Zinner Syndrome

Zinner syndrome is a developmental anomaly of the urogenital tract. This condition is defined by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. The syndrome is due to malformation of the mesonephric duct during embryogenesis. The condition used to be rare but is now frequently encountered due to the advent of MRI and CT. MRI confirms the diagnosis by revealing the seminal vesicle cyst and its contents, and the ejaculatory duct obstruction, while CT confirms renal agenesis. We report the case of a young patient with Zinner syndrome.

Long-Term Surveillance and Laparoscopic Management of Zinner Syndrome

Zinner syndrome was first described in 1914 and represents the triad of unilateral renal agenesis and ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. Seminal vesicle cysts are often asymptomatic but can also present with pain, haematospermia, or other lower urinary tract symptoms. Treatment strategies include observation and surgical excision. We present the laparoscopic management of an enlarged seminal vesicle cyst, consistent with Zinner syndrome, 14 years after the initial diagnosis. A 58-year-old male patient was diagnosed with a left-sided seminal vesicle cyst while undergoing assessment for renal transplant due to progressively worsening renal function in his solitary right kidney. The otherwise asymptomatic cyst enlarged from the time of initial diagnosis in 2004 ( 11.3 cm × 9.7 cm × 13.1 cm ) to nearly double the size in 2018 ( 12.8 cm × 11.9 cm × 14.2 cm ). This cyst size ultimately precluded renal transplant, and the patient was referred for excision. Laparoscopic excision of the cyst was performed, histopathology confirmed seminal vesicle cyst tissue, and there has been no recurrence of the cyst to date. The patient remains active on the renal transplant waitlist. Zinner syndrome is a rare syndrome, with the seminal vesicle cysts being managed by observation or surgical excision. We report the longest documented observation of a seminal vesicle cyst, culminating in a safe and successful laparoscopic excision.

Sperm retrieval by conventional testicular sperm extraction for assisted reproduction in patients with Zinner syndrome

We present data from three Caucasian men with Zinner syndrome who attended our center for the treatment of primary couple’s infertility. Each patient was scheduled for multiple conventional testicular sperm extraction (cTESE) and cryopreservation. The mean duration of infertility was 243 months. Sperm analysis confirmed absolute azoospermia. In patient 1, imaging revealed right and left testis volumes of 24 mL and 23 mL, respectively; dilatation of the right caput epididymis, corpus, and cauda; left seminal vesicle agenesis; severe right seminal vesicle hypotrophy; right renal agenesis; and left nephroptosis. The patient’s levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), and total testosterone (TT) were 3.2 IU/L, 5.2 IU/L, and 22.3 nmol/L, respectively. Patient 2 exhibited right and left testis volumes of 18 mL and 16 mL, respectively; a left seminal vesicle cyst of 3228 mm; ipsilateral kidney absence; and right seminal vesicle agenesis. The patient’s levels of FSH, LH, and TT were 2.8 IU/L, 4.2 IU/L, and 14.3 nmol/L, respectively. In patient 3, ultrasound showed a testicular volume of 10 mL bilaterally, a 6546 mm left seminal vesicle cyst, right seminal vesicle enlargement, and left kidney agenesis. The patient’s levels of FSH, LH, and TT were 32.0 IU/L, 16.3 IU/L, and 9.0 nmol/L, respectively. Sperm retrieval via cTESE was successful in all patients. It may be advisable to perform cTESE to obtain fresh sperm on the day of oocyte retrieval. Sperm cryopreservation should be avoided, since thawing could further compromise the sperm quality.

Ectopic ureter associated with Zinner’s syndrome in a kidney recipient: case report and literature review

SUMMARY INTRODUCTION Zinner’s Syndrome is a triad of mesonephric duct anomalies comprising unilateral renal agenesis, seminal vesicle cyst, and ejaculatory duct obstruction. In this study, we present a kidney recipient with ectopic ureter associated with Zinner’s syndrome and a literature review. CASE PRESENTATION A 59-year-old male with a history of chronic kidney disease and left renal agenesis underwent deceased donor kidney transplantation. After securing optimal renal functions, the patient underwent abdominal computed tomography (CT) scan for the seroma that occurred under the incision. The final diagnosis was an ectopic distal ureter ending in the seminal vesicle cyst’s wall and ipsilateral renal agenesis. The patient was discharged without any complications and the clinical follow up was uneventful. DISCUSSION AND CONCLUSION Congenital seminal vesicle disorders are usually associated with ipsilateral urinary duct anomalies stemming from the same embryonic structure. To our knowledge, this is the first case report that describes kidney transplantation in a patient with ipsilateral renal agenesis and ectopic ureter ending in the seminal vesicle cyst. In patients with renal agenesis, during the ipsilateral urinary tract anastomosis, the possibility of ectopic ureter should be kept in mind otherwise graft loss can occur with a high morbidity rate.