先天性尿道皮肤瘘2例并文献复习

Congenital urethrocutaneous fistula:a report of two cases and literatures review

目的探讨先天性尿道皮肤瘘的发病机制、诊断及治疗。方法回顾性分析我院收治的2例先天性尿道皮肤瘘的临床资料，并文献复习。结果两例患儿均有正常的阴茎头正位尿道开口，不伴有阴茎下屈、尿道下裂，包皮分布均匀。完善术前检查，排除手术禁忌证，行尿瘘修补术，术中取阴囊“舌形”转移带蒂皮瓣加盖固定。随访6个月，无发生尿瘘、尿道狭窄等并发症。结论先天性尿道皮肤瘘是一种罕见的先天性尿道畸形，可伴或不伴其他畸形，如尿道下裂、阴茎下屈及肛门直肠畸形等。临床上易于诊断，但需仔细检查以排除是否合并其他畸形，选择最佳的手术方式。

Objective To explore the pathogenesis, diagnosis and treatment of the congenital urethrocutaneous fistula. Methods Clinical data of two cases with congenital urethrocutaneous fistula were retrospectively analyzed and the related literatures were reviewed. Results The children patients had abnormal opening on the ventral aspect of penis with a normal foreskin and an absence of chordee and hypospadias. And they received a repair with the scrotum ＂ligule＂ flaps. No urethral stricture, urethrocutaneous fistula occurred during 6 months following-up. Conclusion Congenital urethrocutaneous fistula is an rare urinary tract deformities with chordee or hypospadias or not, in children. Congenital urethrocutaneous fistula is an extremely rare but easily manageable anomaly and a good clinical examination is needed for diagnosis and ruling out other associated anomalies, and then select the reasonable operational manner.