Esophageal atresia: data from a national cohort.
OBJECTIVE
A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan.


METHODS
All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report.


RESULTS
Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted.


CONCLUSIONS
Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.
Reference Center for Congenital Oesophageal Anomalies, University Hospital Lille, France. rony.sfeir@chru-lille.fr
Rony
R
Sfeir
Arnaud
A
Bonnard
Naziha
N
Khen-Dunlop
Frederic
F
Auber
Thomas
T
Gelas
Laurent
L
Michaud
Guillaume
G
Podevin
Anne
A
Breton
Virginie
V
Fouquet
Christian
C
Piolat
Jean Louis
JL
Lemelle
Thierry
T
Petit
Frederic
F
Lavrand
Francis
F
Becmeur
Marie Laurence
ML
Polimerol
Jean Luc
JL
Michel
Frederic
F
Elbaz
Eric
E
Habonimana
Hassan
H
Allal
Emmanuel
E
Lopez
Hubert
H
Lardy
Marianne
M
Morineau
Cécile
C
Pelatan
Thierry
T
Merrot
Pascal
P
Delagausie
Philline
P
de Vries
Guillaume
G
Levard
Phillippe
P
Buisson
Emmanuel
E
Sapin
Olivier
O
Jaby
Corinne
C
Borderon
Dominique
D
Weil
Stephane
S
Gueiss
Didier
D
Aubert
Anais
A
Echaieb
Laurent
L
Fourcade
Jean
J
Breaud
Christophe
C
Laplace
Myriam
M
Pouzac
Alain
A
Duhamel
Frederic
F
Gottrand
D000015:Abnormalities, Multiple / Q000453:epidemiology; D000293:Adolescent; D000328:Adult; D001724:Birth Weight; D015331:Cohort Studies; D003131:Combined Modality Therapy; D004933:Esophageal Atresia / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery; D005260:Female; D005602:France / Q000453:epidemiology; D005865:Gestational Age; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D008297:Male; D008423:Maternal Age; D008875:Middle Aged; D006831:Polyhydramnios / Q000453:epidemiology; D011159:Population Surveillance; D011247:Pregnancy; D011296:Prenatal Diagnosis; D015995:Prevalence; D011446:Prospective Studies; D012042:Registries / Q000706:statistics & numerical data; D011795:Surveys and Questionnaires; D015996:Survival Rate; D016526:Workload; D055815:Young Adult
Cohort study; Epidemiology; Esophageal atresia; Neonatal surgery; Population-based registry; Prenatal diagnosis; Rare disease