Imaging in Zinner Syndrome, A Case Series: The Wolf in Sheep’s Clothing





Abstract


Zinner syndrome is a rare congenital malformation of the Wolffian duct with seminal vesicle cyst, ipsilateral renal agenesis and ejaculatory duct obstruction, seen in less than 0.002 % of the male population. An increased awareness among clinicians, urologists, and radiologists is essential, as early diagnosis and management can impact fertility outcomes. This review aims to explain and illustrate Zinner syndrome through imaging case studies. Emphasis is made on considering Zinner syndrome in the differential diagnosis of young males with pelvic pain and renal agenesis, highlighting the importance of radiological evaluation for the management of this uncommon yet clinically significant condition.


Highlights




  • •

    The classic triad of Zinner’s syndrome consists of renal agenesis, seminal vesicle cysts, and ejaculatory duct obstruction.


  • •

    Transabdominal ultrasound, TRUS, and MRI are essential modalities to reach an accurate diagnosis.


  • •

    Symptomatic patients benefit from surgical intervention, with recent advancements in laparoscopic and robotic-assisted techniques providing optimism for better outcomes.


  • •

    Detailed and timely imaging, clinical expertise and early intervention is crucial to diagnose and manage such unique conditions.



Abbreviations:


CT


Computed tomography


H&E


Hematoxylin and Eosin


LIF


Left Iliac Fossa


MRI


Magnetic Resonance Imaging


OPD


Out-Patient Department


TAS


Transabdominal Ultrasound Scan


STIR


Short Tau Inversion Recovery


TRUS


Transrectal Ultrasound Scan


USG –


Ultrasound


UTI


Urinary Tract Infection


VUR


Vesicoureteric Reflux


1


Introduction


Zinner syndrome was first described by Zinner in 1914. It is an unusual congenital Wolffian duct malformation characterized by the triad of seminal vesicle cysts, ipsilateral renal agenesis, and ejaculatory duct obstruction. This condition’s incidence is estimated to be less than 0.002 % in the male population with only a handful of 200 cases reported to date. Normally, both the ureteral buds and seminal vesicles arise from the mesonephric duct. A defect in the formation of the distal/dorsal mesonephric duct results in atresia of the ejaculatory duct, causing obstruction and dilatation of the seminal vesicle. Increasing awareness among clinicians, urologists, surgeons, and radiologists is crucial due to the significant clinical and fertility implications of Zinner syndrome. Timely and accurate diagnosis through multimodal imaging techniques is essential to guide appropriate therapeutic interventions and improve patient outcomes, reproductive health, and quality of life.


2


Case presentation


The first patient is a 38 year old male who presented to the Out-Patient Department (OPD) with symptoms of intermittent perineal and pelvic pain, on and off fever since 8 months, dysuria with burning micturition, occasional haematuria, hematospermia confirmed by urine and semen analysis which also showed oligospermia (3,500,000/milliliter) with reduced volume. Marital history revealed secondary infertility and recent onset painful ejaculation. In view of above clinical findings, a TRUS was advised in suspicion of prostatitis.


TRUS revealed a large complex multilocular cystic lesion with internal echogenic contents, septations and fluid levels arising from the right seminal vesicle. The left seminal vesicle appeared bulky and edematous suggestive of vesiculitis. Dilated right more than left vas deferens secondary to dilated & obstructed distal ejaculatory duct were observed. The proximal ejaculatory duct could not be visualized. Prostate appeared edematous, showing heterogenous echotexture, periprostatic oedema and increased vascularity due to prostatitis ( Fig. 1 ).




Fig. 1


TRUS shows (A) complex cyst replacing the right seminal vesicle (orange arrow). (B) Bulky and edematous left seminal vesicle with heteroegenous echotexture and increased vascularity suggestive of vesiculitis (blue arrow). (C) Dilated right more than left vas deferens (black arrow) secondary to ejaculatory duct obstruction. Heterogenous and edematous prostate with periprostatic oedema and increased vascularity suggestive of prostatitis (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)


A provisional diagnosis on TRUS was suggestive of right complex seminal vesicle cyst. A differential diagnosis of right seminal vesicle abscess was also considered. Patient underwent an extensive antibiotic course and came for a follow up scan after 1 month with no significant interval change in the volume of the cystic lesion. However, there was some intermittent improvement in his clinical symptoms with no further worsening. Patient was further planned for TRUS guided aspiration and drainage.


However, an out of the box follow-up scan with a vigilant and timely abdominal ultrasound beyond the eyes and need of the clinical symptoms was done which incidentally revealed an absent right kidney with compensatory hypertrophy of the left kidney ( Fig. 2 ). Now, a diagnosis of an associated Wolffian duct abnormality was made and MRI pelvis was suggested to confirm the diagnosis.


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May 7, 2025 | Posted by in UROLOGY | Comments Off on Imaging in Zinner Syndrome, A Case Series: The Wolf in Sheep’s Clothing

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