個案報告: Zinner syndrome

蔡宏偉¹ 、徐宏仁 ¹

¹台灣基督長老教會馬偕醫療財團法人馬偕紀念醫院泌尿科

Zinner syndrome: a case report

Hung Wei Tsai¹、Hung Ren Hsu ¹

¹Department of Urology, MacKay Memorial Hospital, Taipei, Taiwan

Introduction:

We all knew that the classic triad of Zinner syndrome included: unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. The syndrome is due to malformation of the mesonephric duct during embryogenesis [1]. Nowadays, the diagnosis might be made in early childhood due to prevalence of prenatal examinations.

Case description:

This 38-year-old male with no significant medical history presented with hematospermia and persistent hematuria and thus visited our ER for evaluation. His lab data showed Hb: 14.7g/dL, Platelet: 239000uL, Creatinine: 1.1mg/dL. Urine analysis revealed hematuria with Occult Blood: 3+, Nitrite: Positive, RBC: Numerous/HPF. Bladder echo showed much blood clot and residue urine in bladder. A 20 Fr. Nephrostomy tube was placed and Foley irrigation was done at ER. Abdominal CT was also arranged due to suspicious of malignant potential. The report discovered that Absence of left kidney & upper ureter and prominence of soft tissue since middle third ureter to UVJ, suspicious of seminal vesicle cyst. Due to above, an emergency operation was made. Cystoscopy was arranged and many blood clots were washed out. A rapture cyst over left trigone with bleeding was noted. A
lumen was also behind. Left URS revealed seminal tubular-like space with much septum and a little bit ozzing, r/o opening of seminal vesicle. Hemostasis was done and a 3-way Foley of continuous irrigation was added as post-OP order. After 3 days of admission, the patient was discharged without Foley and may be arranged further MRI image in OPD.

Discussion and Summary:

The patient was first presented with hematuria with blood clots with any trauma history. In general medicine, our first impression may be malignant tumor.  However, the whole picture of history taking since childhood may be forgotten in non-pediatric doctor. The management of seminal vesicle cyst include: occasional percutaneous drainage or transrectal or transurethral cyst aspiration (for small cysts and those that are asymptomatic or cause only mild symptoms) and laparoscopically or with the use of robotic-assisted technology( for large cyst associated with significant clinical symptoms). Since radical prostatectomy was a prevalence surgery now, Zinner syndrome with symptomatic seminal vesicle cyst could be treated well in many centers.