Downard Cynthia D, Kim Heung Bae, Laningham Fred, Fishman Steven J
Department of Surgery, Children's Hospital Boston, Boston, MA, USA.
J Pediatr Surg. 2004 Aug;39(8):1283-5. doi: 10.1016/j.jpedsurg.2004.04.030.
The association of pure esophageal atresia, duodenal atresia, and unilateral lung agenesis has not been reported previously. Here the authors present a case of a newborn with this constellation of anomalies that underwent staged repair. The primary principle guiding treatment was the avoidance of iatrogenic injury to the single lung. Therefore, the order of operations proceeded as follows: (1) placement of a decompressing gastrostomy tube, (2) repair of the duodenal atresia, and (3) repair of the esophageal atresia. The congenital closed loop obstruction caused by the esophageal and duodenal atresias was beneficial in that it resulted in growth by stretching of the distal esophagus, allowing a tension-free primary repair of the esophageal atresia.
单纯食管闭锁、十二指肠闭锁和单侧肺不发育的关联此前尚未见报道。本文作者报告了一例患有这组异常的新生儿病例,该患儿接受了分期修复手术。指导治疗的主要原则是避免对单肺造成医源性损伤。因此,手术顺序如下:(1)放置减压胃造瘘管,(2)修复十二指肠闭锁,(3)修复食管闭锁。由食管和十二指肠闭锁引起的先天性闭环梗阻有其益处,即它导致远端食管通过伸展而生长,从而能够对食管闭锁进行无张力一期修复。