnosis asthma was accurate.

Twenty eight patients operated for EA in Gothenburg 1968-1983 answered a questionnaire regarding respiratory symptoms and underwent pulmonary function tests. Peripheral airway function was measured by multiple breath washout.

22/28 (79%) patients had a history of respiratory symptoms. Abnormal peripheral airway function was found in 17 (61%) patients, while only 6 (21%) patients displayed values indicating central obstruction. Nine patients had restrictive disease. Airway hyperresponsiveness was frequent and associated with atopy and airway inflammation. However, respiratory symptoms or doctor-diagnosed asthma did not correlate with any specific lung function test abnormality.

Different lung function abnormalities are present in EA survivors, and peripheral airway disease is common. Classical asthma seems to be difficult to diagnose in this patient group. Given the high prevalence of respiratory morbidity, long-term follow-up of pulmonary function, including peripheral airway function, is warranted.

To study the incidence and type of pulmonary function abnormalities after thoracotomy in children.

Children below 12 y of age who had undergone thoracotomy for any condition and have at least 2 y follow up were included in the study. Detailed assessment of the patients included history and general examination, clinical assessment of pulmonary function, bedside tests to assess pulmonary function and laboratory pulmonary function test using portable spirometer.

Fifty two patients were included in the study. Twenty-seven were cases of esophageal atresia with trachea-esophageal fistula (EATEF), nine pulmonary metastasis from abdominal solid tumors, six mediastinal masses, three hydatid cyst, three eventration of diaphragm, two bronchiectasis, and one each of H-type TEF and congenital esophageal stenosis. The mean age at the time of evaluation was 6.3 y (range 2-18 y). While all the patients were clinically assessed, only 25 (48 %) were eligible for bedside tests and 23 (44 %) for spirometery. The incidences of abnormalities picked were: dyspnea during exercise 8/52 (15.4 %), dyspnea on exercise and on climbing stairs 1/52 (2 %), decreased breath holding time 2/25 (8 %), abnormal incentive spirometry 1/25 (4 %), mild restrictive pattern on pulmonary function test (PFT) 11/23 (47.8 %), moderate restrictive pattern on PFT 2/23 (8.7 %). None had an obstructive pattern on PFT.

Though the incidences of pulmonary function abnormalities were high, these were of mild grade. Close follow up of patients after thoracotomy would be needed for early pick up and appropriate management of these abnormalities to prevent long-term consequences.

Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.

Despite early surgical repair, congenital esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) has long-term effects on respiratory and gastrointestinal function. This review updates summarizes research published since 2003 on long-term respiratory complications in patients with a history of EA ± TEF. Pulmonary hypoplasia appears to not be rare in patients with EA ± TEF. Tracheomalacia is common and is associated with respiratory symptoms in childhood. Aspiration, associated with esophageal dysmotility and/or gastroesophageal reflux, may lead to reduced pulmonary function and bronchiectasis. Pulmonary function is generally normal, although lower than in control patients, and restrictive defects tend to be commoner than obstructive defects. Abnormal airway reactivity is common and, along with respiratory symptoms, is associated with atopy. However, the inflammatory profile in EA ± TEF patients based on bronchial biopsies and exhaled nitric oxide differs from typical allergic asthma. Recent studies suggest that in older patients, respiratory symptoms tend to be associated with atopy, but abnormal lung function tends to be associated with gastroesophageal reflux and with chest wall abnormalities. Early detection and management of aspiration may be important to help prevent decrements in pulmonary function and serious long-term complications in EA ± TEF patients.

Esophageal atresia (EA) is a congenital malformation. Nowadays, its initial prognosis is excellent thanks to improvements in neonatal and surgical management. However, the assessment of long-term respiratory outcome has become necessary in affected children and was thus performed in this study. The benefits of cardiopulmonary function testing were also examined.

The medical records of 77 children operated on for EA between 1990 and 2004 were reviewed. The results of respiratory function testing and cardiopulmonary response to effort were collected, together with neonatal and anthropometric data.

Acceptable measurements were obtained in 31 children with EA. These children were comparable to the ones lost during follow-up. The results of pulmonary function tests (PFTs) were abnormal in 21 cases (68%). A poor ventilatory response was detected in 14 children (45%) by cardiopulmonary function testing. Ten children who had abnormal results on PFTs were not under any anti-asthmatic treatment.

Impaired lung function was noted in children with repaired EA. Indeed, cardiopulmonary function tests results correlated with standard spirometric parameters and revealed minimal clinical symptoms. Moreover, many children with EA had a limited ventilatory reserve (VR). These results indicate that respiratory symptoms are often neglected in children with repaired EA and reinforce the need to provide adequate treatment.

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.

Survivors of esophageal atresia and tracheo-esophageal fistula (EA-TEF) often suffer chronic respiratory tract disease. EA-TEF results from abnormal emergence of the trachea from the foregut. This study in a rat model tests the hypothesis that primary lung maldevelopment might be a downstream consequence of this defect.

The lung was hypoplastic in rats with EA-TEF although the histological pattern was normal. Maturation and arteriolar wall thickness were unchanged, but mesenchymal control of airway branching was weakened. This branching was deficient from embryonal day (E13) on in adriamycin-treated explants.

In conclusion, the lungs were hypoplastic in rats with experimental EA-TEF due to defective embryonal airway branching. However, arteriolar wall and respiratory epithelial patterns remained normal. These findings suggest that similarly defective lung development might contribute to chronic respiratory disease in EA-TEF patients.

Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle on E7, E8, and E9. Lungs were recovered at E15, E18, and E2. Lung weight/body weight ratio, total DNA and protein, radial alveolar count, arteriolar wall thickness, lung maturity, and mesenchymal control of airway branching were assessed. E13 lungs were cultured for 72 h and explant airway branching was measured daily. For comparisons, nonparametric tests (*P < 0.05) were used.

Esophageal atresia (EA) affects one in 2,840 newborns, and over half have associated anomalies that typically affect the midline. After EA repair in infancy, gastroesophageal reflux (GER) and esophageal dysmotility and respiratory problems are common. Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, six cases of esophageal cancer have been reported in young adults treated for EA. According to our data, the statistical risk for esophageal cancer is not higher than 500-fold that of the general population. However, the overall cancer incidence among adults with repaired EA does not differ from that of the general population. Adults with repaired EA have had significantly more respiratory symptoms and infections, as well as more asthma and allergies than does the general population. Nearly half the patients have bronchial hyperresponsiveness. Thoracotomy-induced rib fusion and gastroesophageal reflux-associated columnar epithelial metaplasia are the most significant risk factors for the restrictive ventilatory defect that occurs in over half the patients. Over half the patients with repaired EA are likely to develop scoliosis. Risk for scoliosis is 13-fold after repair of EA in relation to that of the general population. Nearly half of the patients have had vertebral anomalies predominating in the cervical spine, and of these, most were vertebral fusions. The natural history of spinal deformities seems, however, rather benign, with spinal surgery rarely indicated.

Long-term impairment of pulmonary function in trachea-esophageal fistula (TEF) patients is, at least in part, commonly ascribed to gastro-esophageal reflux disease (GERD). The objective of this study was to examine the independent effects of the underlying condition and GERD on cardiopulmonary function.

Cardiopulmonary function of TEF patients, who had (severe) GERD (s-GERD) requiring antireflux surgery (TEF + GERD, n = 11) and TEF patients who did not have s-GERD (group TEF-GERD, n = 20) were compared with control patients who had isolated s-GERD requiring antireflux surgery (group GERD, n = 13). All patients performed spirometry, lung volume measurements, measurement of diffusion capacity and maximal cardiopulmonary exercise testing (CPET).

Mean age of the participants was 13.8 ± 2.7 (group TEF + GERD). 13.2 ± 2.9 (group TEF-GERD), and 14.7 ± 1.5 years (group GERD). FVC and TLC were significantly lower in patients with TEF (with and without s-GERD) when compared to patients with isolated s-GERD. Most pulmonary function parameters were similarly affected in both TEF groups, but FEV(1) was lower in the TEF + GERD group than in the TEF-GERD group. Cardiopulmonary exercise parameters were similar in all groups.

TEF patients had restrictive lung function impairment when compared to patients with isolated s-GERD. This difference may be due to several causes, including thoracotomy. FEV(1) was lower in TEF + GERD when compared to TEF-GERD indicating that GERD may affect large airway function in TEF patients. Other differences between TEF patients with and without s-GERD were not significant, suggesting only a minor role for GERD.

Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA) are congenital anomalies commonly associated with pulmonary complications during early childhood. This study investigated the role of the forced oscillation technique (FOT) in assessing lung function in young children with repaired TOF/OA.

Forty children with repaired TOF/OA of median (range) age 8.0 (3.3-10.6) years, and 20 healthy children without TOF aged 6.1 (3.1-10.8) years were studied. FOT measurements were attempted in all subjects and spirometry only in those 6 years and above. Resistance and reactance (both hPasL(-1)) at 6 Hz (Rrs6 and Xrs6, respectively) and 8 Hz (Rrs8 and Xrs8) measured using FOT, and forced expired volume in 1 sec (FEV(1)), forced vital capacity, functional residual capacity, total lung capacity, and residual volume (all L) obtained from spirometry or plethysmography were compared with reference values and expressed as z-scores.

Technically acceptable measurements of Rrs6, Rrs8, Xrs6, Xrs8, Fdep, and Fres were obtained in 37 children with TOF and 20 healthy children without TOF, respectively. Those with TOF had significantly higher mean (SD) z-scores for Rrs6 [0.99 (0.75)] versus healthy children without TOF [0.31 (0.69)] and lower mean (SD) z-scores for Xrs6 [-1.04 (1.07)] versus healthy children without TOF [-0.34 (0.83)]. Spirometry was successful in 24 of the 29 with TOF in whom it was attempted and all healthy children without TOF. Mean (SD) z-score for FEV(1) was significantly lower in those with TOF [-0.86 (1.13)] versus healthy children without TOF [0.67 (0.54)]. z-Scores for Rrs6 and FEV(1) were significantly correlated (r = -0.49; P = 0.003).

Children with repaired TOF have diminished lung function compared with healthy children. FOT is sensitive and correlates well with standard spirometry. It can be used to measure lung function in younger children when spirometry is difficult to perform and should be considered as an objective method for monitoring clinical progress in young children with TOF.

The successful operative management of oesophageal atresia and tracheo-oesophageal atresia has been available for approximately 70 years. During this time neonatal intensive care has evolved, surgical techniques have improved and consequently near 100% survival for this condition may now be achieved. In keeping with promising results, the co-morbidities of the condition have gained increasing recognition. In this article, the clinical course from antenatal assessments, neonatal surgery and co-morbidities from infancy to adulthood are reviewed to provide a broad overview of the condition.

Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.

To characterize symptoms, pulmonary function tests (PFT) and bronchial responsiveness (BR) in adolescents after repaired esophageal atresia with tracheoesophageal fistula and correlate these with endobronchial biopsy findings.

After a primary operation, 31 patients underwent endoscopies and bronchoscopies at the age of <3, 3 to 7, and >7 years. A questionnaire on respiratory and esophageal symptoms was sent to patients at a mean age of 13.7 years (range, 9.7-19.4). The questionnaire was completed by 27 of 31 patients (87%), and 25 of the 31 patients (81%) underwent clinical examination and pulmonary functioning tests. Endobronchial biopsies were analyzed for reticular basement membrane (RBM) thickness and inflammatory cells.

The prevalence of current respiratory and esophageal symptoms was 41% and 44%, respectively. "Doctor-diagnosed asthma" was present in 22% of patients. A restrictive and obstructive spirometric defect was observed in 32% and 30% of patients, respectively. Increased bronchial responsiveness, detected in 24% of patients, was weakly associated with current respiratory symptoms and low forced vital capacity. Mean exhaled nitric oxide was within predicted range. RBM thickness increased slightly with age, whereas inflammatory cell counts varied from normal to moderate, with intraindividual variation.

Inflammation of the airways in adolescents with a history of tracheoesophageal fistula, even in the presence of atopy, does not lead, in most cases, to the type of chronic inflammation and RBM changes seen in asthma.

Oesophageal atresia-tracheo-oesophageal fistula has featured in paediatric surgery since its beginnings. The first successful primary repair was in 1941. With overall survival now exceeding 90% in dedicated centres, the emphasis has changed to reducing morbidity and achieving improvements in the quality of life. An overview of current and emerging strategies in managing patients with this condition is presented. Advances in developmental biology and molecular genetics reflecting improved understanding of the pathogenesis are highlighted.

Our objective was to study long-term respiratory outcomes of congenital diaphragmatic hernia (CDH) treated in the perinatal period. This was a cohort study with 26 adolescent survivors and age- and gender-matched controls. Medical histories were retrieved from hospital charts and questionnaires. Pulmonary function testing included measurement of maximum inspiratory and expiratory pressures (MIPS and MEPS) and maximum voluntary ventilation (MVV). Unpaired two-tailed t-test and nonlinear regression were used for statistical analysis. Significant differences were found in forced expiratory volume in one second (FEV(1)) (79% +/- 16% vs. 94% +/- 10%, P < 0.001), FEF(25-75) (62% +/- 24% vs. 84% +/- 15%, P < 0.001), FRC (114% +/- 20% vs. 95% +/- 13%, P < 0.001), RV/TLC (31% +/- 10% vs. 22% +/- 6%, P < 0.001), MVV (74% +/- 16% vs. 90% +/- 13%, P < 0.001), and MIPS (69% +/- 19% vs. 84% +/- 16%, P < 0.01), with numbers indicating percent predicted of reference values +/- SD. Reduction of MVV was not independent from FEV1 (r = 0.83). Forty-eight percent of patients vs. 4% of controls showed significant improvement of FEV1 after bronchodilators (86% +/- 15 vs. 98% +/- 10, P < 0.01). Forty-six percent of patients had abnormalities of the chest wall or spinal column such as pectus excavatum, pectus carinatum, and scoliosis, mostly mild or moderate. In conclusion, long-term respiratory outcome in adolescent CDH is associated with mild to moderate airway obstruction, a high prevalence of response to bronchodilators, and decreased inspiratory muscle strength. This should guide follow-up scheduling and should be taken into account for perioperative and critical care management.

Congenital esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) are common congenital anomalies. Respiratory and GI complications occur frequently, and may persist lifelong. Late complications of EA/TEF include tracheomalacia, a recurrence of the TEF, esophageal stricture, and gastroesophageal reflux. These complications may lead to a brassy or honking-type cough, dysphagia, recurrent pneumonia, obstructive and restrictive ventilatory defects, and airway hyperreactivity. Aspiration should be excluded in children and adults with a history of EA/TEF who present with respiratory symptoms and/or recurrent lower respiratory infections, to prevent chronic pulmonary disease.

Improvements in the diagnosis and treatment of congenital disorders have resulted in a change in surgical practice. Many conditions that formerly required corrective surgery immediately after birth are no longer surgical emergencies. Most babies with congenital anomalies that can be corrected by surgery are now stabilized and optimized before the procedure. This article focused on the more common conditions that require semi-elective or urgent surgery in the neonatal period. Salient features of each of these disorders were described. Factors unique to each of these conditions that can affect the anesthetic course of these children were discussed. Methods and techniques that may aid in the anesthetic management of these children were delineated.

To determine the relation between respiratory function in infancy and at school age in children who have undergone oesophageal atresia and tracheoesophageal fistula repair, and assess the value of infant respiratory function testing; and to examine the effect of bronchodilators.

Fourteen children (6 girls, and 8 boys) who had undergone respiratory function testing in infancy were retested at school age (7-12 years). Measurements included lung volume, airways resistance, peak flow, and spirometry. Clinical problems were investigated by questionnaire. Twelve children had repeat measurements after taking salbutamol.

Predominant complaints were non-productive cough and dysphagia, but even those children with major problems in infancy reported few restrictions at school or in sport or social activities. Respiratory function and clinical findings at school age appeared unrelated to status in infancy, such that even the patients with severe tracheomalacia requiring aortopexy did not have lung function testing suggestive of malacia at school age. Most patients showed a restrictive pattern of lung volume which would appear to result from reduced lung growth after surgery rather than being a concomitant feature of the primary congenital abnormality. Although six children reported wheeze and four had a diagnosis of asthma, only one responded to salbutamol. This suggests that a tendency to attribute all lower respiratory symptoms to asthma may have led to an overdiagnosis of this condition in this patient group.

Respiratory function testing in infancy is of limited value in medium term prognosis, but may aid management of contemporary clinical signs. In children respiratory function testing is valuable in assessing suspected asthma and effects of bronchodilators.