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一名患有多囊性肾发育不良、肛门闭锁和阴茎阴囊转位的男婴出现膀胱缺如和双侧异位输尿管。

Bladder Agenesis and Bilateral Ectopic Ureters in an Infant Male With Cystic Renal Dysplasia, Imperforate Anus, and Penoscrotal Transposition.

作者信息

Omil-Lima Danly, Gupta Karishma, Prunty Megan, Miyasaka Eiichi A, Joyce Emily L, Nguyen Christina, Hannick Jessica H

机构信息

Urology Institute, University Hospitals Cleveland Medical Center, Cleveland, OH; Division of Pediatric Urology, University Hospitals, Rainbow Babies & Children's Hospital, Cleveland, OH.

Division of Pediatric Surgery, University Hospitals, Rainbow Babies & Children's Hospital, Cleveland, OH.

出版信息

Urology. 2021 Oct;156:256-259. doi: 10.1016/j.urology.2021.02.032. Epub 2021 Mar 6.

Abstract

Bladder agenesis is a rare congenital anomaly infrequently reported in the literature, with an incidence of 1/600,000 patients. Commonly associated with other fatal malformations, the condition is often incompatible with life. Prior reports estimate that over 90% of living children born with this malformation are female, owing to renal preservation resulting from low pressure drainage of urine into the vagina, uterus, and vestibule. Herein we report a rare case of an infant male born with penoscrotal transposition and end stage renal disease secondary to bilateral cystic renal dysplasia found to have concurrent bladder agenesis and bilateral ureteral ectopia.

摘要

膀胱发育不全是一种罕见的先天性异常,文献报道较少,发病率为1/600,000患者。该病症常与其他致命性畸形相关,通常无法存活。先前的报告估计,患有这种畸形的存活儿童中超过90%为女性,这是由于尿液低压引流至阴道、子宫和前庭从而保留了肾脏。在此,我们报告一例罕见病例,一名患有阴茎阴囊转位和终末期肾病(继发于双侧囊性肾发育不良)的男婴,同时存在膀胱发育不全和双侧输尿管异位。

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