先天性肛门闭锁伴前庭窝瘘、并双阴道双子宫、右肾缺如及脊髓栓系畸形1例分析  

A Case Report of Double Vagina,Uterus,Absence of Right Kidney and Thered Cord Malformations in Infant's Congenital Anal Atresia and Vestibular Fistula

作　　者：宋东建[1,2] 岳丽芳[3] 范应中[1] 杨合英[1] 刘秋亮[1] 张大[1] 夏自强[1] 贾佳[1] 岳铭[1] 王家祥[1] 

机构地区：[1]郑州大学第一附属医院小儿外科,河南郑州450052 [2]河南省高等学校临床医学重点学科开放实验室,河南郑州450052 [3]郑州大学第三附属医院超声科,河南郑州450052

出　　处：《医学与哲学（B）》2016年第2期74-76,共3页Medicine & Philosophy(B)

摘　　要：回顾性分析郑州大学第一附属医院小儿外科收治的1例以肛门闭锁伴前庭窝瘘为主要表现,合并双阴道、双宫颈、双子宫、右肾缺如及脊髓栓系罕见组合畸形患儿的临床资料,根据患儿临床症状、体征、彩超、瘘管造影、倒立侧位X线及椎体MRI结果进行诊断,并结合处理过程中的几点争议进行简要讨论。完善术前检查,行改良式会阴部肛门成形+阴道成形术;脊髓栓系未出现症状,对侧肾脏代偿满意,密切观察。小儿肛门闭锁伴前庭窝瘘,合并双阴道、双宫颈、双子宫、右肾缺如及脊髓栓系组合畸形,虽然少见,但应当引起重视;提高各级儿保及儿科医务人员对先天肛门闭锁合并畸形的认识,精心的术前、术后护理是提高肛门闭锁治愈率及术后康复的关键。Clinical data of the infant with congenital anal atresia and vestibular fistula treated in the Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University was retrospectively analyze. According to the symptom, signs, ultrasonography, fistula angiography, inverted lateral X-ray and magnetic resonance imaging, the diagnosis was made and some controversies were discussed during the treatment process. Improving the preoperative examination, surgery of modified perineum anoplasty and vaginoplasty was conducted for the infant. In conjunction with outpatient reexamination with telephone follow-up, the infant was closely observed due to absence of tethered cord symptom and satisfactory left kidney compensatory. Congenital anal atresia and vestibular fistula with double vagina, uterus, absence of right kidney and thered cord malformations is a rare disorder and less recognized in infants and more attention should be focused. Consequently, careful preoperative and postoperative nursing care is the key to improve the cure rate and postoperative rehabilitation by improving the ability of basic unit medical staff in well understanding congenital anal atresia and vestibular fistula accompanied with other malformations.

关 键 词：肛门闭锁 双阴道 双子宫 脊髓栓系 畸形 

分 类 号：R726.1[医药卫生—儿科]