Abstract：
Genitourinary anomalies constitute a large proportion of congenital malformations. However seminal tract anomalies, particularly ejaculatory duct (ED) anomalies are very rare and little information exists on the topic. We are reporting a very rare case of bilateral ectopic EDs opening in the bladder trigone in a 33-year-old gentleman presenting for evaluation for primary infertility. The patient\'s semen analysis showed low-ejaculate-volume, fructose negative, acidic pH and azoospermia. His hormonal profile was normal. Cystoscopy revealed an empty posterior urethra, and the verumontanum and the openings of the EDs could not be identified in the posterior urethra. The ED openings were found inside the bladder trigone. Vasography combined with cystoscopy confirmed the opening of the ED in the trigone following Intra-vasal injection of methylene blue. Our patient had a successful intracytoplasmic sperm injection using testicular spermatozoa that resulted in a healthy baby boy. We also did a formal literature review through PUBMED, MEDLINE and Google Scholar with the search term (ectopic ED). Search results were filtered to exclude vas deferens ectopia. Our literature search revealed five studies comprising 24 patients with ectopic EDs. Mean age at diagnosis was 29.88 ± 12.88 years. The most common presenting symptom was hemospermia. The ectopic EDs most commonly opened in a midline cyst (21 cases), bladder trigone (1 case), or bladder neck (1 case). The most common management used for symptomatic patients with ectopic EDs opening in the midline cyst was through transurethral fenestration. In conclusion, ectopic ED openings in the bladder trigone are very rare. Management varies by case depending on the presentation, anatomy of underlying anomaly, associated complication/s and desire for fertility.
摘要：
泌尿生殖系统异常占先天性畸形的很大比例。然而，精道异常，特别是射精管（ED）异常非常罕见，关于该主题的信息很少。我们正在报道一例非常罕见的病例，该病例是一位33岁的绅士，正在评估原发性不孕症。患者的精液分析显示射精量低，果糖阴性,酸性pH和无精子症。他的荷尔蒙状况正常。膀胱镜检查显示后尿道空,在后尿道中无法识别Verumontanum和ED的开口。在膀胱三角区内发现了ED开口。血管造影结合膀胱镜检查证实了血管内注射亚甲蓝后三角部的ED开放。我们的患者使用睾丸精子成功进行了胞浆内精子注射，从而产生了一个健康的男婴。我们还通过PUBMED进行了正式的文献综述，MEDLINE和谷歌学者与搜索词（ectopicED）。对搜索结果进行过滤以排除输精管异位。我们的文献检索显示了5项研究，包括24例异位ED患者。诊断时的平均年龄为29.88±12.88岁。最常见的症状是血精症。异位ED最常见于中线囊肿（21例），膀胱三肽（1例），或膀胱颈（1例）。对于有症状的中线囊肿异位ED开口的患者，最常见的治疗方法是通过经尿道开窗术。总之,膀胱三角区的异位ED开口非常罕见。管理因情况而异，具体取决于演示文稿，潜在异常的解剖结构，相关的并发症和对生育的渴望。
