Zinner syndrome的儲精囊囊腫造成之下泌尿道症狀

吳冠儒¹、陳建志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

Case Report: Zinner Syndrome Presenting with Seminal Vesicle Cyst: A Rare Congenital Anomaly

Kuan-Ju Wu¹、Mareclo Chen¹

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

Introduction-

Zinner Syndrome is a rare congenital anomaly characterized by a triad of unilateral renal agenesis or dysplasia, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction. It is a variant of the more common clinical entity known as the "trigonitis-trigono-adenoma" complex. Herein, we present a case of Zinner Syndrome in an 18-year-old male, emphasizing the diagnostic challenges and management strategies associated with this condition.

Case Presentation-

An 18-year-old male presented with a history of intermittent urine flow over the past year. He reported weak stream, incomplete emptying, urination pain, urgency, and frequency. There were no associated symptoms of fever or abdominal pain. The patient had a past medical history significant for a right multicystic dysplastic kidney (MCDK) with follow-up, left ear hearing impairment, and a seminal vesicle cyst noted on imaging. The seminal vesicle cyst, initially clear in content, had become turbid over time. Renal ultrasonography revealed a left solitary kidney with parapelvic multiple cystic lesions and a seminal vesicle cyst measuring 30.1 x 19.8 x 21.1 mm. Uroflowmetry demonstrated a plateau-staccato shaped curve with a small voided volume (174.9 ml) and elevated post-void residual (PVR) volume (221.2 ml). The patient was admitted for further evaluation and management.

On examination, vital signs were within normal limits, and the patient appeared alert and oriented. Urine analysis showed hematuria, while urine culture was negative. Preoperative evaluation revealed no contraindications for surgery. Fibrocystoscopy revealed a large protruding lesion at the trigone, necessitating transurethral resection. Pathological examination confirmed the presence of a seminal vesicle cyst. Postoperatively, the patient recovered well and was discharged with a silicone Foley catheter in place for one month.

Discussion-

Zinner Syndrome, a rare developmental anomaly, results from the embryological malformation of the Wolffian duct derivatives. The classic triad includes unilateral renal agenesis or dysplasia, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction. Diagnosis often requires a high index of suspicion and multimodal imaging studies, including ultrasonography, computed tomography, and magnetic resonance imaging. Treatment aims to alleviate symptoms and prevent complications, typically through surgical intervention. Close follow-up is essential to monitor renal function and manage any associated complications.

In our case, the patient presented with symptoms related to bladder outlet obstruction secondary to the seminal vesicle cyst. Surgical resection provided symptomatic relief and confirmed the diagnosis. Although Zinner Syndrome is rare, clinicians should consider it in young males presenting with lower urinary tract symptoms and genital tract anomalies. Collaboration between urology and nephrology specialties is crucial for comprehensive management and long-term follow-up.

Conclusion-

Zinner Syndrome is a rare congenital anomaly characterized by a triad of renal, seminal vesicle, and ejaculatory duct anomalies. Early recognition and multidisciplinary management are essential for optimizing outcomes in affected individuals. Further studies are warranted to elucidate the underlying pathophysiology and refine treatment strategies for this rare condition.