Zinner Syndrome

Abstract

Zinner syndrome is a less common birth anomaly of the Wolffian duct consisting of unilateral kidney absence, ipsilateral ejaculatory duct obstruction, and seminal vesicle cyst. A failure of embryogenesis of the ureteric bud between the fourth and 13th week of gestation results in Zinner syndrome. Conservative treatment is recommended for asymptomatic patients, whereas invasive treatment is reserved for symptomatic patients and for those who have failed conservative treatment. In this case report, we describe the non-specific presentation of lower abdominal pain and dysuria, as well as episodes of hematuria and new-onset hypertension, in a male patient, who was otherwise deemed healthy, with no other previous medical or surgical history. An imaging study and laboratory investigations were performed, and the patient was detected to have left renal agenesis and hypointense/hyperintense cysts in the left seminal vesicle of the left kidney. The findings supported the diagnosis of Zinner syndrome. The patient did not present with any symptoms or findings that would suggest infertility at the time of the study.Zinner syndrome is a rare cause of painful micturition and hematuria in males and can be diagnosed using ultrasound (USS), computer tomography (CT), and magnetic resonance imaging (MRI) techniques. Zinner syndrome should be considered as a differential diagnosis in male patients with unilateral renal agenesis and cystic pelvic masses. Patients who are asymptomatic typically undergo conservative treatment and are followed up to prevent infertility. For patients with symptomatic cysts who fail to respond to conservative treatment or whose cysts are larger than 5 cm in diameter, surgical intervention is recommended (open or laparoscopic surgery and ejaculatory duct balloon dilatation).

Diseases and syndromes associated with unilateral renal agenesis in children

“…Синдром Циннера -редкая врождённая аномалия развития, характеризующаяся триадой признаков: наличием кисты семенного пузырька, ипсилатеральной аплазией почки или АгП и обструкцией семявыносящего протока. Эта аномалия протекает, как правило, бессимптомно и приводит к грозному осложнению -мужскому бесплодию [7,[38][39][40].…”

Unilateral Renal Agenesis, Think in Zinner Syndrome

“…vesicle cyst and ipsilateral ejaculatory duct obstruction [1][2][3][4]. This condition is quite uncommon, with fewer than 200 cases reported in the medical literature, and its estimated incidence is approximately 0.00035% to 0.0046% [2].…”

“…Infertility is a significant concern, affecting approximately 45% of individuals with this syndrome. Other clinical manifestations, while nonspecific, can include urinary tract infections, hematuria, dysuria, urinary urgency, suprapubic/ perineal pain, pain after ejaculation, epididymitis, and prostatitis [1][2][3][4].…”

“…Imaging is essential for the diagnosis of Zinner syndrome, with magnetic resonance imaging (MRI) being the most accurate exam. In the vast majority of patients, treatment is conservative (rather than surgical), aimed at managing symptoms and complications as they arise [2,3].…”