title: Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification.
abstract: OBJECTIVE
  The aim of this study was to investigate contemporary patterns of presentation and outcome in newborns with esophageal atresia with or without tracheoesophageal fistula (OA) using modern prognostic criteria to appraise survival.
  
  
  METHODS
  Over a 12-year period (1986 through 1997), 134 patients with OA were admitted to a single institution. Patient demographics, the presence of cardiac defects, other associated abnormalities (VACTERL), surgical intervention, and patient outcome were recorded. To identify and evaluate changes in the pattern of clinical presentation, frequency of associated anomalies and outcome, patients were analyzed during 4 consecutive time periods, 1986 to 1988, 1989 to 1991, 1992 to 1994, and 1995 to 1997.
  
  
  RESULTS
  A primary repair or delayed primary repair was performed in 113 (84%) patients, with a staged procedure in 19 (14%). Two babies with trisomy 18 did not undergo surgery. Thirty-eight newborns (28%) had a major cardiac malformation (excluding patent ductus arteriosus, unless needing ligation), and 25 (19%) had recognized VACTERL associations. There was a significant increase in the proportion of infants with major cardiac defects diagnosed over the study period, 5 of 34 patients between 1986 and 1988 to 19 of 41 patients between 1995 and 1997 (chi(2) test, P <.001), but the incidence of VACTERL associations remained unchanged. Overall survival rate was 86% in those who underwent surgery. The relative risk of mortality in patients with major cardiac disease and VACTERL associations was 3.47 (95% CI; 1.51 to 7.96) and 2.54 (95% CI; 1.14 to 4.86), respectively. Birth weight was significantly higher in infants who survived (2.68 kg) compared with those who died (2.16 kg, P =.003). Thirty percent of infants with more than one system abnormality died compared with 8% of infants with 1, system abnormality (P =.004).
  
  
  CONCLUSIONS
  This study has found a significant increase in the frequency of cardiac abnormalities encountered in a cohort of OA patients during the period under review. Cardiac disease and multiple abnormalities carried a substantial increased risk of mortality. In the era of the Spitz classification, the phenotypic presentation is important to accurately assess caseload severity and prognosis.
authors.affiliation: Department of Paediatric Surgery and Institute of Child Health, Alder Hey Children's Hospital, The University of Liverpool, UK.
authors.forename: C P
authors.initials: CP
authors.lastname: Driver
authors.forename: K R
authors.initials: KR
authors.lastname: Shankar
authors.forename: M O
authors.initials: MO
authors.lastname: Jones
authors.forename: G A
authors.initials: GA
authors.lastname: Lamont
authors.forename: R R
authors.initials: RR
authors.lastname: Turnock
authors.forename: D A
authors.initials: DA
authors.lastname: Lloyd
authors.forename: P D
authors.initials: PD
authors.lastname: Losty
mesh_terms: D000015:Abnormalities, Multiple / Q000145:classification* / Q000401:mortality / Q000601:surgery*; D015331:Cohort Studies; D016001:Confidence Intervals; D004933:Esophageal Atresia / Q000145:classification* / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010641:Phenotype; D011336:Probability; D012189:Retrospective Studies; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D016019:Survival Analysis; D014138:Tracheoesophageal Fistula / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome