Congenital Seminal Vesicle Cyst and Ipsilateral Renal Agenesis (Zinner Syndrome): A Rare Association and Its Evolution from Early Childhood to Adolescence

Abstract

Zinner syndrome, the association of congenital seminal vesicle cyst and ipsilateral renal agenesis, is more often reported in adults or older adolescents. We present a case of a boy, followed up in our hospital since birth for right renal agenesis who at the age of 4 years presented a right paravesical cyst on ultrasound. The cyst was initially considered as an ureterocele. The diagnosis of Zinner syndrome was made later, at the age of 15 years by ultrasound and magnetic resonance imaging; at that moment the cyst had increased in size and had changed in aspect. This malformation should be considered in the differential diagnosis of a pelvic cyst in male patients with renal agenesis.

Incidental finding of Zinner syndrome in a Greek military recruit: a case report of a rare clinical entity

“…Zinner syndrome is characterized by the triad of renal agenesis, ipsilateral SVC and ejaculatory duct obstruction [2, 8]. It is considered as the male counterpart of Mayer-Rokitansky-Kustner-Hauser female syndrome [3, 9].…”

“…Hematospermia, recurrent episodes of visible hematuria and ejaculation failure are the most commonly associated symptoms [1, 6]. Nonspecific symptoms, namely intermittent scrotal pain, perianal discomfort, and urinary frequency, have also been reported [3, 6, 9].…”

“…Contrary to our case, van den Ouden et al [6] demonstrated a right-sided preponderance of the syndrome. In 2015, Kanavaki et al [3] reported the case of a 4-year-old boy who presented with a right paravesical cyst on ultrasound that was initially diagnosed as ureterocele. Eventually, after 11 years of annual ultrasonographic follow-up, the diagnosis of Zinner syndrome was established.…”

“…Zinner syndrome, which was first described in 1914, comprises a rare congenital malformation of the genitourinary tract [1–4]. An aberrant development of the mesonephric duct and absence of the ureteric bud during embryogenesis leads to ipsilateral renal agenesis and atresia of the ejaculatory duct, which subsequently progresses to cystic dilation of seminal vesicles, a unique characteristic of this syndrome [3, 5, 6]. Patients can remain asymptomatic for an extended period [3–5].…”

“…An aberrant development of the mesonephric duct and absence of the ureteric bud during embryogenesis leads to ipsilateral renal agenesis and atresia of the ejaculatory duct, which subsequently progresses to cystic dilation of seminal vesicles, a unique characteristic of this syndrome [3, 5, 6]. Patients can remain asymptomatic for an extended period [3–5]. Indeed, the clinical manifestation strongly correlates to the onset of sexual activity [6].…”