Seminal vesicle cysts, ipsilateral renal agenesis, and obstruction of the ejaculatory duct characterize Zinner syndrome. This condition is associated with embryological abnormalities of the mesonephric or Wolffian duct between the 4th and 13th gestational weeks. Parallels can be made between ZS and female congenital anomalies, such as Mullerian agenesis or Herlyn-Werner-Wunderlich syndrome (HWWS). Like HWWS, which involves uterine and vaginal malformations due to mesonephric duct, leading to ejaculatory duct obstruction, seminal vesicle issues and, renal agenesis/dysplasia.
During the typical embryological development of the kidneys, the mesonephric stage is essential in establishing basic renal architecture. At approximately week 5 of gestation, the mesonephric duct extends toward the cloaca. At this stage, the metanephric blastema will typically secrete glial cell-derived neurotrophic factor to induce outgrowth of the ureteric bud from the distal portion of the mesonephric duct to the collecting system and its components, including glomeruli and tubules.
In Zs, the ureteric bud fails to properly contact the mesonephric blastema, an essential step for the formation of the renal system. These developmental abnormalities lead to reproductive and renal system consequences- renal agenesis as well as ipsilateral atresia of the ejaculatory duct. This results in the inability to drain fluid through the ejaculatory ducts, leading to seminal vesicle cysts. The clinical symptoms associated with this condition, which are nonspecific, include frequent dysuria, epididymitis, haematospermia, perineal discomfort, infertility- painful ejaculation, obstruction of the ejaculation duct can cause discomfort during ejaculation and may lead to infertility issues. The severity of symptoms may correlate with the size of the seminal vesicle cyst. The majority of patients present in the second to fourth decade due to high levels of reproductive and sexual activity, but they may also present asymptomatically. It is often misinterpreted due to its non-specific signs and lack of symptoms. Usually, microcysts measuring less than 5 cm in diameter are dormant and only detected incidentally. It is observed that patients with this condition usually present with obstruction of the bladder outlet, resulting in the increased incidence of infection within the urinary bladder, and that bladder compression can occur in the presence of cysts up to 12 cm in size.