A rare case of Zinner syndrome: Triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ejaculatory duct obstruction

Abstract

A rare triad of Wolffian duct anomaly known as Zinner syndrome includes unilateral renal agenesis with ipsilateral seminal vesicle cyst and ejaculatory duct obstruction. It is often diagnosed in third and fourth decades of life. Patient presents with dysuria, perineal pain, infertility and painful ejaculation. The aim of this case report is to show the importance of the radiological imaging on diagnosis of Zinner syndrome. MRI being the modality of the choice for the confirmation of the diagnosis is vital in further management of the syndrome.

Zinner Syndrome—A Rare Cause of Recurrent Epididymitis and Infertility

“…Due to the increasing availability of imaging tests, the recognition of ZS is increasing. The characteristic of the syndrome includes unilateral renal agenesis, ipsilateral seminal vesicle cyst, and atresia of the ejaculatory tract [ 3 ]. The simultaneous occurrence of anomalies of the kidney and seminal vesicle has been described by embryogenesis.…”

“…The disturbing process at this stage explains the one-sided and related nature of these changes. The female counterpart of the ZS, which includes Muller ducts, is the Mayer-Rokitansky-Kustner-Hauser syndrome [ 3 ].…”

“…Patients also report pain in the perineum, abdomen, and pelvis. The disorders also affect the reproductive system and include, among others, infertility, painful ejaculation, and hematospermia [ 3 , 5 ]. Some publications indicate that up to 45% of patients with Zinner Syndrome will suffer from infertility [ 6 ].…”

“…CT images will visualize the cysts modeling the bladder and the ipsilateral agenesis of the kidney. The gold standard that most accurately depicts the anatomy of the male reproductive system is MRI [ 3 ]. It shows the connection between the cystic lesion and the seminal vesicle, which will confirm the origin of the cyst.…”

“…In the differential diagnosis, we should include the ejaculatory cyst, ureterocele, and bladder diverticula. MRI is an excellent way to differentiate these changes [ 3 ]. Typically, an uncomplicated cyst is anechoic [ 8 ].…”