VACTERL联合征45例临床分析  

Clinical analysisi of 45 cases with VACTERL association

作　　者：刘仕祺 姜敏 齐宇洁 刘靖媛 董世霄 翁景文 黄金狮 黑明燕 靳绯 Liu Shiqi;Jiang Min;Qi Yujie;Liu Jingyuan;Dong Shixiao;Weng Jingwen;Huang Jinshi;Hei Mingyan;Jin Fei(Neonatal Center,Beijing Children,Hospital,Capital Medical University,National Center for Children Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院新生儿中心,100045

出　　处：《北京医学》2021年第7期597-600,共4页Beijing Medical Journal

摘　　要：目的总结VACTERL联合征患儿围产期特点、临床特征、治疗及疾病转归情况。方法选择2016年1月至2020年7月首都医科大学附属北京儿童医院新生儿中心收治的VACTERL联合征患儿45例,回顾性分析一般资料、围产期情况、临床特点、治疗及出院转归情况,分析转归相关影响因素。并根据存在畸形的特点,探讨食管闭锁组及非食道闭锁组间治疗及转归可能存在的差异。结果45例患儿中,男29例,女16例,小于胎龄儿14例(31.1%)。母孕期合并妊娠期糖尿病7例(15.6%),孕期发现羊水多7例(15.6%),羊水少6例(13.3%)。最常见的畸形为肛门闭锁及心脏发育畸形,均为34例(75.6%),其次为食管闭锁21例(46.7%),脊柱畸形20例(44.4%),肾发育畸形20例(44.4%),肢体畸形6例(13.3%)。以肛门闭锁、肾畸形及心脏发育畸形的组合最常见。共38例行外科手术治疗,食管闭锁组抗菌药物应用时间、住院时间、平均费用均高于非食管闭锁组,差异有统计学意义(P<0.05),围术期合并症更多。合并食管闭锁是疾病不良转归的危险因素(P<0.05)。结论VACTERL联合征是一组先天性联合畸形,畸形的类型与治疗及转归密切相关,需要早期进行全面系统的检查及评估。Objective To summarize the perinatal characteristics,clinical features,treatment and outcome of VACTERL association.Methods A retrospective analysis was conducted on the clinical data of neonates with VACTERL association,in the neonatal center of Beijing Children’s Hospital,Capital Medical University from January 2016 to July 2020.The general data,perinatal conditions,clinical characteristics,treatment and outcome were summarized,the related influencing factors of outcome were analyzed.The patients were divided into esophageal atresia group and non-esophageal atresia group to explore the difference of treatment and outcome between the two groups.Results A total of 45 cases were diagnosed with VACTERL association,including 29 males,16 females,14 infants were small for gestational age.Gestational diabetes mellitus was found in seven cases(15.6%),polyhydramnios was found in seven cases(15.6%)and oligohydramnios was found in six cases(13.3%).The most common malformations were anal atresia and cardiac defects(34 cases respectively,75.6%),followed by esophageal atresia(21 cases,46.7%),vertebral defects(20 cases,44.4%),renal defects(20 cases,44.4%),limb anomalies(six cases,13.3%).The combination of anal atresia,renal defect and cardiac defect was the most common.A total of 38 cases were carried out surgical treatment,the application time of antibiotics,hospitalization time and average cost in esophageal atresia group were higher,with more complications in perioperative period.Univariate analysis showed that esophageal atresia was a risk factor for adverse outcome(P<0.05).Conclusions VACTERL association is a group of congenital combined malformations.The type of the malformation are closely related to the treatment and outcome of the disease.It needs early comprehensive examination and evaluation.

关 键 词：VACTERL联合征 婴儿 临床特征 预后 

分 类 号：R725.9[医药卫生—儿科]