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患有VATER相关异常的患者的手术问题。

Surgical problems in patients with VATER-associated anomalies.

作者信息

Muraji T, Mahour G H

出版信息

J Pediatr Surg. 1984 Oct;19(5):550-4. doi: 10.1016/s0022-3468(84)80102-5.

DOI:10.1016/s0022-3468(84)80102-5
PMID:6502426
Abstract

From 1960 through 1980, 14 patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) with two or more other associated anomalies of VATER have been seen. Seventy percent of patients were preterm or low in birth weight. In addition to the esophageal abnormality, vertebral anomalies were found in 7 patients, anorectal anomalies in 11, limb anomalies in 9 and renal anomalies in 9 patients. Three patients had associated duodenal atresia and 4 had cardiac anomaly. Eight patients are dead, 5 are living, and 1 is lost to follow-up. Six of the eight deaths occurred during 1960 to 1970. Preliminary gastrostomy was done in all patients. Primary esophageal repair was carried out in 9 patients--5 died and 4 survived. Three patients had delayed esophageal repair and two of these patients survived. Cervical esophagostomy was done in one patient with EA without TEF after unsuccessful repeated bouginage for elongation of the upper esophageal pouch. Three of the survivors showed growth retardation--below 5th percentile. Aggressive nutritional support and management of chronic problems related to the anomalies are important.

摘要

从1960年到1980年,共收治了14例食管闭锁(EA)患者,伴有或不伴有气管食管瘘(TEF),且合并两种或更多其他VATER相关畸形。70%的患者为早产儿或低体重儿。除食管异常外,7例患者存在椎体畸形,11例有肛门直肠畸形,9例有肢体畸形,9例有肾脏畸形。3例患者合并十二指肠闭锁,4例有心脏畸形。8例患者死亡,5例存活,1例失访。8例死亡患者中有6例发生在1960年至1970年期间。所有患者均进行了初步胃造瘘术。9例患者进行了一期食管修复,其中5例死亡,4例存活。3例患者进行了延迟食管修复,其中2例存活。1例无TEF的EA患者经反复探条扩张上食管袋延长术失败后,进行了颈部食管造口术。3例存活患者出现生长发育迟缓,低于第5百分位数。积极的营养支持以及对与畸形相关的慢性问题的处理很重要。

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引用本文的文献

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Surgical correction of tracheo-oesophageal fistula and oesophageal atresia in infants with VACTERL association: a retrospective case-control study.患有VACTERL综合征的婴儿气管食管瘘和食管闭锁的手术矫正:一项回顾性病例对照研究。
Pediatr Surg Int. 2012 Oct;28(10):967-70. doi: 10.1007/s00383-012-3165-8. Epub 2012 Aug 22.
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Posterior vertebral column resection for VATER/VACTERL associated spinal deformity: a case report.后路脊柱切除术治疗VATER/VACTERL相关脊柱畸形:1例病例报告
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