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

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: Azoospermia; Case report; Ejaculatory duct cyst; Zinner syndrome.

Fig. 1

A: Coronal T2 MRI: The left kidney and left ureter were absent (red arrow); B: sagittal view T2 MRI: the round cystic lesion (red arrow); the bladder (yellow arrow); C: axial T2 MRI: the round cystic lesion (red arrow); the bladder (yellow arrow); the left seminal vesicle can only be seen at this level and it had diminished in size (blue arrow); D: The melanin deposits on both sides of the verumonis (red arrow); E: The left ureteral orifice could not be explored (blue arrow); F-H: ①The bladder, ②cyst, ③the right vas deferens,④ the left vas deferens, ⑤the left shrunken seminal vesicle, ⑥which was similar to the seminal vesicle structure on the right side wall of the cyst; I-J: ①The bladder, ②the cyst was empty,③ the left vas deferens, ④the right vas deferens, ⑤the cyst was opened with no visible exit; K-L: Methylene blue was injected into both sides of the proximal vas deferens and methylene blue was seen draining from the inner surface of the cyst (yellow circle); M-N: Postoperative pathology. A: Gross specimen; B: cyst wall (black arrow). A small amount of atrophied seminal vesicle tissue was observed in the focal area around the cyst wall (red circle)