Zinner syndrome incidentally diagnosed in a man with ureteropelvic junction stone and hydronephrosis: A case report

Abstract

Zinner syndrome is a congenital anomaly characterized by seminal vesicle cysts, ipsilateral renal agenesis, and ejaculatory duct obstruction possibly associated with infertility. Only 200 cases of Zinner syndrome have been reported since its discovery in 1914. We present the case of a 63-year-old man seeking treatment for a ureteropelvic junction stone causing severe hydronephrosis. After the patient’s history and the computed tomography findings were reviewed, the diagnosis of Zinner syndrome was confirmed. The patient has been nearly asymptomatic and has had three children during his lifetime. Our case could serve as a reference for future diagnoses of this rare anomaly.

1 Introduction

Zinner syndrome is an extremely rare congenital anomaly of the mesonephric or Wolffian duct. The syndrome is characterized by seminal vesicle cysts, ipsilateral renal agenesis, and ejaculatory duct obstruction. This condition was first described by Zinner in 1914, and approximately 200 cases have been reported ever since. . The most common symptoms are urinary frequency, dysuria, and perineal pain. The enlargement of seminal vesicle cysts often leads to compression of the bladder, ureter, and reproductive system, which may cause infertility. . The diagnosis often depends on radiological examination, including magnetic resonance imaging and computed tomography (CT).

In this report, we present the case of a 63-year-old man presenting with flank pain without any history of symptoms related to Zinner syndrome. However, the results of radiological examination revealed anomalies compatible with Zinner syndrome.

2 Case presentation

A 63-year-old man with three daughters presented to the outpatient department with left flank pain for 3 days and right flank pain for 1 day. He denied experiencing frequent urination, fever, or trauma. Physical examination revealed mild flank pain elicited by percussion. Renal ultrasound revealed a left ureteropelvic junction (UPJ) stone with severe hydronephrosis (Grade 4) in the thin cortex of the left kidney, whereas the right kidney was invisible on the ultrasound image. The patient initially hesitated when percutaneous nephrostomy was suggested. He then underwent a laboratory examination and CT scanning.

The laboratory findings indicated decreased kidney function (creatinine: 1.81 mg/dL, enhanced glomerular filtration rate: 38 mL/min/1.73 <SPAN role=presentation tabIndex=0 id=MathJax-Element-1-Frame class=MathJax style="POSITION: relative" data-mathml='m2′>𝑚2
m 2
) and microscopic hematuria (urine occult blood: 3+).

A CT scan highlighted 1) a 15-mm left UPJ stone with moderate left hydronephrosis ( Fig. 1 ); 2) a 48-mm cystic lesion in the right pelvic region (between the ureter bladder and prostate gland), indicative of a right seminal vesicle cyst ( Fig. 2 ); and 3) nonvisualization of the right kidney with residual dilated right ureter connecting to the right seminal vesicle cyst ( Fig. 1 ). Dilation of the right vas deferens was observed that potentially indicated an obstruction in the right ejaculatory duct ( Fig. 2 ). Other findings from the CT scan included a double inferior vena cava and an accessory spleen.