产前超声诊断双胎之一尿直肠隔畸形序列征1例  

Prenatal sonographic diagnosis of urorectal septum malformation sequence in one of twins: a case report

作　　者：胡培 郑东平 郑霜 肖彬 胡天鑫 曹婧芳 景红霞 Hu Pei;Zheng Dongping;Zheng Shuang;Xiao Bin;Hu Tianxin;Cao Jingfang;Jing Hongxia(Department of Ultrasound,Renmin Hospital,Hubei University of Medicine,Shiyan 442000,China)

机构地区：[1]湖北医药学院附属人民医院超声影像中心,十堰442000

出　　处：《中华围产医学杂志》2023年第9期766-768,共3页Chinese Journal of Perinatal Medicine

摘　　要：尿直肠隔畸形序列征(urorectal septum malformation sequence,URSMS)是一种罕见的先天性的复杂畸形,通常包括严重的泌尿系统、生殖系统以及消化系统畸形。URSMS超声表现复杂多变,产前超声诊断比较困难。本文报告了1例双胎之一合并部分型URSMS。患儿女性,产前超声表现为盆腔“三叶状”囊性包块、骶尾部半椎体畸形、肛门闭锁及一过性腹腔积液等,出生后未见肛门,且结肠、尿道及阴道汇入共同通道后开口于会阴部,考虑URSMS,拟行手术治疗,家属放弃治疗后新生儿死亡。Urorectal septum malformation sequence(URSMS)is a rare congenital complex malformation characterized by severe abnormalities in the urinary,reproductive and digestive systems.It is difficult to diagnose URSMS by prenatal ultrasound due to its complex and variable manifestations.This paper reported a twin with partial URSMS.Prenatal ultrasound findings included pelvic"trilobe"cystic masses,sacrococcygeal hemivertebral malformations,imperforate anus,and transient ascites.Postnatal examination confirmed the diagnosis of URSMS,as the baby girl was born with anal atresia.Her colon,urethra,and vagina converged and formed a common tract with a single perineal opening.The baby died after her parents'refusal to surgical treatment.

关 键 词：畸形 多发性 泌尿生殖系统畸形 肛门闭锁 泄殖腔 超声检查 产前 双生 

分 类 号：R445.1[医药卫生—影像医学与核医学] R714.5[医药卫生—诊断学]