A rare variant of zinner syndrome with ejaculatory duct cyst: case report and challenges in diagnosis and management

Abstract

Abstract Background Zinner syndrome (ZS) is a congenital malformation characterized by a triad of mesonephric (Wolffian) duct dysplasia, first identified by Zinner in 1914. The classical presentation of ZS includes unilateral renal hypoplasia or dysplasia, ipsilateral seminal vesicle cysts, and obstruction of the ejaculatory duct. This case presents a rare variant of the syndrome, where an ejaculatory duct cyst is observed instead of the typical ipsilateral seminal vesicle cyst. The ejaculatory duct cyst affected the vas deferens bilaterally, leading to bilateral atrophy or erosion of the seminal vesicle glands, and resulted in the absence of seminal fluid, ultimately causing azoospermia and infertility. Case presentation Prior to surgery, the patient experienced a sensation of incomplete defecation, accompanied by mild anal distension. Two semen analyses revealed azoospermia, and magnetic resonance imaging/magnetic resonance urography indicated the absence of the left renal component and suggested the presence of a seminal vesicle cyst. It was hypothesized that the azoospermia resulted from compression of the contralateral ejaculatory duct by the seminal vesicle cyst on the affected side. Consequently, a decision was made to proceed with laparoscopic resection.During the surgical procedure, no seminal vesicle cyst was identified; however, an ejaculatory duct cyst was discovered, wherein the bilateral vasa deferentia converged without any alternative outlet. The cyst was subsequently resected. Postoperatively, the patient’s clinical symptoms resolved, although the issue of infertility remained unaddressed. Conclusion This case describes a rare Zinner syndrome variant where an ejaculatory duct cyst replaces the seminal vesicle cyst, leading to seminal vesicle atrophy, azoospermia, and infertility. An unreported variant was discovered during surgery, underscoring the importance of preoperative imaging. Laparoscopic removal alleviated symptoms but not infertility, indicating that assisted reproduction might be necessary for ZS-related azoospermia. This case expands knowledge of ZS variants and their impact on fertility.

Keywords

• Case report
• Zinner syndrome
• Ejaculatory duct cyst
• Azoospermia