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目的分析新生儿VACTERL联合征的临床表现及影像学特点,提高对本病的认识和诊断水平。方法选择2009年1月至2012年11月在本院放射科检查的食管闭锁和肛门闭锁患儿,按照VACTERL联合征的诊断标准,回顾X线影像资料,并分析患儿临床表现及影像学特点。结果研究期间在本院放射科进行检查的食管闭锁和肛门闭锁患儿共38例,其中4例食管闭锁患儿符合VACTERL联合征的诊断。1例食管闭锁并气管食管瘘、胸椎半椎畸形、右侧第11肋骨缺如、多指畸形;1例食管闭锁并先天性心脏病(房间隔缺损、动脉导管未闭)、右心房右心室扩大、双手拇指畸形;1例食管闭锁并气管食管瘘、双侧第13肋骨、脊柱裂、左肾积水、右肾缺如;1例食管闭锁并气管食管瘘、先天性心脏病(室间隔缺损、动脉导管未闭)、左手赘指畸形。结论新生儿VACTERL联合征临床和影像表现具有一定特点,临床和影像学检查结合可以做出该诊断。
Objective To analyze the clinical manifestations and imaging characteristics of neonatal VACTERL syndrome and to improve the understanding and diagnosis of this disease. Methods Children with esophageal atresia and anus at radiology from January 2009 to November 2012 were retrospectively reviewed according to the diagnostic criteria of VACTERL and the clinical manifestations and imaging features were analyzed . Results A total of 38 children with esophageal and anal atresia were examined in our hospital’s radiology department during the study period. Four of the children with esophageal atresia were diagnosed with VACTERL syndrome. 1 case of esophageal atresia and tracheal esophageal fistula, thoracic hemivertebra, absent 11th rib on the right, multiple finger deformities; 1 case of esophageal atresia with congenital heart disease (atrial septal defect, patent ductus arteriosus), right atrium right ventricle 1 case of esophageal atresia and tracheal esophageal fistula, bilateral 13th rib, spina bifida, left kidney hydronephrosis, right kidney lack; 1 case of esophageal atresia and tracheal esophageal fistula, congenital heart disease (interventricular septum Defects, patent ductus arteriosus), left hand refers to deformity. Conclusion Neonatal VACTERL conjunctival sign has some characteristics of clinical and imaging, clinical and imaging studies can make this diagnosis.