PURPOSE: The aim of this study was to investigate contemporary patterns of presentation and outcome in newborns with @entity1152 with or without @entity4501 ( @entity571 ) using modern prognostic criteria to appraise survival. METHODS: Over a 12-year period (1986 through 1997), 134 @entity1 with @entity571 were admitted to a single institution. @entity1 demographics, the presence of @entity299 , other associated abnormalities (VACTERL), surgical intervention, and @entity1 outcome were recorded. To identify and evaluate changes in the pattern of clinical presentation, frequency of associated anomalies and outcome, @entity1 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%) @entity1 , with a staged procedure in 19 (14%). Two babies with trisomy 18 did not undergo surgery. Thirty-eight newborns (28%) had a major @entity299 (excluding patent ductus arteriosus, unless needing ligation), and 25 (19%) had recognized VACTERL associations. There was a significant increase in the proportion of @entity1 with major @entity299 diagnosed over the study period, 5 of 34 @entity1 between 1986 and 1988 to 19 of 41 @entity1 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 @entity1 with major @entity299 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 @entity1 who survived (2.68 kg) compared with those who died (2.16 kg, P =.003). Thirty percent of @entity1 with more than one system abnormality died compared with 8% of @entity1 with 1, system abnormality (P =.004). CONCLUSIONS: This study has found a significant increase in the frequency of @entity299 encountered in a cohort of @entity571 @entity1 during the period under review. @entity299 and @entity156 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.

Phenotypic presentation and outcome of XXXX in the era of the Spitz classification.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'infants', 'Patient'] @entity4501 :: ('MESH:D014138', 'Disease') :: ['tracheoesophageal fistula'] @entity571 :: ('MESH:D010003', 'Disease') :: ['OA'] @entity1152 :: ('MESH:D004933', 'Disease') :: ['esophageal atresia'] @entity156 :: ('MESH:D000015', 'Disease') :: ['multiple abnormalities'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac defects', 'cardiac disease', 'cardiac abnormalities', 'Cardiac disease', 'cardiac malformation']
@entity1152 :: (MESH:D004933,Disease) :: ['esophageal atresia']
