Results from the French National Esophageal Atresia register: one-year outcome
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Titre | Results from the French National Esophageal Atresia register: one-year outcome |
Type de publication | Journal Article |
Year of Publication | 2014 |
Auteurs | Schneider A, Blanc S, Bonnard A, Khen-Dunlop N, Auber F, Breton A, Podevin G, Sfeir R, Fouquet V, Jacquier C, Lemelle J-L, Lavrand F, Becmeur F, Petit T, Poli-Merol M-L, Elbaz F, Merrot T, Michel J-L, Hossein A, Lopez M, Habonimana E, Pelatan C, De lagausie P, Buisson P, de Vries P, Gaudin J, Lardy H, Borderon C, Borgnon J, Jaby O, Weil D, Aubert D, Geiss S, Breaud J, Echaieb A, Languepin J, Laplace C, Pouzac M, Lefebvre F, Gottrand F, Michaud L |
Journal | ORPHANET JOURNAL OF RARE DISEASES |
Volume | 9 |
Pagination | 206 |
Date Published | DEC 11 |
Type of Article | Article |
Mots-clés | Esophageal atresia, Long-term follow-up, One-year outcome, Population-based registry |
Résumé | Background: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). Methods: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. Results: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). Conclusions: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding. |
DOI | 10.1186/s13023-014-0206-5 |