A Triad of Unilateral Renal Dysgenesis with Ipsilateral Seminal Vesical and Ejaculatory Duct Obstruction: An Uncommon Urogenital Congenital Anomaly, Zinner Syndrome—A Case Report

Zinner syndrome is a rare congenital anomaly of the urogenital system resulting from an in utero insult during the first trimester. This entity comprises a triad of unilateral renal agenesis/dysgenesis with ipsilateral seminal vesical and ejaculatory duct obstruction. This combination of urinary and genital abnormalities occurs because of the closely related embryological origin of these structures from the distal mesonephric (Wolffian) duct. Nearly 200 cases of seminal vesical cysts with ipsilateral renal agenesis have been reported in the literature. The affected person generally presents in early adulthood when the reproductive activity commences. In this report, we present a case of a 22-year-old male with complaints of painful ejaculation.

Zinner Syndrome with Ectopic Ureter Remnant

: Zinner syndrome is a rare congenital abnormality defined by a clinical triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. Most patients are asymptomatic, but if the cystic dilatation of the seminal vesicle becomes significant, it can result in urinary symptoms such as dysuria and urinary retention. This rare developmental anomaly related to the mesonephric duct can also present with other abnormalities. Here, we report our experience of Zinner syndrome with bladder outlet obstruction and an ectopic ureter remnant.

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.

Zinner syndrome: two cases and review of the literature

Zinner syndrome (ZS) is a rare congenital malformation due to abnormal development of the urogenital tract. It is caused by a growth failure of the distal part of the Müllerian duct in early embryogenesis. It is characterised by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. Over the years, several cases have been reported in the literature since the initial report by Zinner in 1914. This syndrome is frequently misdiagnosed because it may present different patterns and the symptoms may be not specific. In this paper, we present two patients with two different patterns of presentation of Zinner syndrome.

Zinner Syndrome with Ectopic Ureter Emptying into Seminal Vesicle

A 66-year-old male patient in follow-up in the urology department for a non-muscle-invasive bladder cancer was detected by ultrasound to have absence of the left kidney and a cystic, multilobed image at the location of the seminal vesicle. Magnetic resonance imaging reveals left renal agenesis and the existence of multiple cysts in the ipsilateral seminal vesicle that reaches a size of 6.9 × 3.7 cm , as well as a ureteral remnant that opens into the seminal vesicle. The patient does not present urinary symptoms, neither pain with ejaculation nor hematuria. A triad of seminal vesicle cyst, ipsilateral renal agenesis, and ipsilateral ejaculatory duct obstruction is known as Zinner syndrome. Congenital anomalies of the seminal vesicles are rare; some of them are associated with malformations of the upper urinary system. Seminal vesicle cysts are associated with ipsilateral renal agenesis and ectopic or dysplastic ureter. Patients may remain asymptomatic and be diagnosed incidentally or may present with symptoms such as increased urinary frequency, dysuria, recurrent infections, pain with ejaculation, and perineal discomfort.

A wide spectrum of rare clinical variants of Zinner syndrome

Congenital malformations of the seminal vesicles (SVs) are rare and are associated with abnormalities of the ipsilateral urinary tracts as embryologically both the ureteral buds and SVs arise from the mesonephric ducts. The triad of SV cysts, ipsilateral renal agenesis and ejaculatory duct obstruction is known as the Zinner syndrome. We, herein, present three very rare presentations of Zinner syndrome. Case 1 presented with haematuria, and was found to have a large SV cyst with stones and underwent a robotic cyst excision. Case 2 presented with primary infertility, and was found to have a variant of Zinner syndrome. Case 3 was a known case of chronic kidney disease on maintenance haemodialysis who presented with fever and oliguria. He was found to have Zinner syndrome and underwent aspiration of SV abscess. To the best of our knowledge, such varying presentations of Zinner syndrome have been rarely reported thus far.