Zinner syndrome presenting with intermittent scrotal pain in a young man

Abstract

Congenital malformations of the seminal vesicle are uncommon, and most of them are cystic malformations. If an insult occurs during the first trimester of gestation, the embryogenesis of the kidney, ureter, seminal vesicle, and vas deferens could be altered. The mutual embryological origins of the seminal vesicle and ureteral bud from the mesonephric (Wolffian) duct result in association between ipsilateral renal agenesis and seminal vesical cysts. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction and ipsilateral ejaculatory duct obstruction. This syndrome were first described by Zinner in 1914, and 200 cases have been reported in the literature. Most patients with this anomaly are asymptomatic until the second or third decade of life. Some cases have nonspecific symptoms such as prostatism, urinary urgency, dysuria, painful ejaculation, and perineal discomfort. In this paper, we present a uncommon case of a 21-year-old patient which the initial presentation of this condition was intermittent scrotal pain. A brief review of the literature is undertaken, regarding the main clinical, imaging implications, and the developmental anomalies that are involved in this congenital anomaly.

Keywords: Ejaculation; Genital diseases; Hemospermia; Infertility; Mesonephric duct abnormality; Zinner syndrome.

Fig. 1

Abdominopelvic computerized tomography. (A and B) Axial and coronal CT-enhanced phase, reveal vicariant left kidney (*) and right kidney fossa empty. (C and D) Axial CT-enhanced phases reveal, seminal vesical asymmetric, right seminal vesicle is enlarged (white arrow) and efferent duct (orange arrow). Left seminal vesicle is normal morphology (red arrow). (Color version of figure is available online.)

Fig. 2

Pelvic MRI. (A and B) Axial T1- and T2-weighted MRI image showing dilated and cystic right seminal vesicle, which reveals hyperintensity in T1-weighted images (A). (C and D) Saggital and coronal T2-weighted MRI image with fat saturation showing enlargement of ejaculatory duct (orange arrow). (Color version of figure is available online.)