Zinner syndrome: two cases and review of the literature

Abstract

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—A Rare Cause of Recurrent Epididymitis and Infertility

“…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 ]. The decreased semen parameters may be due to atresia of the ejaculatory tract, the presence of a hematoma in the seminal vesicle, or it may be secondary to inflammation of the genitourinary system [ 5 ].…”

“…Asymptomatic cysts are usually treated with alpha-blockers. Surgical treatment should be indicated only in patients with symptoms, in case of failure of previous therapy, or when the size of the cyst is greater than 5 cm [ 6 ]. To date, there are no official guidelines for the treatment of Zinner syndrome.…”

Unilateral scrotal discomfort in a patient with known renal agenesis: do not forget about Zinner syndrome

“…Transabdominal US is preferred because of less discomfort for the patient, whereas transrectal US provides excellent visualisation of seminal vesicle cysts and can be a good choice for the follow-up. 4 MRI on the other hand allows a multiplanar study, facilitates the differential diagnosis with other pelvic cystic malformation, and can be useful for surgical planning. 5 Typical findings are paramedian and periprostatic cysts, hypointense on T1-weighted and hyperintense on T2-weighted images, without contrast enhancement after gadolinium intravenous administration.…”

“…Ultrasound is usually the first diagnostic step because it does not involve radiation exposure, it is inexpensive and noninvasive. Transabdominal US is preferred because of less discomfort for the patient, whereas transrectal US provides excellent visualisation of seminal vesicle cysts and can be a good choice for the follow-up 4…”

Zinner syndrome: A first case report from Ethiopia

“…It occurs due abnormality of distal mesonephric duct and ureteric buds during early embryonic period [2] . Patients usually present in the 2nd to 4th decade of life with nonspecific urinary symptoms, local perineal symptoms or abnormal reproductive function [3 , 4] . The symptoms include pain, dysuria, frequency, perineal pain, postejaculation pain etc [4] .…”