新生儿VACTERL联合征

目的：提高对新生儿VACTERL联合征的诊断和治疗水平。方法：回顾分析食道闭锁12例和肛门闭锁28例，按VACTERL联合征的诊断标准，六种畸形中至少有三种畸形同时存在时，就可诊为VACTERL联合征。结果：共有11例诊为VACTERL联合征，诊断时间最早为2h，最迟为22d，平均诊断年龄3d。肛门闭锁半瘘9例，并食道闭锁7例，并先心7例，并椎体畸形6例，并泌尿生殖畸形5例，合并肢体畸形3例。11例中6例手术治疗，5例成活，1例死亡，另5例放弃治疗。5例随访6个月至2年，均健康。结论：VACTERL联合征是一组少见的先天性畸形，早期诊断很有必要，尤其是食道闭锁的诊断临床上应有足够的认识；积极治疗可获得正常的生存。

VACTERL Association in Neonates

Objective: To elevate the levels of diagnosis and treatment in neonate VACTERL association.Methods:12 cases of esophageal atresia and 28 cases of anal atresia in our hospital from 1999 to 2001 were retrospectively analyzed, according to the diagnostic criterion of VACTERL association.If there were at least 3 or more of 6 malformations,one could be diagnosed as the VACTERL association.Results:There were 11 cases of diagnosed VACTERL association.Time of early diagnosis was in 2 hours and lasted into 22 days,with an average time of 3 days.There were 9 cases of anal atresia or fistula,7 cases of esophageal atresia,7 cases of congenital heart diseases,and 6 cases of limb malformations.Of 11 cases, 6 cases were operated,5 survivals and 1 death.The other 5 cases were given up.All 5 survival cases had good health as follow up for 6 month to 2 years.Conclusion:VACETERL association is a group of rate congenital malformations.Early diagnosis is important,especially to detect some problems related to esophageal atresia.Following treatment actively can obtain normal survivals.