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一例青少年先天性尿道会阴瘘管理的病例报告,这是一种罕见的先天性异常。

A case report on the management of congenital urethroperineal fistula in an adolescent, a rare congenital anomaly.

作者信息

Shiferaw Samuel Fekadu, Molla Mezgeb Gedefe, Feye Yoseph Abebe

机构信息

Addis Ababa University, CHS, School of Medicine, Department of Surgery, Urology Unit, Addis Ababa, Ethiopia.

Addis Ababa University, CHS, School of Medicine, Department of Surgery, Urology Unit, Addis Ababa, Ethiopia; Menelik II Comprehensive Specialized Hospital, Department of Surgery, Addis Ababa, Ethiopia.

出版信息

Int J Surg Case Rep. 2025 Mar;128:111043. doi: 10.1016/j.ijscr.2025.111043. Epub 2025 Feb 11.

Abstract

INTRODUCTION

Congenital posterior urethra perineal fistula (CUPF) is a rare congenital GU anomaly with abnormal epithelium-lined communication between the posterior urethra and perineum. Some consider it to be a variant of Y-type urethral duplicate with ventral hypoplastic urethra and have suggested its adoption to the Eiffmans classification.

CASE PRESENTATION

An 18-year-old male patient presented with urine dribbling from the perineum since childhood. He has no history of urinary tract infection and surgical procedure and instrumentation. There was normal external genitalia with meatus positioned at the tip of the glans; there was a small opening on the posterior scrotal wall near the perineum that admits a 6fr NG tube. VCUG showed a fistula from the prostatic urethra to the perineum. He was managed with near-total excision of the fistula tract, with no recurrence on a 3-month follow-up.

DISCUSSION

Patients with this rare condition present with dribbling from perineum during voiding, underwear wetting and recurrent UTI. Voiding CUG is a diagnostic procedure of choice for patients with CUPF. MRI can be used if there is interest in detail anatomy of the fistula tract and its relationship to the surrounding structures and is also helpful if other modalities show a non-conclusive result. In managing this patient, differentiating the dominant and functional urethra is of clinical significance. Complete or partial excision, electrofulgration of the fistula tract are options.

CONCLUSION

Voiding CUG is a diagnostic procedure of choice for patients with CUPF and partial excision of the fistula tract is an option of management with satisfactory result.

摘要

引言

先天性后尿道会阴瘘(CUPF)是一种罕见的先天性泌尿生殖系统异常,后尿道与会阴之间存在异常的上皮内衬通道。一些人认为它是Y型重复尿道伴腹侧发育不全尿道的一种变体,并建议将其纳入艾夫曼分类法。

病例报告

一名18岁男性患者自童年起就出现会阴部滴尿症状。他无尿路感染及手术、器械操作史。外生殖器正常,尿道口位于龟头顶端;会阴附近阴囊后壁有一小开口,可插入一根6F的鼻胃管。排尿性膀胱尿道造影显示前列腺尿道与会阴之间存在瘘管。对其进行了瘘管近全切除术,3个月随访无复发。

讨论

患有这种罕见疾病的患者在排尿时会出现会阴部滴尿、内裤浸湿及反复尿路感染。排尿性膀胱尿道造影是诊断CUPF患者的首选检查方法。如果需要了解瘘管的详细解剖结构及其与周围结构的关系,可使用磁共振成像(MRI),当其他检查方法结果不明确时,MRI也有帮助。在处理该患者时,区分主要和功能性尿道具有临床意义。完全或部分切除、瘘管电灼都是可选的治疗方法。

结论

排尿性膀胱尿道造影是诊断CUPF患者的首选检查方法,瘘管部分切除术是一种治疗选择,效果满意。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3dd/11872632/a074c79dd16d/gr1.jpg

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