案例報告：罕見泌尿道疾病– Zinner綜合症

李昀叡¹、林仁泰¹、余家政¹、陳逸軒^1,2

高雄榮總外科部泌尿外科¹

高雄榮總外科部移植外科²

Case report: A rare urological disease – Zinner syndrome

Yun-Jui Li¹, Jen-Tai Lin¹, Chia-Cheng Yu¹, I-Hsuan Chen^1,2

¹Division of Urology, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

²Division of Transplant Surgery, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

Introduction:

Zinner syndrome is a rare urological triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. Generally, it is associated with the abnormal development of the Wolffian duct in the first trimester of gestation. Incomplete migration of the ureteric bud causes failure to connect with the metanephros, which leads to renal agenesis. However, the seminal vesicles continue to develop with insufficient drainage, resulting in cyst formation. This syndrome is mostly asymptomatic until the third or fourth decade of life, and symptoms usually manifest with the beginning of sexual activity. Patients commonly present with unspecific symptoms such as voiding (dysuria, urgency or frequency), perineal pain, hematuria, recurrent urinary tract infections, painful ejaculation, and infertility.

Case Report:

This is a 40-year-old male without underlying disease. According to medical history, he suffered from terminal dribbling in 2011 when he was just 28 years old. The military check-up revealed an atrophied right kidney with seminal vesicle cyst. Then, he received cystoscopy with retrograde pyelography which showed right ureteral remnant with communication to seminal vesicle and cystic formation of seminal vesicle. However, he lost follow up since last admission. Until 2024, he visited our OPD again with complain of blood-tinged urine in early February. He also received flexible cystoscopic biopsy at KCGMH which showed cystitis glandularis. Then, the whole abdominal CT was arranged which showed agenesis of right kidney and upper urinary tract, with a complex cystic lesion in size of 4.5 cm at the right side seminal vesicle region connecting to urinary bladder, companied with seminal vesicle cyst with right lower third ureteric stump. Considering the CT finding, Zinner syndrome was highly suspected. 

Conclusion:

Zinner syndrome showed with varied clinical presentation. Physicians and radiologists should be aware of the clinical condition and radiological presentation. Magnetic resonance imaging is the imaging modality of choice for diagnosis. Management with surgical intervention is aimed at pain relief for severe case; otherwise, observation with regular follow up is available for asymptomatic case.