Zinner Syndrome: Case report of atypical symptoms and literature

Abstract

Zinner Syndrome is a rare congenital anomaly of the urogenital tract, characterized by unilateral renal agenesis, ejaculatory duct obstruction and ipsilateral seminal vesicle cyst. Here, we present a case of a 54-year-old-male with anejaculation and frequent nocturnal emissions, denies fever, scrotal or perineal pain, hematospermia, hematuria, or lower urinary tract symptoms. Imaging studies revealed atypical findings of this syndrome. This study highlights the importance of recognizing atypical symptoms and appropriate management to relieve symptoms and improve quality of life.

1 Introduction

Zinner Syndrome, first described in 1914, is a rare congenital anomaly consists of the classic triad of unilateral renal agenesis, ipsilateral ejaculatory duct obstruction, and ipsilateral seminal vesicle cyst. Since then, approximately 200 cases of this condition have been reported in the literature. The accurate diagnosis can be easily achieved through a thorough physical examination and precise diagnostic imaging. Obstruction of the ejaculatory duct results in the accumulation of seminal fluid, which subsequently leads to the enlargement of the seminal vesicles. Symptoms are generally non-specific and primarily include pain, dysuria, frequent urination, perineal pain, epididymitis, and pain following ejaculation. Additionally, some studies have indicated that up to 45 % of patients with Zinner syndrome may develop infertility. ^,

The objective of this case report is to describe and analyze rare symptoms associated with Zinner syndrome that have not been previously documented in the medical literature.

2 Case description

The patient was a 54-year-old male with right renal agenesis from birth, diagnosed 20 years ago ( Fig. 1 ). The patient first experienced symptoms in 2019 but did not seek medical evaluation due to the COVID-19 pandemic. He later presented with complaints of anejaculation and persistent nocturnal emissions. The patient denied having any associated symptoms, including fever, scrotal or perineal pain, hematospermia, hematuria, or lower urinary tract symptoms.

On physical examination, the patient’s genitalia were found to be appropriate for his age. The penis was uncircumcised with no excoriations on the glans, and the meatus was patent and non-stenotic. Both testicles were well descended into the scrotal sacs without signs of calcifications, nodules, or indurations. Bilateral varicocele was present. Digital rectal examination revealed a 30 cc prostate that was smooth, non-fixed, and free of nodules, with a soft, fluctuant, and enlarged right lobe. The prostate was normothermic and non-tender upon palpation.

Laboratory evaluations were normal. Bladder ultrasonography measured a seminal vesicle associated cyst ( Fig. 2 a and b ) (see Fig. 3 ).