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膀胱缺如及双侧异位输尿管一例:病例报告

A case of urinary bladder agenesis and bilateral ectopic ureters: a case report.

作者信息

Nazer Iman Ibrahim, Alhashmi Ghufran, Sharief Sara Nawfal, Hefni Nada Abdullatif, Ibrahim Abdulrahman, El-Desoky Sherif M, Alsayyad Ahmed Jalal, Safdar Osama Yousef, Kari Jameela A

机构信息

King Abdulaziz University (KSA), Jeddah, Kingdom of Saudi Arabia.

Department of Radiology, Jeddah, Kingdom of Saudi Arabia.

出版信息

BMC Urol. 2018 Sep 26;18(1):83. doi: 10.1186/s12894-018-0396-6.

Abstract

BACKGROUND

Urinary bladder agenesis is a very rare congenital anomaly with very few cases reported in the literature.

CASE PRESENTATION

We report a one-month-old baby presenting with ambiguous genitalia and recurrent urinary tract infections. Her clinical course was complicated by renal impairment. Magnetic resonant imaging (MRI) revealed a diagnosis of bladder agenesis with bilateral ectopic insertion of the ureters into the vagina, associated with several other anomalies. The patient underwent bilateral high anterior ureterostomies in an hospital abroad at 5.5 months of age. She then developed ureteral necrosis that had to be corrected with left pyeloplasty and by placing a left nephrostomy tube for drainage. Eventually, the patient's renal function declined, and she developed chronic kidney disease (CKD).The case with its imaging findings and pathogenesis as well as a review of the literature are presented.

CONCLUSIONS

Urinary bladder agenesis is a rare congenital condition that can be associated with multiple anomalies. Early diagnosis and therapeutic intervention can prevent progression to chronic kidney disease.

摘要

背景

膀胱发育不全是一种非常罕见的先天性异常,文献报道的病例极少。

病例介绍

我们报告一名1个月大的婴儿,表现为生殖器模糊和反复尿路感染。她的临床病程因肾功能损害而复杂化。磁共振成像(MRI)显示诊断为膀胱发育不全,双侧输尿管异位插入阴道,并伴有其他几种异常。该患者在国外一家医院5.5个月大时接受了双侧高位前输尿管造口术。随后她出现输尿管坏死,必须通过左肾盂成形术和放置左肾造瘘管进行引流来纠正。最终,患者的肾功能下降,并发展为慢性肾脏病(CKD)。本文介绍了该病例的影像学表现、发病机制以及文献复习。

结论

膀胱发育不全是一种罕见的先天性疾病,可伴有多种异常。早期诊断和治疗干预可预防进展为慢性肾脏病。

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