Zinner 症候群導致青少年長期嚴重會陰疼痛之個案報告

蔡勝閔、謝昆霖、黃冠華

奇美醫療財團法人奇美醫院 外科部 泌尿科

Case report: Zinner syndrome in adolescent with chronic severe perineal pain

Sheng-Min Tsai, Kun-Lin Hsieh, Guan-Hua Huang

Divisions of Urology, Department of Surgery, Chi Mei Hospital

 

Introduction:

Zinner's syndrome is a rare congenital abnormality of mesonephric (Wolffian) duct consisting of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction, which was first described by Zinner et al. in 1914. Most patients remain asymptomatic till the second to fourth decade of life relating to the period of highest sexual and reproductive activity. It may cause symptoms such as perineal pain, painful ejaculation, hematospermia, abnormal micturition, lower urinary tract symptoms, and dysuria. Here, we present the case of a teenager with long term perineal pain which was later diagnosed with Zinner syndrome.

Case presentation:

The illustrated case is a 16-year-old teenager who has a medical history of congenital single kidney. He presented to the urology department due to chronic perineal pain and dysuria. He suffered from similar symptoms since he was 12. Recurrent prostatitis and bacterial sperm infection were impressed initially. Transrectal ultrasound showed seminal vesicle cyst. MRI revealed the agenesis of the right kidney and lobulated cystic lesion with hemorrhage in the right seminal vesicle. Zinner syndrome was then diagnosed. Refractory perineal pain was noted under palliative treatment. Transurethral seminal vesiculoscopy was performed and the pain subsided dramatically. CT-guided percutaneous aspiration of the right seminal vesicle cyst was further arranged for relief of symptoms.

Discussion:

Zinner syndrome is one of the rarest congenital anomalies of urogenital tract. The mesonephric duct gives rise to most of the genital tract including epididymis, vas deferens, ejaculatory duct, and seminal vesicle. Any malformation of the mesonephric duct can cause Zinner syndrome. There is a need to raise awareness among young patients with renal agenesis and chronic perineal pain. The treatment of Zinner syndrome depends on the size of the cyst and clinical symptoms of the patient. If the symptoms are mild, percutaneous drainage, or transrectal or transurethral aspiration of seminal vesicle cyst is suitable. Surgical intervention includes open, laparoscopic or robotic vesiculectomy.
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    台灣泌尿科醫學會
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    2021-05-24 16:37:51
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