Repaired oesophageal atresia: respiratory morbidity and pulmonary function in adults

Abstract

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.

A patient led, international study of long term outcomes of esophageal atresia: EAT 1

“…It also importantly examines the often ignored quality of life issues faced by this patient group. The large size of this study group makes it more representative of the wider international population of OA patients than most of the existing literature which is limited to smaller national [4,10] and institutional data series [3,7,13] or meta-analyses [8]. The study participants are broadly representative of the wider population based on anatomy of original defect in comparison to EUROCAT figures [14].…”

“…How transition is managed in the future is an important issue for OA care and other chronic paediatric conditions, as the population of long term survivors continues to increase. International studies such as this alongside work from Australia [22], Finland [9,10] Holland [23,24] and more recently the USA [25] are key in highlighting the need for careful planning of patient centred transitional and adult care. This may include the need for some stratification of transition and follow up.…”

“…Whilst our understanding of short term outcomes in infants with OA is good [4][5][6][7], longer term functional outcomes are more poorly understood with a limited research base [8][9][10]. There is increasing interest therefore in longer term outcome studies, and results of such studies potentially impact current practice in management of OA.…”

Lung hypoplasia in rats with esophageal atresia and tracheo–esophageal fistula

“…A recent follow-up study of a large cohort of adult EA-TEF survivors revealed that 21% had restrictive respiratory disease, 21% had obstructive respiratory disease, and 36% had both ventilatory defects. In addition, 41% had bronchial hyperreactivity often consistent with asthma, and these patients had overall impaired respiratory quality of life in comparison with a group of age-matched controls (40). Postoperative rib fusions, scoliosis, and gastro-esophageal reflux were found to account in part for these respiratory sequelae, but other explanations are possible.…”

Current Controversies in the Surgical Treatment of Esophageal Atresia

“…Patients may suffer apneic spells (72,73) or barking cough (74) during infancy and childhood, frequent vomiting (75), repeated pneumonia (69,76,77) and/or chronic respiratory tract disease (78) that are considered as sequelae of the esophageal malformation but that might be related directly to the GER. Recurrent anastomotic strictures are indeed related to GER and become manageable after effective anti-reflux treatment (79).…”