Respiratory and esophageal morbidity in adults with repaired esophageal atresia

Background: Esophageal atresia (EA) often leads to persistent esophageal and respiratory symptoms, as well as impaired esophageal and lung function in adulthood. !e reasons for this, and the connections between symptoms and documented abnormalities, are not fully understood. Purpose: We wanted to investigate a cohort of adults previously operated on for EA in order to describe the prevalence of symptoms and functional abnormalities, as well as to investigate whether, and if so how, they are connected. Methods: Seventy-three of 79 patients operated on for EA in Gothenburg in 19681983 agreed to participate in the "rst part of the study involving questionnaires relating to symptoms from the esophagus and respiratory tract. Twenty-nine patients agreed to undergo further investigations of pulmonary and esophageal function. Results: From the questionnaire studies, we found that, even though the overall quality of life was good, a considerable number of patients had troublesome symptoms. Both esophageal and respiratory symptoms were frequent. Fifty-seven percent experienced swallowing disturbances (dysphagia) which appeared to be associated with regurgitation, which was in turn noted in 40%. !irty-two percent experienced heartburn. Di#erent respiratory symptoms, such as wheeze and long-standing cough (44% and 30% respectively), were much more common in this cohort than in the general population. Asthma was reported by 30%, even though no predisposing factors were noted. Impaired respiratory function, either obstructive and/or restrictive, was noted in 22/28 (79%). !e obstruction was mainly in the peripheral airways, 17/28 (61%) subjects (measured by multiple-breath inert gas washout, MBW), while only six (21%) subjects displayed values indicating central obstruction. Nine patients had restrictive disease. Airway hyper-responsiveness was frequent and associated with atopy and airway in$ammation. However, respiratory symptoms or doctor-diagnosed asthma (DDA) did not correlate with any speci"c lung function test abnormality. !ere was a high prevalence of gastro-esophageal re$ux (GER) measured by pH multichannel intraluminal impedance (pH-MII) involving both pathological re$ux episodes with a pH of < 4 and of > 4 (5/15 and 10/15 subjects respectively). Dysphagia correlated to the number of weakly acidic re$ux episodes, while esophageal mucosal damage (14/24 subjects with esophagitis, two of whom had Barrett’s esophagus) correlated to the re$ux index (RI) and the number of episodes of weakly acidic re$ux. Lower esophageal sphincter incompetence to any extent was frequent (21/24 subjects) and correlated to the number of acid re$ux episodes and RI. Conclusion: A high prevalence of both respiratory and esophageal symptoms remains in adulthood. !e impaired pulmonary function appears to be more pronounced than previously described. Even non-acidic re$ux episodes appear to contribute to the esophageal morbidity. New investigative modalities such as MBW and pH-MII have helped us in further describing and understanding the late sequelae of EA. Classical asthma appears to be di%cult to diagnose in this patient group. Given http://hdl.handle.net/2077/34841 ISBN 978-91-628-8922-7 Göteborg 2014 the high prevalence of both respiratory and esophageal morbidity, further studies and long-term follow-up, including MBW and pH-MII, are warranted.