Yue Zhao1, Xiuhong Guan2, Yongzhou Xu3, Xinqing Jiang2, and Ruimeng Yang2
1Guangzhou First People’s Hospital, Guangzhou, China, 2Department of Radiology, Guangzhou First People’s Hospital, Guangzhou, China, 3Philips Healthcare, Guangzhou, China
Synopsis
Keywords: Urogenital, Pelvis
Zinner's syndrome is a rare congenital urogenital abnormality. Since this disease is rare, knowledge about its imaging manifestations by clinical surgeons and radiologists is inadequate. In this study, data of 11 cases of Zinner's syndrome were retrospectively analyzed, and their morphological characteristics on imaging changes were investigated, aiming to improve the knowledge and differential diagnosis of this disease. The relevant images of Zinner's syndrome show characteristic manifestations, so the seminal vesicle cysts and isolateral kidney development must be carefully observed. Comprehensive judgement should be made according to urogenital mutation or deformity and systemic development situation.
Introdution
Zinner's
syndrome is a rare congenital urogenital abnormality and mainly includes unilateral renal agenesis or renal hypoplasia
complicated with isolateral seminal vesicle cyst and ejaculatory duct
obstruction [1-4]. Clinical statistics show the incidence of
Zinner's syndrome is only 2.14/100000 [5]. Since this disease is rare, knowledge about its imaging
manifestations by clinical surgeons and radiologists is inadequate. In this
study, data of 11 cases of Zinner's syndrome were retrospectively analyzed,
aiming to improve the knowledge and differential diagnosis of this disease. Materials and methods
The
data from 11 Zinner's syndrome patients diagnosed clinically and by imaging
between June 2010 and December 2021 were collected. All 11 patients were males
aged 22 to 68 years. Their clinical symptoms
include hemospermia and infertility (4 cases), frequent urination and urinary
incontinence (3 cases), dysuria (2 cases), and occasional discovery upon
medical examination (2 cases).
Six of
the 11 patients received magnetic resonance (MR) plain scan plus enhanced scan,
4 cases underwent computed tomography (CT) plain scan plus enhanced scan, 1
case received CT plain scan first and then MR plain scan plus enhanced scan. The major contents of
image reading were to determine: (1) the concrete positions of cysts: lower
ureter segment - inner bladder walls, prostate, seminal vesicle or
verumontanum;(2) shape: regular shape or not, tube-like or circular,
round-like, single or frequently-occurring; (3) size: the largest cyst diameter
measured on the cross- section;(4) boundary: whether cystwalls were smooth or
not; whether mural nodules existed or not; whether focal margins were blur or
not;(5) density or signal of contents: compared with the density or signal of
intrabladder urine, and whether bleeding occurred or not;(6) changes in
adjacent organs and tissues: whether bladders, prostate and rectum were
compressed, and the degree of compression. Results
On the
abdominal pelvic CT or MR images, 8 cases are manifested as unilateral renal
agenesis complicated with isolateral seminal vesicle cyst (6 cases on the
right, 2 cases on the left); 2 cases as left renal agenesis
complicated with bilateral seminal vesicle dysplasia; 1 case as left
renal dysplasia complicated with left seminal vesicle cyst. Nine cases
have single-shot cysts, which are ovate-shaped with the largest diameter of
3.8-6.5 cm (6.2±2.3 cm); 2 cases have significant cystic dilation. On
the CT images, the cysts are manifested as hypointensity (2 cases, simple
cysts), or hyperintensity (3 cases, complicated cysts) higher than that of
intrabladder urine. The range of CT values is 3-52 HU (median of 36 HU). The inner
part of enhanced scans is not enhanced. MR images show hypointensity (2 cases,
pure cysts) or hyperintensity (5 cases, complicated cysts) on T1WI, and
hyperintensity on T2WI in all cases, with liquid-liquid planes in 3 cases. Seven patients were compressed obviously at unilateral bladder posterior
walls, 2 patients were slightly compressed at unilateral prostate, and 6 cases
were compressed and changed at rectum anterior walls.Discussion
The
major cystic lesions of prostate-seminal vesicle are seminal vesicle cysts,
Mullerian duct cysts, and ejaculatory duct cysts. According to the position,
shape and compression way of cysts, seminal vesicle cysts can be easily
differentiated from Mullerian duct cysts and ejaculatory duct cysts. The
seminal vesicle cyst is located at one side or both sides of the seminal
vesicle and is separated by prostate capsules from the prostate and surrounded
by seminal ducts. It is either single or multiple, and the large cysts can
compress the posterior bladderic walls [6]. The Mullerian duct cyst is located at
the verumontanum between the middle of the prostate and the bilateral seminal
vesicle, and results from incomplete degeneration of the Mullerian duct formed
inside the prostate. The Mullerian duct cyst is single and not connected with
the seminal duct. It protrudes upwards the bladder, and its lower part is sharp
and extends to the prostate. The sagittal plane or coronary plane is typically
inverted and drop-like [7]. The ejaculatory duct cyst is located at the
verumontanum in the posterior urethra prostate and is often single and
unilateral. It is induced by ejaculatory duct dilatation. On the cross-section,
it points from the outer posterior to the inner anterior. On the coronary
plane, it extends from the outer superior to the lower inner part. It often
does not compress or alter the bladder and rectum.
After
confirmation as seminal vesicle cyst, the key to differential diagnosis is to
differentiate between Zinner's syndrome-related or simple seminal vesicle cyst.
A Zinner's syndrome seminal vesicle cyst can be regarded as a special seminal
vesicle cyst and is further developed from the central nephridium structure
(ureteric residues) remaining in the seminal vesicle and is correlated with the
maturity and hormone level of the reproductive system [8]. When symptoms appear, it
is usually manifested as a large-volume round-like or irregular polycystic
tube-like structure, which significantly suppresses the ipsolateral posterior
bladder walls. In comparison, simple seminal vesicle cysts only locally expand
in the seminal duct and are regularly shaped, which is either round or
ovate-like.Conclusion
The images of Zinner's syndrome show
characteristic manifestations, so the seminal vesicle cysts and isolateral
kidney development must be carefully observed. Comprehensive judgement should
be made according to urogenital mutation or deformity and systemic development
situation. Acknowledgements
No acknowledgement found.References
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