Driver C P, Shankar K R, Jones M O, Lamont G A, Turnock R R, Lloyd D A, Losty P D
Department of Paediatric Surgery and Institute of Child Health, Alder Hey Children's Hospital, The University of Liverpool, UK.
J Pediatr Surg. 2001 Sep;36(9):1419-21. doi: 10.1053/jpsu.2001.26389.
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.
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.
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).
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.
本研究旨在利用现代预后标准评估生存情况，调查患有或不患有气管食管瘘（OA）的食管闭锁新生儿的当代临床表现模式和预后。
在12年期间（1986年至1997年），134例OA患者被收治于单一机构。记录患者的人口统计学资料、心脏缺陷的存在情况、其他相关异常（VACTERL综合征）、手术干预及患者预后。为确定和评估临床表现模式、相关畸形频率及预后的变化，对1986年至1988年、1989年至1991年、1992年至1994年以及1995年至1997年这4个连续时间段的患者进行分析。
113例（84%）患者接受了一期修复或延迟一期修复，19例（14%）患者接受了分期手术。两名18三体综合征患儿未接受手术。38例新生儿（28%）患有严重心脏畸形（不包括动脉导管未闭，除非需要结扎），25例（19%）患有已确认的VACTERL综合征。在研究期间，诊断出患有严重心脏缺陷的婴儿比例显著增加，从1986年至1988年34例患者中的5例增加到1995年至1997年41例患者中的19例（χ²检验，P<.001），但VACTERL综合征的发生率保持不变。接受手术的患者总体生存率为86%。患有严重心脏疾病和VACTERL综合征的患者的相对死亡风险分别为3.47（95%CI：1.51至7.96）和2.54（95%CI：1.14至4.86）。存活婴儿的出生体重（2.68kg）显著高于死亡婴儿（2.16kg，P=.003）。有一个以上系统异常的婴儿中有30%死亡，而有1个系统异常的婴儿中这一比例为8%（P=.004）。
本研究发现，在所审查期间，一组OA患者中遇到的心脏异常频率显著增加。心脏疾病和多种异常显著增加了死亡风险。在斯皮茨分类时代，表型表现对于准确评估病例负荷的严重程度和预后很重要。
