单侧肺不发育合并食管闭锁及远端气管食管瘘：两例报告
Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report of two cases.
作者信息
Hoffman M A, Superina R, Wesson D E
机构信息
Department of Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
出版信息
J Pediatr Surg. 1989 Oct;24(10):1084-5. doi: 10.1016/s0022-3468(89)80222-2.
The combined anomalies of pulmonary agenesis with esophageal atresia and distal tracheoesopahgeal fistula are an exceedingly rare and highly lethal association. The longest survivor in the literature is 10 months. We describe two neonates with this association who underwent primary repair shortly after birth and who are alive and well at 24 and 18 months. One of these neonates also demonstrated duodenal atresia. The prime goal in the management of these patients is early protection and preservation of respiratory units. This goal is best accomplished by primary repair of the tracheoesophageal lesion, when feasible, rather than by more complex procedures.
肺发育不全合并食管闭锁及远端气管食管瘘这种联合畸形极为罕见且致死率很高。文献中报道的最长存活时间为10个月。我们描述了两名患有这种联合畸形的新生儿，他们在出生后不久即接受了一期修复手术，目前分别为24个月和18个月，均存活且状况良好。其中一名新生儿还合并十二指肠闭锁。这些患者治疗的首要目标是早期保护和保留呼吸单位。在可行的情况下，通过对气管食管病变进行一期修复而非采用更复杂的手术来实现这一目标是最佳选择。
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