Matteo Cassina1, Michele Ruol2, Riccardo Pertile3, Paola Midrio4, Silvano Piffer3, Virginia Vicenzi1, Mario Saugo5, Carmen Fiorella Stocco6, Piergiorgio Gamba2, Maurizio Clementi1. 1. Clinical Genetics Unit, Department of Women's and Children's Health, University of Padova, Padova, Italy. 2. Pediatric Surgery Unit, Department of Women's and Children's Health, University of Padova, Padova, Italy. 3. Epidemiological Department, Trento Province, Trento, Italy. 4. Pediatric Surgery Unit, "Ca' Foncello" Hospital, Treviso, Italy. 5. Regional Epidemiological Department, Veneto Region, Padova, Italy. 6. Veneto Tumor Registry, Veneto Region, Padova, Italy.
Abstract
BACKGROUND: Esophageal atresia (EA) is a congenital malformation of the upper gastrointestinal tract with an estimated prevalence varying from 1 in 2500 to 1 in 4500 births. The aim of this study was to describe the epidemiology of EA between 1981 and 2012 and evaluate patients' survival. METHODS: This study used data from a population-based Italian Congenital Malformation Registry. The survival status was ascertained by linking the registry records, vital records and the regional registries of patients. Kaplan-Meier methods were used to estimate survival probabilities up to 25 years and Cox proportional hazards regression was used to evaluate factors that affected survival. RESULTS: A total of 407 cases of EA were identified among 1,417,724 total births. After the exclusion of cases with chromosomal anomalies, 49.9% of the patients presented with at least one associated congenital anomaly. The 25-year survival probability was 85.1% (95% confidence interval [CI], 80.8-89.4), with most deaths occurring during the first months of life. Patients' characteristics associated with decreased survival probability were low birth weight (hazard ratio, 3.7; 95% CI, 1.7-8.3) and presence of additional major defects (hazard ratio, 2.8; 95% CI, 1.3-6.0). A significant improvement in survival over the decades was observed for patients with nonisolated EA. CONCLUSION: This study detected a significant improvement in survival of individuals with EA over the past decades and identified the strongest predictors of mortality. These results will be important for the planning of the clinical management and formulation of prognosis when EA is diagnosed in a newborn. Birth Defects Research (Part A) 106:542-548, 2016.
BACKGROUND:Esophageal atresia (EA) is a congenital malformation of the upper gastrointestinal tract with an estimated prevalence varying from 1 in 2500 to 1 in 4500 births. The aim of this study was to describe the epidemiology of EA between 1981 and 2012 and evaluate patients' survival. METHODS: This study used data from a population-based Italian Congenital Malformation Registry. The survival status was ascertained by linking the registry records, vital records and the regional registries of patients. Kaplan-Meier methods were used to estimate survival probabilities up to 25 years and Cox proportional hazards regression was used to evaluate factors that affected survival. RESULTS: A total of 407 cases of EA were identified among 1,417,724 total births. After the exclusion of cases with chromosomal anomalies, 49.9% of the patients presented with at least one associated congenital anomaly. The 25-year survival probability was 85.1% (95% confidence interval [CI], 80.8-89.4), with most deaths occurring during the first months of life. Patients' characteristics associated with decreased survival probability were low birth weight (hazard ratio, 3.7; 95% CI, 1.7-8.3) and presence of additional major defects (hazard ratio, 2.8; 95% CI, 1.3-6.0). A significant improvement in survival over the decades was observed for patients with nonisolated EA. CONCLUSION: This study detected a significant improvement in survival of individuals with EA over the past decades and identified the strongest predictors of mortality. These results will be important for the planning of the clinical management and formulation of prognosis when EA is diagnosed in a newborn. Birth Defects Research (Part A) 106:542-548, 2016.
Authors: Pierre Fayoux; Martin Morisse; Rony Sfeir; Laurent Michaud; Sam Daniel Journal: Eur Arch Otorhinolaryngol Date: 2018-01-04 Impact factor: 2.503
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Authors: Jane C Bell; Gareth Baynam; Jorieke E H Bergman; Eva Bermejo-Sánchez; Lorenzo D Botto; Mark A Canfield; Saeed Dastgiri; Miriam Gatt; Boris Groisman; Paula Hurtado-Villa; Karin Kallen; Babak Khoshnood; Victoria Konrad; Danielle Landau; Jorge S Lopez-Camelo; Laura Martinez; Margery Morgan; Osvaldo M Mutchinick; Amy E Nance; Wendy Nembhard; Anna Pierini; Anke Rissmann; Xiaoyi Shan; Antonin Sipek; Elena Szabova; Giovanna Tagliabue; Lyubov S Yevtushok; Ignacio Zarante; Natasha Nassar Journal: Birth Defects Res Date: 2021-03-18 Impact factor: 2.661