The Zinner Trifecta with Ipsilateral Hypoplastic Ectopic Ureter: A Radiological Revelation

Abstract Zinner syndrome is an uncommon birth defect affecting both the urinary and reproductive systems. This condition is characterized by the presence of three key features: unilateral renal agenesis, seminal vesicle cysts, and ejaculatory duct obstruction. Beyond the core characteristics of Zinner syndrome, individuals may also exhibit ureterocele, hypospadias, and abnormalities affecting the epididymides, testes, or adrenal glands. The underlying cause of this condition lies in the improper formation of the Wolffian duct and its associated components during the embryonic period. From a developmental perspective, any disruption to the distal portion of the Wolffian duct can result in blockage of the ejaculatory duct and the formation of cysts within the seminal vesicles. The development of an ectopic ureter can result from the ureteric bud emerging below its normal point of insertion, which is the urogenital sinus. This case report documents a highly uncommon presentation of Zinner syndrome with aberrant insertion of the ureter into the seminal vesicle. We discuss the clinical manifestations, diagnostic procedures, and treatment approaches for this condition. This case also highlights the importance of multimodality imaging in identifying rare variants of Zinner syndrome. Magnetic resonance imaging (MRI), with its superior tissue contrast and radiation-free nature, can serve as a single-stop modality for comprehensive evaluation, reducing the need for computed tomography (CT) in such cases. This case report describes a 64-year-old man who presented with mild suprapubic discomfort and burning sensation during micturition. Based on radiological investigations, Zinner syndrome was diagnosed, with the identification of an ipsilateral hypoplastic ectopic ureter. The patient's treatment plan involved conservative measures, including antibiotics for the urinary tract infection, and scheduled follow-up visits. Despite its rarity and often inconspicuous nature, Zinner syndrome should be regarded as a crucial consideration in the differential diagnosis of males presenting with unilateral renal agenesis and cystic pelvic lesions. Although ultrasound can provide valuable insights, CT and MRI offer superior diagnostic accuracy.