Ein S H, Shandling B, Wesson D, Filler R M
Division of General Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr Surg. 1989 Oct;24(10):1055-9. doi: 10.1016/s0022-3468(89)80214-3.
Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.
1979年至1985年（含）期间，共治疗了97例患有食管闭锁并远端气管食管瘘（EA - TEF）的新生儿；其中男54例，女43例。他们的体重在800克至4000克之间（平均2.5千克）。这些新生儿包括：28例患有心脏缺陷的新生儿（最常见的是动脉导管未闭[PDA]、室间隔缺损和房间隔缺损[VSD - ASD]），其中18例存活（64%）；17例患有其他胃肠道异常（肛门闭锁、十二指肠闭锁）的婴儿，其中12例存活（70%）；12例患有骨骼畸形（多指、脊椎）的患者，其中11例存活（91%）；8例患有泌尿生殖系统异常（尿道下裂、隐睾）的新生儿，其中6例存活（75%）；以及16例患有其他先天性病变（18三体综合征、肺不发育 - 发育不全）的婴儿，其中3例存活（18%）。46例婴儿（平均体重2.7千克）无其他异常，全部存活。随着有缺陷系统数量的增加，婴儿体重和存活率均下降。在这97例患有EA - TEF的新生儿中，81例（83%）存活（平均体重2.3千克）。16例婴儿死亡（平均体重1.9千克）；11例存在与生命不相容的缺陷。16例中11例未接受手术（7例18三体综合征患者在5天内死亡，4例患有复杂心脏缺陷的患者在3周内死亡）。16例接受手术的患者中有4例在3个月至2岁之间因胸部问题死亡，1例新生儿在手术室死亡。我们得出结论：（1）如果没有其他异常，患有常见类型EA - TEF的新生儿几乎肯定会存活；（2）最常伴发的先天性缺陷是心血管系统（28%）、胃肠道（17%）、骨骼（12%）和泌尿生殖系统（8%）；（3）随着有缺陷系统数量的增加，婴儿体重及其存活率下降；（4）11%的患者会患有18三体综合征和/或复杂心脏缺陷，无法存活。
