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

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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十阴道成形术;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.
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