Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict development of epithelial metaplasia after repair of EA.

Although after oesophageal atresia (OA) repair in infancy, respiratory problems are common, their natural history remains unclear. We assessed morbidity, pulmonary function (PF), and bronchial hyperresponsiveness (BHR) in adults with repaired OA respiratory.588 patients who underwent surgery for OA during 1947-1985 were identified and those 262 who were alive and had their native oesophagus were included. Respiratory symptoms and respiratory symptom-related quality of life (RSRQoL) were assessed by questionnaire and interview, and the patients underwent spirometry, a histamine challenge test, and an exhaled nitric oxide test. For the questionnaires, we added 287 carefully matched general populationderived controls.Among the 101 (58 male) patients, median age 36 yrs (range 22-56 yrs), respiratory morbidity was significantly increased compared to controls. Patients had more respiratory symptoms and infections, as well as asthma and allergies, and more often impaired RSRQoL (p,0.001 for all). PF tests revealed restrictive ventilatory defect in 21 (21%) patients, obstructive ventilatroy defect in 21 (21%) patients, and both in 36 (36%) patients. A total of 41 (41%) had BHR, and in 15 (15%), it was consistent with asthma. The most significant risk factors for restrictive ventilatory defect were thoracotomy-induced rib fusions (OR 3.4, 95% CI 1.3-8.7; p50.01) and oesophageal epithelial metaplasia (OR 3.0, 95% CI 1.0-8.9; p50.05).After repair of OA, respiratory-related morbidity, restrictive ventilatory defect and BHR extended into adulthood. Nearly half the patients had BHR and over half had a restrictive ventilatory defect. Thoracotomy-induced rib fusions and gastro-oesophageal reflux-associated oesophageal epithelial metaplasia were the strongest risk factors for restrictive ventilatory defect.

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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.

The risk of scoliosis is 13-fold after repair of EA in relation to general population. Nearly half of the patients have vertebral anomalies predominating in the cervical spine. Most of these deformities were not diagnosed primarily or during growth. Spinal surgery is rarely indicated.

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S urvivors of oesophageal atresia are reaching their adulthood in large numbers for the first time, thus allowing assessment of true long-term outcome among these patients. Long-term outcomes in terms of oesophageal function and respiratory complications as well as quality of life have been reported in children and adolescents (1,2), These studies have mainly been based on institutional patient series. There is no previous population-based long-term follow-up on oesophageal atresia; therefore, the long-term sequelae are unclear.In 2003, we initiated a study project to evaluate late outcomes in adult patients who had undergone neonatal repair of oesophageal atresia. We aimed to study the incidence of oesophageal cancer, oesophageal function and morbidity, respiratory morbidity, and clinical characteristics of musculoskeletal anomalies, especially spinal defects in adulthood.

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